La Jolla Pharmaceutical Company Highlights Recent Corporate Progress and Key Objectives

– Fourth-Quarter 2018 Net Sales of GIAPREZA Were Approximately $4.2 Million, Up 20% from the Third Quarter of 2018 –

– Full-Year 2019 Net Sales Guidance for GIAPREZA of $24 Million to $28 Million –

– Decision on GIAPREZA MAA by EMA Expected in June of 2019 –

– NDA Planned for New Investigational Product, LJPC-0118, for the Treatment of Severe Malaria, in Fourth Quarter of 2019 –

– Topline Results of Phase 2 Study of LJPC-401 in Patients with Hereditary Hemochromatosis Expected in Second Half of 2019 –

– Topline Results of Pivotal Study of LJPC-401 in Patients with Beta Thalassemia Expected in Mid-2020 –

SAN DIEGO, CA – January 7, 2019 – La Jolla Pharmaceutical Company (Nasdaq: LJPC), a leader in the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases, today highlighted recent corporate progress and key objectives.

Recent Corporate Progress and Key Objectives

GIAPREZA™ (angiotensin II)

Fourth-Quarter 2018 Net Sales: Fourth-quarter 2018 net sales were approximately $4.2 million, up 20% from the third quarter of 2018.

• 2018 Net Sales: 2018 net sales were approximately $10.1 million. GIAPREZA was launched in March 2018.

Full-Year 2019 Net Sales Guidance: La Jolla expects full-year 2019 net sales of $24 million to $28 million.

• Decision on GIAPREZA MAA by EMA Expected in June of 2019: La Jolla expects a decision on the GIAPREZA Marketing Authorisation Application (MAA) by the European Medicines Agency (EMA) in June of 2019.

Investigational Products Update

NDA Planned for New Investigational Product, LJPC-0118, for the Treatment of Severe Malaria, in Fourth Quarter of 2019: La Jolla plans to file a New Drug Application (NDA) with the U.S. Food and Drug Administration (FDA) in the fourth quarter of 2019 for LJPC-0118. LJPC-0118 is La Jolla’s new investigational product for the treatment of severe malaria. The active pharmaceutical ingredient in LJPC-0118 was demonstrated to be superior to quinine in reducing mortality in patients with severe falciparum malaria infection in two randomized, controlled, clinical studies.

• Topline Results of Phase 2 Study of LJPC-401 in Patients with Hereditary Hemochromatosis Expected in Second Half of 2019: La Jolla expects topline results in the second half of 2019 for LJ401-HH01, a multinational, multicenter, randomized, Phase 2 study that is designed to evaluate the safety and efficacy of LJPC-401, La Jolla’s proprietary formulation of synthetic human hepcidin, as a treatment for hereditary hemochromatosis (HH). The primary efficacy endpoint of the study is the change in transferrin saturation, a standard measurement of iron levels in the body and one of the two key measurements used to detect iron overload, from baseline to end of treatment.

• Topline Results of Pivotal Study of LJPC-401 in Patients with Beta Thalassemia Expected in Mid-2020: La Jolla expects topline results in mid-2020 for LJ401-BT01, a pivotal, multinational, multicenter, randomized, controlled study that is designed to evaluate the safety and efficacy of LJPC-401 as a treatment for beta thalassemia (BT) patients who, despite chelation therapy, have cardiac iron levels above normal. The primary efficacy endpoint of this study is the change in iron content in the heart after 6 months, as measured by cardiac magnetic resonance imaging (MRI). If this study is successful, La Jolla anticipates filing an MAA for LJPC-401 in the European Union (EU).

As of December 31, 2018, La Jolla had approximately $172 million in cash and cash equivalents and no debt. In 2019, La Jolla expects that its net cash used in operating activities will be $89 million to $94 million.

“We are excited to have launched GIAPREZA, our first commercial product, in 2018. We believe that GIAPREZA has the potential to help many patients suffering from septic or other distributive shock, and we have a number of initiatives that we believe will support its continued, increased adoption in 2019,” said George Tidmarsh, M.D., Ph.D., La Jolla’s President and Chief Executive Officer. “Our two randomized studies of LJPC-401 in BT and HH have the potential to demonstrate improved patient outcomes in these important diseases. Furthermore, we are excited to announce a planned NDA submission for LJPC-0118, our new investigational product for the treatment of severe malaria, in the fourth quarter of 2019.”

About GIAPREZA

In December 2017, GIAPREZA™ (angiotensin II) was approved by the U.S. Food and Drug Administration (FDA) as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. GIAPREZA mimics the body’s endogenous regulatory peptide that is central to the renin-angiotensin-aldosterone system to increase blood pressure. Prescribing information for GIAPREZA is available at www.giapreza.com. GIAPREZA is marketed by La Jolla Pharmaceutical Company on behalf of La Jolla Pharma, LLC, its wholly owned subsidiary.

IMPORTANT SAFETY INFORMATION

Contraindications

None

Warnings and Precautions

There is a potential for venous and arterial thrombotic and thromboembolic events in patients who receive GIAPREZA. Use concurrent venous thromboembolism (VTE) prophylaxis.

Adverse Reactions

The most common adverse reactions that were reported in greater than 10% of GIAPREZA-treated patients were thromboembolic events.

Drug Interactions

Angiotensin converting enzyme (ACE) inhibitors may increase response to GIAPREZA. Angiotensin II receptor blockers (ARB) may reduce response to GIAPREZA.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1- 800-FDA-1088.

For additional information, please see Full Prescribing Information.

About LJPC-0118

LJPC-0118 is La Jolla’s investigational product for the treatment of severe malaria. The active pharmaceutical ingredient in LJPC- 0118 was demonstrated to be superior to quinine in reducing mortality in patients with severe falciparum malaria infection in two randomized, controlled, clinical studies. La Jolla plans to file a New Drug Application (NDA) with the U.S. Food and Drug Administration (FDA) in the fourth quarter of 2019 for LJPC-0118. Severe malaria is a serious and sometimes fatal disease caused by a parasite that commonly infects a certain type of mosquito, which feeds on humans. Symptoms include but are not limited to: fever, chills, sweating, hypoglycemia and shock. Severe malaria is often complicated by central nervous system infections that may lead to delirium, which may progress to coma. Infections usually occur a few weeks after being bitten. In 2017, an estimated 219 million cases of malaria occurred worldwide, with an estimated 200 million of these cases occurring in the World Health Organization (WHO) African Region, and, in 2013, the global annual incidence of severe malaria was estimated to be 2 million cases. In 2017, an estimated 435,000 people died from malaria worldwide.

About LJPC-401

LJPC-401, a clinical-stage investigational product, is La Jolla’s proprietary formulation of synthetic human hepcidin. Hepcidin, an endogenous peptide hormone, is the body’s naturally occurring regulator of iron absorption and distribution. In healthy individuals, hepcidin prevents excessive iron accumulation in vital organs, such as the liver and heart, where it can cause significant damage and even result in death. La Jolla is developing LJPC-401 for the potential treatment of iron overload, which occurs as a result of primary iron overload diseases such as hereditary hemochromatosis (HH), or secondary iron overload diseases such as beta thalassemia, sickle cell disease (SCD) and myelodysplastic syndrome (MDS). The European Medicines Agency (EMA) Committee for Orphan Medicinal Products (COMP) has designated LJPC1401 as an orphan medicinal product for the treatment of beta thalassemia intermedia and major and SCD.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases.
GIAPREZA™ (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) on December 21, 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. LJPC-0118 is La Jolla’s investigational product for the treatment of severe malaria. LJPC1401 (synthetic human hepcidin), a clinical-stage investigational product, is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information, please visit www.ljpc.com.

Forward-Looking Statements

This press release contains forward-looking statements as defined by the Private Securities Litigation Reform Act of 1995, including, but not limited to, statements related to La Jolla’s expectations regarding net sales and net cash used in operating activities for the full-year 2019, the expectation for future clinical and regulatory milestones, such as NDA submission and expected timing for commencement and completion of clinical studies. These statements relate to expectations regarding future events, preliminary results subject to audit by their independent registered public accounting firm, or La Jolla’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors that may cause actual results to be materially different from those anticipated by the forward- looking statements. La Jolla cautions readers not to place undue reliance on any such forward-looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties and other factors are described in greater detail in La Jolla’s filings with the U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s website at www.sec.gov. These risks include, but are not limited to, risks relating to: our ability to successfully commercialize, market and achieve market acceptance of GIAPREZA; our ability to grow net sales of GIAPREZA; potential market sizes, including for septic or other distributive shock; the timing and prospects for approval of GIAPREZA by the European Medicines Agency (EMA) or other regulatory authorities; the scope of product label(s) and potential market sizes, as well as the broader commercial opportunity for GIAPREZA and our product candidates; the impact of pharmaceutical industry regulation and healthcare legislation in the United States; the success of future development activities; potential indications for which La Jolla’s product candidates may be developed; the timing, costs, conduct and outcome of clinical studies; risks relating to the development of drug candidates; the anticipated treatment of future clinical data by the U.S. Food and Drug Administration (FDA), EMA and other regulatory authorities, including whether such data will be sufficient for approval; the expected duration over which La Jolla’s cash balances will fund its operations; and other risks and uncertainties identified in our filings with the SEC. Forward- looking statements are presented as of the date of this press release, and La Jolla expressly disclaims any intent to update any forward-looking statements to reflect the outcome of subsequent events.

Company Contacts

Sandra Vedrick
Director, Investor Relations & Human Resources
La Jolla Pharmaceutical Company

Phone: (858) 207-4264 Ext: 1135
Email: svedrick@ljpc.com

and

Dennis M. Mulroy
Chief Financial Officer
La Jolla Pharmaceutical Company

Phone: (858) 207-4264 Ext: 1040
Email: dmulroy@ljpc.com

La Jolla Pharmaceutical Company to Present at the 37th Annual J.P. Morgan Healthcare Conference

SAN DIEGO, Jan. 04, 2019 (GLOBE NEWSWIRE) — La Jolla Pharmaceutical Company (Nasdaq: LJPC), a leader in the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases, today announced that George F. Tidmarsh, M.D., Ph.D., President and Chief Executive Officer of La Jolla, will present at the 37th Annual J.P. Morgan Healthcare Conference on Wednesday, January 9, 2019, at 5:00 p.m. PST at the Westin St. Francis Hotel in San Francisco, CA.

A live webcast of this presentation will be available on the Company’s website at www.ljpc.com in the Investor Relations section. A replay of the presentation will be archived on the site for 60 days.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. GIAPREZA™ (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) on December 21, 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. LJPC‑401 (synthetic human hepcidin), a clinical-stage investigational product, is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information, please visit www.ljpc.com.

Company Contacts

Sandra Vedrick
Director, Investor Relations & Human Resources
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1135
Email: svedrick@ljpc.com

and

Dennis M. Mulroy
Chief Financial Officer
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1040
Email: dmulroy@ljpc.com 
La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company Announces Financial Results for the Three and Nine Months Ended September 30, 2018

SAN DIEGO, Oct. 24, 2018 (GLOBE NEWSWIRE) — La Jolla Pharmaceutical Company (Nasdaq: LJPC), a leader in the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases, today announced financial results for the three and nine months ended September 30, 2018.

For the three months ended September 30, 2018, GIAPREZA™ (angiotensin II) net product sales were $3.5 million. This compares to $1.6 million for the three months ended June 30, 2018 and $0.8 million for the three months ended March 31, 2018. La Jolla launched GIAPREZA in the U.S. in March 2018. For the nine months ended September 30, 2018, GIAPREZA net product sales were $5.9 million. In December 2017, GIAPREZA was approved by the U.S. Food and Drug Administration (FDA) as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. La Jolla’s net loss for the three and nine months ended September 30, 2018 was $50.7 million and $154.1 million, or $1.93 per share and $6.15 per share, respectively, compared to $26.3 million and $76.3 million, or $1.19 per share and $3.65 per share, respectively, for the same periods in 2017.

As of September 30, 2018, La Jolla had $204.3 million in cash and cash equivalents, compared to $90.9 million as of December 31, 2017. The increase in cash and cash equivalents was the result of $109.8 million of net proceeds from the March 2018 common stock offering and $124.3 million of net proceeds from the May 2018 royalty financing, offset primarily by cash used for operating activities. Cash used for operating activities for the three and nine months ended September 30, 2018 was $36.9 million and $120.3 million, respectively, compared to $18.5 million and $59.7 million, respectively, for the same periods in 2017.

In October 2018, La Jolla effected a Company-wide realignment to increase its focus and efficiency on achieving its corporate goals. La Jolla’s highest priorities remain the effective commercialization of GIAPREZA and development of its product pipeline, including LJPC-401 for multiple indications. As a result of the realignment, La Jolla expects that its cash and cash equivalents as of September 30, 2018 will be sufficient to fund its operations at least into the second half of 2020.

“Interest in GIAPREZA by intensive care physicians and their institutions continues to grow, and we are pleased to report that GIAPREZA is now on formulary in more than 300 hospitals in the U.S.,” said George Tidmarsh, M.D., Ph.D., La Jolla’s President and Chief Executive Officer. “With the validation of our Marketing Authorisation Application for GIAPREZA in June 2018, we look forward to also bringing this important treatment to patients in Europe in 2019, pending approval by the European Medicines Agency.”

Conference Call Details

La Jolla will host a conference call and webcast today, October 24, 2018, at 4:30 p.m. Eastern Time (1:30 p.m. Pacific Time). The conference call can be accessed by dialing 877-359-9508 for domestic callers and 224-357-2393 for international callers. Please provide the operator with the conference ID number 5899629 to join the conference call or click here for the webcast. An archive of the conference call and webcast will be available on La Jolla’s website for 30 days following the call. 

About Shock and Septic or Other Distributive Shock

Over 1 million Americans are affected by shock on an annual basis, with 1 in 3 patients being treated for shock in the intensive care unit. Distributive shock is the most common type of shock in the inpatient setting with approximately 800,000 distributive shock cases in the United States per year. Of these cases, an estimated 90% are septic shock patients. Approximately 300,000 do not achieve adequate blood pressure response with standard of care vasopressor therapy (catecholamines and vasopressin). The inability to achieve or maintain adequate blood pressure results in inadequate blood flow to the body’s organs and tissue and is associated with a mortality rate exceeding most acute conditions requiring hospitalization. In the European Union, the annual incidence of sepsis in adults is estimated to be more than 500,000, with more than 170,000 progressing to septic shock.

About GIAPREZA

In December 2017, GIAPREZA™ (angiotensin II) was approved by the U.S. Food and Drug Administration (FDA) as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. GIAPREZA mimics the body’s endogenous regulatory peptide that is central to the renin-angiotensin-aldosterone system to increase blood pressure. Prescribing information for GIAPREZA is available at www.giapreza.com. GIAPREZA is marketed by La Jolla Pharmaceutical Company on behalf of La Jolla Pharma, LLC, its wholly owned subsidiary.

IMPORTANT SAFETY INFORMATION
Contraindications

None

Warnings and Precautions

There is a potential for venous and arterial thrombotic and thromboembolic events in patients who receive GIAPREZA. Use concurrent venous thromboembolism (VTE) prophylaxis.

Adverse Reactions

The most common adverse reactions that were reported in greater than 10% of GIAPREZA-treated patients were thromboembolic events.

Drug Interactions

Angiotensin converting enzyme (ACE) inhibitors may increase response to GIAPREZA. Angiotensin II receptor blockers (ARB) may reduce response to GIAPREZA.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

For additional information, please see Full Prescribing Information.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. GIAPREZA™ (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) on December 21, 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. LJPC‑401 (synthetic human hepcidin), a clinical-stage investigational product, is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information, please visit www.ljpc.com.

Forward-looking Statements

This press release contains forward-looking statements, as that term is defined in the Private Securities Litigation Reform Act of 1995. These statements relate to expectations regarding future events or La Jolla’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors that may cause actual results to be materially different from those anticipated by the forward-looking statements. La Jolla cautions readers not to place undue reliance on any such forward-looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties and other factors are described in greater detail in La Jolla’s filings with the U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s website at www.sec.gov. These risks include, but are not limited to, risks relating to: our ability to successfully commercialize, market and achieve market acceptance of GIAPREZA and other product candidates; potential market sizes, including for septic or other distributive shock; the success of development activities for LJPC-401 and other product candidates, including post-approval studies for GIAPREZA; the successful and timely completion of clinical trials; unforeseen safety issues from the administration of product and product candidates in patients; the expected duration over which La Jolla’s cash balances will fund its operations; and other risks and uncertainties identified in our filings with the SEC. Forward-looking statements are presented as of the date of this press release, and La Jolla expressly disclaims any intent to update any forward‑looking statements to reflect the outcome of subsequent events.

LA JOLLA PHARMACEUTICAL COMPANY

Unaudited Condensed Consolidated Statements of Operations

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LA JOLLA PHARMACEUTICAL COMPANY

Condensed Consolidated Balance Sheets

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Company Contacts

Sandra Vedrick
Director, Investor Relations & Human Resources
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1135
Email: svedrick@ljpc.com

and

Dennis M. Mulroy
Chief Financial Officer
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1040
Email: dmulroy@ljpc.com

La Jolla Pharmaceutical Company to Release Third Quarter 2018 Financial Results on October 24, 2018

SAN DIEGO, Oct. 18, 2018 (GLOBE NEWSWIRE) — La Jolla Pharmaceutical Company (Nasdaq: LJPC), a leader in the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases, today announced that it will release its third quarter 2018 financial results on Wednesday October 24, 2018 and will host a webcasted conference call at 4:30 p.m. Eastern Time.

Conference Call at 4:30 p.m. Eastern Time on October 24, 2018

The Company will host a conference call and webcast at 4:30 p.m. Eastern Time (1:30 p.m. Pacific Time) on October 24, 2018. The conference call can be accessed by dialing 877-359-9508 for domestic callers and 224-357-2393 for international callers. Please provide the operator with the conference ID number 5899629 to join the conference call or click here for the webcast. An archive of the conference call and webcast will be available on La Jolla’s website for 30 days following the call.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. GIAPREZA™ (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) on December 21, 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. LJPC‑401 (synthetic human hepcidin), a clinical-stage investigational product, is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information, please visit www.ljpc.com.

Company Contacts

Sandra Vedrick
Director, Investor Relations & Human Resources
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1135
Email: svedrick@ljpc.com

and

Dennis M. Mulroy
Chief Financial Officer
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1040
Email: dmulroy@ljpc.com

Media Contact

Sylvia Wheeler
Wheelhouse Life Science Advisors
Email: swheeler@wheelhouselsa.com

La Jolla Pharmaceutical Company Announces Financial Results for the Three Months Ended March 31, 2018 and Recent Corporate Progress

La Jolla Pharmaceutical Company Announces Financial Results for the Three Months Ended March 31, 2018 and Recent Corporate Progress
SAN DIEGO, May 10, 2018 (GLOBE NEWSWIRE) — La Jolla Pharmaceutical Company (Nasdaq:LJPC), a leader in the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases, today announced financial results for the three months ended March 31, 2018 and highlighted recent corporate progress.

Recent Corporate Progress

  • In May 2018, La Jolla closed a $125 million royalty financing agreement with HealthCare Royalty Partners (HCR). Under the terms of the agreement, La Jolla will receive $125 million in exchange for tiered royalty payments on worldwide net sales of GIAPREZA. Payments under the agreement start annually at a maximum royalty rate, with step-downs based on the achievement of annual net sales thresholds. Through December 31, 2021, the royalty rate will be a maximum of 10%. Starting January 1, 2022, the maximum royalty rate may increase by 4% if an agreed-upon, cumulative sales threshold has not been met, and, starting January 1, 2024, the maximum royalty rate may increase by an additional 4% if a different agreed-upon, cumulative sales threshold has not been met. The agreement is subject to maximum aggregate royalty payments to HCR of 180% of the $125 million to be received by La Jolla, at which time the payment obligations under the agreement would expire. The agreement was entered into by La Jolla’s wholly owned subsidiary, La Jolla Pharma, LLC, and HCR has no recourse under the agreement against La Jolla or any assets other than GIAPREZA.
  • In March 2018, La Jolla announced the commercial availability of GIAPREZA™ (angiotensin II) injection for intravenous infusion (formerly known as LJPC-501). In December 2017, GIAPREZA was approved by the U.S. Food and Drug Administration (FDA) to increase blood pressure in adults with septic or other distributive shock. GIAPREZA mimics the body’s endogenous regulatory peptide that is central to the renin-angiotensin-aldosterone system to increase blood pressure. Prescribing information for GIAPREZA is available at www.giapreza.com.
  • In March 2018, an analysis, entitled “Outcomes in Patients with Acute Kidney Injury Receiving Angiotensin II for Vasodilatory Shock,” was presented at the 23rd International Conference on Advances in Critical Care Nephrology AKI & CRRT 2018. The manuscript of this analysis, entitled “Outcomes in patients with vasodilatory shock and renal replacement therapy treated with intravenous angiotensin II,” was published online in Critical Care Medicine. The presentation and manuscript detail the outcomes of patients with acute kidney injury (AKI) and vasodilatory shock enrolled in the ATHOS-3 (Angiotensin II for the Treatment of High-Output Shock) Phase 3 study of GIAPREZA. In this post-hoc analysis, the data from 105 AKI patients (GIAPREZA n=45; placebo n=60) requiring renal replacement therapy (RRT) at study drug initiation were analyzed. Survival through day 28 was 53% (95% CI: 38%-67%) for the GIAPREZA group compared to 30% (95% CI: 19%-41%) for the placebo group (p = 0.012). By day 7, 38% (95% CI: 25%-54%) of patients treated with GIAPREZA discontinued RRT compared to 15% (95% CI: 8%-27%) of patients treated with placebo (p = 0.007). Mean arterial pressure (MAP) response at hour 3 was achieved in 53% (95% CI: 38%-68%) of patients treated with GIAPREZA compared to 22% (95% CI: 12%-34%) of patients treated with placebo (p = 0.001).
  • In February 2018, an abstract, entitled “Effect of Disease Severity on Survival in Patients Receiving Angiotensin II for Vasodilatory Shock,” was presented at the Society of Critical Care Medicine’s (SCCM) 47th Critical Care Congress. The abstract, which was published in the January Supplement of Critical Care Medicine, includes results from a pre-specified analysis from the ATHOS-3 Phase 3 study of GIAPREZA in patients with high severity of illness, defined as an APACHE II (Acute Physiology and Chronic Health Evaluation II) score > 30 or baseline MAP < 65 mmHg, despite treatment with high-dose vasopressors. The authors presented data showing a lower 28-day mortality rate in patients with baseline APACHE II scores > 30 in the GIAPREZA group versus the placebo group: 28-day mortality was 51.8% (n = 58) for the GIAPREZA group compared to 70.8% (n = 65) for the placebo group (hazard ratio=0.62 [95% CI: 0.39, 0.98; p=0.037]). In patients with a baseline MAP < 65 mmHg, a trend towards improved 28-day mortality was seen in the GIAPREZA group compared to the placebo group: 28-day mortality was 54.2% (n = 52) for the GIAPREZA group compared to 70.4% (n = 50) for the placebo group (hazard ratio=0.66 [95% CI: 0.40, 1.09; p=0.10]).

“This first quarter was the start of an exciting and transformational year for La Jolla, highlighted by the commercial launch of GIAPREZA,” said George Tidmarsh, M.D., Ph.D., La Jolla’s President and Chief Executive Officer. “We are excited to bring this new treatment option to the many critically ill patients suffering from septic or other distributive shock.”

Results of Operations

As of March 31, 2018, La Jolla had $154.4 million in cash and cash equivalents, compared to $90.9 million as of December 31, 2017. On a pro-forma basis, adjusting for the net proceeds from the May 2018 royalty financing, La Jolla’s cash and cash equivalents as of March 31, 2018 were $279 million. Cash used for operating activities for the three months ended March 31, 2018 was $45.9 million, compared to $22.0 million for the same period in 2017. La Jolla recognized GIAPREZA net product sales of $0.8 million for the three months ended March 31, 2018. La Jolla launched GIAPREZA in March 2018. La Jolla’s net loss for the three months ended March 31, 2018 was $50.5 million, or $2.22 per share, compared to $23.2 million, or $1.26 per share, for the same period in 2017.

About Septic or Other Distributive Shock

Over one million Americans are affected by shock on an annual basis, with one in three patients being treated for shock in the intensive care unit. Distributive shock is the most common type of shock in the inpatient setting with approximately 800,000 distributive shock cases in the U.S. per year. Of these cases, an estimated 90% are septic shock patients. Approximately 300,000 do not achieve adequate blood pressure response with standard of care vasopressor therapy (catecholamines and vasopressin). The inability to achieve or maintain adequate blood pressure results in inadequate blood flow to the body’s organs and tissue and is associated with a mortality rate exceeding most acute conditions requiring hospitalization.

About GIAPREZA

In December 2017, GIAPREZA™ (angiotensin II) was approved by the U.S. Food and Drug Administration (FDA) to increase blood pressure in adults with septic or other distributive shock. GIAPREZA mimics the body’s endogenous regulatory peptide that is central to the renin-angiotensin-aldosterone system to increase blood pressure. Prescribing information for GIAPREZA is available at www.giapreza.com.

IMPORTANT SAFETY INFORMATION

Contraindications

None

Warnings and Precautions

There is a potential for venous and arterial thrombotic and thromboembolic events in patients who receive GIAPREZA. Use concurrent venous thromboembolism (VTE) prophylaxis.

Adverse Reactions

The most common adverse reactions that were reported in greater than 10% of GIAPREZA-treated patients were thromboembolic events.

Drug Interactions

Angiotensin converting enzyme (ACE) inhibitors may increase response to GIAPREZA. Angiotensin II receptor blockers (ARB) may reduce response to GIAPREZA.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

For additional information, please see Full Prescribing Information.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. GIAPREZA™ (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) on December 21, 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. LJPC‑401 (synthetic human hepcidin), a clinical-stage investigational product, is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information, please visit www.ljpc.com.

Forward-looking Statements

This press release contains forward-looking statements, as that term is defined in the Private Securities Litigation Reform Act of 1995. These statements relate to expectations regarding future events or La Jolla’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors that may cause actual results to be materially different from those anticipated by the forward-looking statements. La Jolla cautions readers not to place undue reliance on any such forward-looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties and other factors are described in greater detail in La Jolla’s filings with the U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s website www.sec.gov. These risks include, but are not limited to, risks relating to: our ability to successfully commercialize, market and achieve market acceptance of GIAPREZA and other product candidates; potential market sizes, including for septic or other distributive shock; the success of development activities for LJPC-401 and other product candidates, including post-approval studies for GIAPREZA; the successful and timely completion of clinical trials; unforeseen safety issues from the administration of product and product candidates in patients; and other risks and uncertainties identified in our filings with the SEC. Forward-looking statements are presented as of the date of this press release, and La Jolla expressly disclaims any intent to update any forward‑looking statements to reflect the outcome of subsequent events.

LA JOLLA PHARMACEUTICAL COMPANY

Unaudited Condensed Consolidated Statements of Operations
(in thousands, except per share amounts)

Three Months Ended
March 31,
2018 2017
Revenue
Net product sales $ 809 $
Total revenue 809
Operating expenses
Cost of product sales 58
Research and development 28,429 17,765
Selling, general and administrative 23,016 5,503
Total operating expenses 51,503 23,268
Loss from operations (50,694 ) (23,268 )
Other income, net 166 28
Net loss $ (50,528 ) $ (23,240 )
Net loss per share, basic and diluted $ (2.22 ) $ (1.26 )
Weighted-average common shares outstanding, basic and diluted 22,742 18,410

LA JOLLA PHARMACEUTICAL COMPANY

Condensed Consolidated Balance Sheets
(in thousands, except share and par value amounts)

March 31,
2018
December 31,
2017
(Unaudited)
ASSETS
Current assets:
Cash and cash equivalents $ 154,408 $ 90,915
Inventory 820
Prepaid expenses and other current assets 6,326 3,147
Total current assets 161,554 94,062
Property and equipment, net 24,438 24,568
Restricted cash 909 909
Total assets $ 186,901 $ 119,539
LIABILITIES AND SHAREHOLDERS’ EQUITY
Current liabilities:
Accounts payable $ 7,321 $ 11,484
Accrued clinical and other expenses 4,280 703
Accrued payroll and related expenses 3,044 4,995
Deferred rent, current portion 1,370 1,370
Total current liabilities 16,015 18,552
Deferred rent, less current portion 13,473 12,785
Total liabilities 29,488     31,337
Shareholders’ equity:
Common Stock, $0.0001 par value; 100,000,000 shares authorized,
26,154,439 and 22,167,529 shares issued and outstanding at March 31, 2018 and December 31, 2017, respectively
3 2
Series C-12 Convertible Preferred Stock, $0.0001 par value; 11,000 shares authorized, 3,906 shares issued and outstanding at March 31, 2018 and December 31, 2017, and liquidation preference of $3,906 at March 31, 2018 and December 31, 2017 3,906 3,906
Series F Convertible Preferred Stock, $0.0001 par value; 10,000 shares authorized,
2,737 shares issued and outstanding at March 31, 2018 and December 31, 2017, and liquidation preference of $2,737 at March 31, 2018 and December 31, 2017
2,737 2,737
Additional paid-in capital 922,809 803,071
Accumulated deficit (772,042 ) (721,514 )
Total shareholders’ equity 157,413 88,202
Total liabilities and shareholders’ equity $ 186,901 $ 119,539

Company Contacts

Sandra Vedrick
Director, Investor Relations & Human Resources
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1135
Email: svedrick@ljpc.com

and

Dennis M. Mulroy
Chief Financial Officer
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1040
Email: dmulroy@ljpc.com

GIAPREZA™ (angiotensin II) Significantly Improved Survival and Reduced Time on Renal Replacement Therapy in Patients with Acute Kidney Injury

Analyses to Be Presented in Oral and Poster Sessions at The 23rd International Conference on Advances in Critical Care Nephrology AKI & CRRT 2018

Manuscript Published Online in Critical Care Medicine

SAN DIEGO, March 06, 2018 (GLOBE NEWSWIRE) — La Jolla Pharmaceutical Company (Nasdaq:LJPC) today announced the release of data from analyses of the impact of GIAPREZA™ (angiotensin II) on outcomes of a subset of patients with acute kidney injury requiring renal replacement therapy (AKI-RRT) enrolled in the ATHOS-3 (Angiotensin II for the Treatment of High Output Shock) study.

The presentation, entitled “Outcomes in Patients with Acute Kidney Injury Receiving Angiotensin II for Vasodilatory Shock,” will take place during The 23rd International Conference on Advances in Critical Care Nephrology – AKI & CRRT 2018, being held March 6-9, 2018 in San Diego, California. The manuscript, entitled “Outcomes in patients with vasodilatory shock and renal replacement therapy treated with intravenous angiotensin II” was published online in Critical Care Medicine.

“Acute kidney injury requiring dialysis associated with distributive shock, also referred to as vasodilatory shock, represents a significant medical risk for patients and a significant financial burden to the health care system,” said James Tumlin M.D., Professor of Medicine and Director of NephroNet Clinical Trials Consortium. “These analyses of the effect of angiotensin II on AKI patients requiring dialysis in the ATHOS-3 study demonstrated angiotensin II is a promising therapy to address this unmet need.”

The presentations and manuscript detail the outcomes of patients with acute kidney injury (AKI) and vasodilatory shock enrolled in the ATHOS-3 study of GIAPREZA. In this post-hoc analysis, the data from 105 AKI patients (GIAPREZA n=45; placebo n=60) requiring renal replacement therapy (RRT) at study drug initiation were analyzed. Survival through day 28 was 53% (95% CI: 38%-67%) for the GIAPREZA group compared to 30% (95% CI: 19%-41%) for the placebo group (p = 0.012). By day 7, 38% (95% CI: 25%-54%) of patients treated with GIAPREZA discontinued RRT compared to 15% (95% CI: 8%-27%) of patients treated with placebo (p = 0.007). Mean arterial pressure (MAP) response at hour 3 was achieved in 53% (95% CI: 38%-68%) of patients treated with GIAPREZA compared to 22% (95% CI: 12%-34%) of patients treated with placebo (p = 0.001).

23rd International Conference on Advances in Critical Care Nephrology – AKI & CRRT 2018 Presentation Details
Presentation Title: Outcomes in Patients with Acute Kidney Injury Receiving Angiotensin II for Vasodilatory Shock
Presenter: James Tumlin, M.D., Professor of Medicine and Director of NephroNet Clinical Trials Consortium
Poster Presentation Date: Tuesday, March 6, 2018 5:30 pm – 7:00 pm and Wednesday, March 7, 2018 6:00 pm – 8:00 pm Pacific Time
Oral Presentation Date: Friday, March 9, 2018 11:00 am – 12:25 pm Pacific Time
Location: Manchester Grand Hyatt, San Diego, CA

About GIAPREZA

In December 2017, GIAPREZA™ (angiotensin II) was approved by the U.S. Food and Drug Administration (FDA) to increase blood pressure in adults with septic or other distributive shock. GIAPREZA mimics the body’s endogenous regulatory peptide that is central to the renin-angiotensin-aldosterone system to increase blood pressure. Prescribing information for GIAPREZA is available at www.giapreza.com.

IMPORTANT SAFETY INFORMATION

Contraindications

None

Warnings and Precautions

There is a potential for venous and arterial thrombotic and thromboembolic events in patients who receive GIAPREZA. Use concurrent venous thromboembolism (VTE) prophylaxis.

Adverse Reactions

The most common adverse reactions reported in greater than 10% in GIAPREZA treated patients were thromboembolic events.

Drug Interactions

Angiotensin converting enzyme (ACE) inhibitors may increase response to GIAPREZA.
Angiotensin II receptor blockers (ARB) may reduce response to GIAPREZA.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

For additional information, please see Full Prescribing Information.

About Shock and Septic or Other Distributive Shock

Over 1 million Americans are affected by shock on an annual basis, with 1 in 3 patients being treated for shock in the intensive care unit. Distributive shock is the most common type of shock in the inpatient setting with approximately 800,000 distributive shock cases in the United States per year. Of these cases, an estimated 90% are septic shock patients. Approximately 300,000 do not achieve adequate blood pressure response with standard of care vasopressor therapy (catecholamines and vasopressin). The inability to achieve or maintain adequate blood pressure results in inadequate blood flow to the body’s organs and tissue and is associated with a mortality rate exceeding most acute conditions requiring hospitalization.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. GIAPREZA™ (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) on December 21, 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. LJPC‑401 (synthetic human hepcidin), a clinical-stage investigational product, is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information, please visit www.ljpc.com.

Forward-looking Statements

This press release contains forward-looking statements, as that term is defined in the Private Securities Litigation Reform Act of 1995. These statements relate to expectations regarding future events or La Jolla’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors that may cause actual results to be materially different from those anticipated by the forward-looking statements. La Jolla cautions readers not to place undue reliance on any such forward-looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties and other factors are described in greater detail in La Jolla’s filings with the U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s website www.sec.gov. These risks include, but are not limited to, risks relating to: unforeseen safety issues from the administration of GIAPREZA in patients; the anticipated treatment of future clinical data by the FDA, the EMA or other regulatory authorities; potential market sizes, including for septic or other distributive shock; our ability to successfully commercialize, market and achieve market acceptance of GIAPREZA; and other risks and uncertainties identified in our filings with the SEC. La Jolla expressly disclaims any intent to update any forward‑looking statements to reflect the outcome of subsequent events.

Company Contacts

Sandra Vedrick
Director, Investor Relations & Human Resources
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1135
Email: svedrick@ljpc.com

and

Dennis M. Mulroy
Chief Financial Officer
La Jolla Pharmaceutical Company
Phone: (858) 207-4264 Ext: 1040
Email: dmulroy@ljpc.com

La Jolla Pharmaceutical Company Announces Financial Results for the Three and Twelve Months Ended December 31, 2017 and Recent Corporate Progress

SAN DIEGO, Calif., Feb. 22, 2018 (GLOBE NEWSWIRE) — La Jolla Pharmaceutical Company (Nasdaq:LJPC), a leader in the development of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases, today announced financial results for the three and twelve months ended December 31, 2017 and highlighted recent corporate progress.

Recent Corporate Progress

  • In December 2017, GIAPREZA™ (angiotensin II), injection for intravenous infusion, formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) as a vasoconstrictor indicated to increase blood pressure in adults with septic or other distributive shock.
  • In December 2017, La Jolla announced the initiation of LJ401-HH01, a multinational, multicenter, randomized, placebo-controlled, double-blind, Phase 2 study that is designed to evaluate the safety and efficacy of LJPC-401 (synthetic human hepcidin) as a treatment for hereditary hemochromatosis (HH). Approximately 60 patients in 5 countries will be randomized to receive weekly subcutaneous injections of either LJPC-401 or placebo for 12 weeks. The primary efficacy endpoint of the study is the change in transferrin saturation, a standard measurement of iron levels in the body and one of the two key measurements used to detect iron overload, from baseline to end of treatment. Secondary efficacy endpoints include: (i) the change in serum ferritin, the other key measurement used to detect iron overload, from baseline to end of treatment; and (ii) the requirement for and frequency of phlebotomy procedures used during the study.
  • In December 2017, La Jolla announced the initiation of LJ401-BT01, a pivotal, multinational, multicenter, randomized, controlled study of LJPC-401 in patients with transfusion-dependent beta thalassemia who, despite chelation therapy, have cardiac iron levels above normal. Approximately 100 patients in 9 countries, including the U.S., will be randomized 1:1 to receive either: (i) weekly subcutaneous injections of LJPC-401, while continuing standard-of-care chelation therapy (LJPC-401 treatment arm); or (ii) a continuation of standard-of-care chelation therapy only (observation arm). After 6 months of treatment, patients randomized to the observation arm will cross over to receive LJPC-401 (plus standard-of-care chelation therapy) for 6 months, while patients randomized to the LJPC-401 treatment arm will continue with LJPC-401 (plus standard-of-care chelation therapy) for an additional 6 months (for a total of one year). The primary efficacy endpoint of this study is the change in iron content in the heart after 6 months, as measured by cardiac magnetic resonance imaging (MRI). If this study is successful, we would anticipate filing a market authorization application (MAA) for LJPC-401 in Europe.

“2017 was an exciting year for La Jolla, highlighted by the FDA’s approval of GIAPREZA,” said George Tidmarsh, M.D., Ph.D., La Jolla’s President and Chief Executive Officer. “We are excited to bring this new treatment option to the many critically ill patients suffering from septic or other distributive shock.”

Results of Operations

As of December 31, 2017, La Jolla had $90.9 million in cash and cash equivalents, compared to $65.7 as of December 31, 2016. La Jolla’s net cash used for operating activities for the twelve months ended December 31, 2017 was $84.9 million compared to net cash used for operating activities of $58.7 million for the same period in 2016. La Jolla’s net loss for the three and twelve months ended December 31, 2017 was $38.5 million and $114.8 million, or $1.74 per share and $5.41 per share, respectively, compared to a net loss of $24.9 million and $78.2 million, or $1.44 per share and $4.54 per share, respectively, for the same periods in 2016.

About GIAPREZA

In December 2017, GIAPREZA™ (angiotensin II) was approved by the U.S. Food and Drug Administration (FDA) to increase blood pressure in adults with septic or other distributive shock. GIAPREZA mimics the body’s endogenous regulatory peptide that is central to the renin-angiotensin-aldosterone system to increase blood pressure. In the ATHOS-3 Phase 3 study, the primary endpoint of: (i) a mean arterial pressure (MAP) increase of ≥ 10 mmHg; or (ii) a MAP of ≥ 75 mmHg, was achieved by 70% of patients randomized to GIAPREZA, compared to 23% of patients randomized to placebo (p < 0.0001); both arms were treated with standard-of-care vasopressors. The recommended starting dosage of GIAPREZA is 20 nanograms (ng)/kg/min via continuous intravenous infusion. Close monitoring during the first 5 minutes of GIAPREZA initiation is recommended. GIAPREZA is available in 1 mL single dose vials, each containing 2.5 mg of angiotensin II (as a sterile liquid). Prescribing information for GIAPREZA is available at www.giapreza.com.

IMPORTANT SAFETY INFORMATION

Warnings and Precautions

The safety of GIAPREZA was evaluated in 321 adults with septic or other distributive shock in the randomized, double-blind, placebo-controlled Phase 3 study, ATHOS-3. There was a higher incidence of venous and arterial thrombotic and thromboembolic events in patients who received GIAPREZA compared to placebo-treated patients in the ATHOS-3 study [13% (21/163 patients) vs. 5% (8/158 patients)]. The major imbalance was in deep venous thromboses. Use concurrent venous thromboembolism prophylaxis.

Adverse Reactions

Adverse reactions that occurred in ≥4% of patients treated with GIAPREZA and ≥1.5% more often than placebo-treated patients in the ATHOS-3 study were thromboembolic events (including deep vein thrombosis), thrombocytopenia, tachycardia, fungal infection, delirium, acidosis, hyperglycemia and peripheral ischemia.

Drug Interactions

Angiotensin converting enzyme (ACE) inhibitors may increase response to GIAPREZA. Angiotensin II receptor blockers (ARB) may reduce response to GIAPREZA.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

For additional information, please see Full Prescribing Information.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. GIAPREZA™ (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration (FDA) on December 21, 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. LJPC‑401 (synthetic human hepcidin), a clinical-stage investigational product, is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information, please visit www.ljpc.com.

Forward-looking Statements

This press release contains forward-looking statements, as that term is defined in the Private Securities Litigation Reform Act of 1995. These statements relate to expectations regarding future events or La Jolla’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors that may cause actual results to be materially different from those anticipated by the forward-looking statements. La Jolla cautions readers not to place undue reliance on any such forward-looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties and other factors are described in greater detail in La Jolla’s filings with the U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s website www.sec.gov. These risks include, but are not limited to, risks relating to: our ability to successfully commercialize, market and achieve market acceptance of GIAPREZA and other product candidates; potential market sizes, including for septic or other distributive shock; the success of development activities for LJPC-401 and other product candidates; and other risks and uncertainties identified in our filings with the SEC. Forward-looking statements are presented as of the date of this press release, and La Jolla expressly disclaims any intent to update any forward‑looking statements to reflect the outcome of subsequent events.

Company Contacts

Sandra Vedrick

Director, Investor Relations & Human Resources

La Jolla Pharmaceutical Company

Phone: 858 207 4264 Ext: 1135

Email: svedrick@ljpc.com

and

Dennis M. Mulroy

Chief Financial Officer

La Jolla Pharmaceutical Company

Phone: 858 207 4264 Ext: 1040

Email: dmulroy@ljpc.com

La Jolla Pharmaceutical Company Announces FDA Approval of Giapreza™ (angiotensin II)

Giapreza increases blood pressure in adults with septic or other distributive shock

La Jolla to host conference call and webcast at 8:30 a.m. EST on December 22, 2017

SAN DIEGO, Dec. 21, 2017 (GLOBE NEWSWIRE) — La Jolla Pharmaceutical Company (Nasdaq:LJPC) (the Company or La Jolla) today announced that the U.S. Food and Drug Administration (FDA) has approved GiaprezaTM (angiotensin II) to increase blood pressure in adults with septic or other distributive shock.

“We appreciate FDA’s rapid review and approval of Giapreza and are especially grateful to the patients, families and dedicated critical care teams who made the development of Giapreza possible,” said George F. Tidmarsh, M.D., Ph.D., President and Chief Executive Officer of La Jolla. “We look forward to bringing this new treatment option to the many critically ill patients suffering from septic or other distributive shock.”

“Vasopressors are critical to treat patients with shock. The critical care community now has another tool to use,” said John A. Kellum, M.D., Director of Center for Critical Care Nephrology, Vice Chair for Research, and Professor of Critical Care Medicine, University of Pittsburgh. “The approval of angiotensin II represents a major advance in the treatment of patients with septic or distributive shock.”

La Jolla plans to make Giapreza available for patients in the U.S. in March 2018. Prescribing information for Giapreza is available at www.giapreza.com.

About Septic or Other Distributive Shock

Distributive shock is the most common type of shock in the inpatient setting, affecting approximately one-third of intensive care unit patients. There are approximately 800,000 distributive shock cases in the United States per year. Of these cases, an estimated 90% are septic shock patients. Approximately 300,000 do not achieve adequate blood pressure response with current standard therapy. The inability to achieve or maintain adequate blood pressure results in inadequate blood flow to the body’s organs and tissue and is associated with a mortality rate exceeding most acute conditions requiring hospitalization.

Conference Call at 8:30 a.m. Eastern Time on December 22, 2017

The Company will host a conference call and webcast at 8:30 a.m. Eastern Time (5:30 a.m. Pacific Time) on December 22, 2017. The conference call can be accessed by dialing 877‑359‑9508 for domestic callers and 224‑357‑2393 for international callers. Please provide the operator with the passcode 7979625 to join the conference call or click here for the webcast. A slide presentation accompanying today’s press release and the conference call may also be found on La Jolla’s website at www.ljpc.com thirty minutes prior to the call under the investor relations section. An archive of the conference call and webcast will be available on La Jolla’s website for 30 days following the call.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. Giapreza (angiotensin II), formerly known as LJPC-501, was approved by the U.S. Food and Drug Administration in December 2017 as a vasoconstrictor to increase blood pressure in adults with septic or other distributive shock. La Jolla plans to make Giapreza available for patients in the U.S. starting in March 2018. LJPC‑401 (synthetic human hepcidin) is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information on La Jolla, please visit www.ljpc.com.

Forward Looking Statement Safe Harbor

This press release contains forward-looking statements as that term is defined in the Private Securities Litigation Reform Act of 1995. These statements relate to expectations regarding future events or La Jolla’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors that may cause actual results to be materially different from those anticipated by the forward-looking statements. La Jolla cautions readers not to place undue reliance on any such forward-looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties and other factors are described in greater detail in La Jolla’s filings with the U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s website www.sec.gov. These risks include, but are not limited to, risks relating to: the timing for commercial launch of Giapreza (angiotensin II); the degree of physician or pharmacy and therapeutics committee adoption of Giapreza and La Jolla’s success in commercializing Giapreza; the timing and availability of Giapreza in the market; the anticipated treatment of future clinical data by the FDA, the EMA or other regulatory authorities, including whether such data will be sufficient for approval of Giapreza in the EMA and for approval of other product candidates by either the FDA or EMA; risks relating to the scope of the Giapreza product label; potential market sizes, including for septic or other distributive shock; potential indications for which La Jolla’s products and product candidates may be developed; the anticipated timing for regulatory actions; the timing, costs, conduct and outcome of clinical studies; the impact of pharmaceutical industry regulation and healthcare legislation in the United States; and the success of future development activities. La Jolla expressly disclaims any intent to update any forward‑looking statements to reflect the outcome of subsequent events.

Company Contacts

Sandra Vedrick

Director, Investor Relations & Human Resources

La Jolla Pharmaceutical Company

Phone: 858 207 4264 Ext: 1135

Email: svedrick@ljpc.com

and

Dennis M. Mulroy

Chief Financial Officer

La Jolla Pharmaceutical Company

Phone: 858 207 4264 Ext: 1040

Email: dmulroy@ljpc.com

La Jolla Pharmaceutical Company Announces Initiation of Multicenter, Randomized, Phase 2 Clinical Study of LJPC-401 in Patients with Hereditary Hemochromatosis

SAN DIEGO, CA – December 18, 2017 – La Jolla Pharmaceutical Company (NASDAQ: LJPC) (the Company or La Jolla) today announced the initiation of LJ401-HH01, a Phase 2 clinical study of LJPC-401 (synthetic human hepcidin) in patients with hereditary hemochromatosis (HH). The most common genetic disease in Caucasians, HH is a disease characterized by a genetic mutation that results in a deficiency in the production of hepcidin, which is the body’s naturally occurring regulator of iron absorption and distribution. Without normal levels of hepcidin, excessive amounts of iron accumulate in the body. Symptoms of the disease include joint pain, abdominal pain, fatigue and weakness. If left untreated, HH can lead to liver cirrhosis, liver cancer, heart disease and/or failure and diabetes.

LJ401-HH01 is a multinational, multicenter, randomized, placebo-controlled, double-blind, Phase 2 study that is designed to evaluate the safety and efficacy of LJPC-401 as a treatment for HH. Approximately 60 patients will be randomized to receive weekly subcutaneous injections of either LJPC-401 or placebo for 12 weeks. The primary efficacy endpoint of the study is the change in transferrin saturation, a standard measurement of iron levels in the body and one of the two key measurements used to detect iron overload, from baseline to end of treatment. Secondary efficacy endpoints include: (i) the change in serum ferritin, the other key measurement used to detect iron overload, from baseline to end of treatment; and (ii) the requirement for and frequency of phlebotomy procedures during the study.

“There is a major need for new treatment modalities that improve the quality of life of the many patients suffering from hereditary hemochromatosis,” stated Jeff Vacirca, M.D., Chief of Clinical Research at New York Cancer & Blood Specialists and investigator in the study. “I am excited to be included in the clinical evaluation of a treatment that harnesses the body’s natural mechanism for iron regulation in a patient-friendly treatment regimen that could potentially reduce or eliminate the need for phlebotomy procedures in these patients.”

“Following the results of Phase 1 clinical studies that demonstrated that the administration of LJPC-401 resulted in dose-dependent reductions in iron levels, we are pleased to initiate LJ401- HH01 to evaluate the therapeutic potential of LJPC-401 in the important patient population of hereditary hemochromatosis,” said George F. Tidmarsh, M.D., Ph.D., President and Chief Executive Officer of La Jolla. “With the recent initiation of LJ401-BT01, our pivotal study of LJPC-401 in beta thalassemia patients, LJPC-401 now is being evaluated in two, multicenter, randomized, controlled clinical studies. These studies are important components of our overall development program for LJPC-401, which is aimed at helping the many patients suffering from the effects of iron overload due to a variety of underlying causes.”

The current standard treatment for HH is a blood removal procedure known as phlebotomy. Each phlebotomy procedure, which is usually conducted at a hospital, medical office or blood center, typically involves the removal of approximately a pint of blood. The required frequency of procedures varies by patient, but often ranges from one to two times per week for an initial period after diagnosis and once every one to three months chronically. Since most of the body’s iron is stored in red blood cells, chronic removal of blood can effectively lower iron levels if a phlebotomy regimen is adhered to. However, phlebotomy procedures may cause and may be associated with pain, bruising and scarring at the venous puncture site, fatigue and dizziness during and following the procedure and disruption of daily activities. Furthermore, phlebotomy is not appropriate in patients with poor venous access, anemia or heart disease.

About LJPC-401

La Jolla is developing LJPC-401 (synthetic human hepcidin) for the potential treatment of iron overload, which occurs as a result of diseases such as hereditary hemochromatosis (HH), beta thalassemia, sickle cell disease (SCD) and myelodysplastic syndrome (MDS). Hepcidin, an endogenous peptide hormone, is the body’s naturally occurring regulator of iron absorption and distribution. In healthy individuals, hepcidin prevents excessive iron accumulation in vital organs, such as the liver and heart, where it can cause significant damage and even result in death. The European Medicines Agency (EMA) Committee for Orphan Medicinal Products (COMP) has designated LJPC-401 as an orphan medicinal product for the treatment of beta thalassemia intermedia and major and SCD.

In September 2016, La Jolla reported positive results from a Phase 1 study of LJPC-401 in patients at risk of iron overload suffering from hereditary hemochromatosis, thalassemia and SCD. Single, escalating doses of LJPC-401 were associated with a dose-dependent, statistically significant reduction in serum iron. LJPC-401 was well-tolerated with no dose-limiting toxicities. Injection-site reactions were the most commonly reported adverse event and were all mild or moderate in severity, self-limiting and fully resolved.

In December 2017, La Jolla announced the initiation of a pivotal, multinational, multicenter, randomized, controlled study of LJPC-401 in patients with transfusion-dependent beta thalassemia who, despite chelation therapy, have cardiac iron levels above normal. La Jolla had previously announced that it had reached agreement with the European Medicines Agency (EMA) on the design of this registration study of LJPC-401.

About Hereditary Hemochromatosis

Hereditary hemochromatosis (HH) is the most common genetic disease in Caucasians. HH is a disease characterized by a genetic mutation that results in a deficiency in the production of hepcidin, which is the body’s naturally occurring regulator of iron absorption and distribution. Without normal levels of hepcidin, excessive amounts of iron accumulate in the body. Symptoms of the disease include joint pain, abdominal pain, fatigue and weakness. If left untreated, HH can lead to liver cirrhosis, liver cancer, heart disease and/or failure and diabetes.

The current standard treatment for HH is a blood removal procedure known as phlebotomy. Each phlebotomy procedure, which is usually conducted at a hospital, medical office or blood center, typically involves the removal of approximately a pint of blood. The required frequency of procedures varies by patient, but often ranges from one to two times per week for an initial period after diagnosis and once every one to three months chronically. Since most of the body’s iron is stored in red blood cells, chronic removal of blood can effectively lower iron levels if a phlebotomy regimen is adhered to. However, phlebotomy procedures may cause and may be associated with pain, bruising and scarring at the venous puncture site, fatigue and dizziness during and following the procedure and disruption of daily activities. Furthermore, phlebotomy is not appropriate in patients with poor venous access, anemia or heart disease.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. The Company has several product candidates in development. LJPC-501 (synthetic human angiotensin II) is being developed for the potential treatment of hypotension in adult patients with distributive or vasodilatory shock who remain hypotensive despite fluid and vasopressor therapy. LJPC-401 (synthetic human hepcidin) is being developed for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. For more information on La Jolla, please visit www.ljpc.com.

Forward Looking Statement Safe Harbor

This press release contains forward-looking statements as that term is defined in the Private Securities Litigation Reform Act of 1995. These statements relate to future events or the Company’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors, that may cause actual results to be materially different from those anticipated by the forward-looking statements. The Company cautions readers not to place undue reliance on any such forward- looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties and other factors are described in greater detail in the Company’s filings with the U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s website www.sec.gov. These risks include, but are not limited to, risks relating to: the timing, costs, conduct and outcome of clinical studies; the anticipated treatment of future clinical data by the FDA, the EMA or other regulatory authorities, including whether such data will be sufficient for approval; the timing and prospects for approval of LJPC-501 or LJPC-401 by the FDA, the EMA or other regulatory authorities; risks relating to the scope of product labels (if approved); potential market sizes; the success of future development activities; potential indications for which the Company’s product candidates may be developed; the anticipated timing for regulatory actions; the impact of pharmaceutical industry regulation and healthcare legislation in the United States; and the success of future development activities. The Company expressly disclaims any intent to update any forward-looking statements to reflect the outcome of subsequent events.

Company Contacts

Sandra Vedrick

Associate Director, Investor Relations & Human Resources

La Jolla Pharmaceutical Company Phone: 858 207 4264 Ext: 1135 Email: svedrick@ljpc.com

and

Dennis M. Mulroy

Chief Financial Officer

La Jolla Pharmaceutical Company Phone: 858 207 4264 Ext: 1040 Email: dmulroy@ljpc.com

La Jolla Pharmaceutical Company Announces Intent to Submit Marketing Authorization Application for LJPC-501 in the Third Quarter of 2018

— Decision follows successful Scientific Advice meeting with European Medicines Agency —

 

SAN DIEGO, CA – September 25, 2017 – La Jolla Pharmaceutical Company (NASDAQ: LJPC) (the Company or La Jolla) today announced that the European Medicines Agency’s (EMA) Committee for Medicinal Products for Human Use (CHMP) has issued favorable Scientific Advice regarding the EU regulatory pathway for LJPC-501 (angiotensin II) for the treatment of hypotension in adults with distributive or vasodilatory shock who remain hypotensive despite fluid and vasopressor therapy. Based on this Advice, La Jolla intends to submit a Marketing Authorization Application (MAA) for LJPC-501 in the third quarter of 2018.

About LJPC-501

LJPC-501 is La Jolla’s proprietary formulation of synthetic human angiotensin II. Angiotensin II is a major bioactive component of the renin-angiotensin-aldosterone system (RAAS). The

RAAS system is one of three central regulators of blood pressure. LJPC-501 is a vasopressor that leverages the RAAS system. LJPC-501 is being developed for the treatment of patients with distributive or vasodilatory shock who remain hypotensive despite fluid and vasopressor therapy (catecholamines and/or vasopressin).

In August 2017, the Company announced that its New Drug Application (NDA) for LJPC-501 had been accepted for review by the U.S. Food and Drug Administration (FDA). The review classification for the application is Priority, and the user fee goal date under the Prescription Drug User Fee Act (PDUFA) is February 28, 2018. The NDA is based on results of the ATHOS- 3 (Angiotensin II for the Treatment of High Output Shock) multicenter, randomized, double- blind, placebo-controlled, Phase 3 clinical study of LJPC-501 in patients with distributive or vasodilatory shock who remain hypotensive despite fluid and vasopressor therapy. The study was conducted under a Special Protocol Assessment (SPA) agreed to with the FDA in 2015. A total of 344 patients were randomized across nine countries, 321 of whom received study treatment and were included in the primary analysis. In May 2017 the results of the ATHOS-3 study were published by The New England Journal of Medicine in an article entitled “Angiotensin II for the Treatment of Vasodilatory Shock”.

About Patients with Distributive or Vasodilatory Shock Failing Standard Therapy

Distributive or vasodilatory shock (dangerously low blood pressure with adequate cardiac function) can become life-threatening when a patient is unable to achieve or maintain target mean arterial pressure (MAP) despite treatment with the currently available standard of care (fluids and vasopressors). This life-threatening syndrome has been described as clinically refractory hypotension, catecholamine resistant hypotension, high-dose vasopressor-dependent shock, catecholamine or vasopressor refractory shock, or catecholamine-resistant vasodilatory shock. There are approximately 500,000 distributive or vasodilatory shock patients in the United States per year with an estimated 200,000 patients failing standard therapy. Approximately 50% of these patients die within 30 days.

About La Jolla Pharmaceutical Company

La Jolla Pharmaceutical Company is a biopharmaceutical company focused on the discovery, development and commercialization of innovative therapies intended to significantly improve outcomes in patients suffering from life-threatening diseases. The Company has several product candidates in development. LJPC-501 is La Jolla’s proprietary formulation of synthetic human angiotensin II for the treatment of hypotension in adult patients with distributive or vasodilatory shock who remain hypotensive despite fluid and vasopressor therapy. LJPC-401 is La Jolla’s proprietary formulation of synthetic human hepcidin for the potential treatment of conditions characterized by iron overload, such as hereditary hemochromatosis, beta thalassemia, sickle cell disease and myelodysplastic syndrome. LJPC-30S is La Jolla’s next-generation gentamicin derivative program that is focused on the potential treatment of serious bacterial infections as well as rare genetic disorders, such as cystic fibrosis and Duchenne muscular dystrophy. For more information on La Jolla, please visit www.ljpc.com.

Forward Looking Statement Safe Harbor

This press release contains forward-looking statements as that term is defined in the Private Securities Litigation Reform Act of 1995. These statements relate to future events or the Company’s future results of operations. These statements are only predictions or statements of current expectations and involve known and unknown risks, uncertainties and other factors, that may cause actual results to be materially different from those anticipated by the forward-looking statements. The Company cautions readers not to place undue reliance on any such forward- looking statements, which speak only as of the date they were made. Certain of these risks, uncertainties, and other factors are described in greater detail in the Company’s filings with the

U.S. Securities and Exchange Commission (SEC), all of which are available free of charge on the SEC’s web site www.sec.gov. These risks include, but are not limited to, risks relating to: the timing and prospects for approval of LJPC-501 by the FDA, EMA and other regulatory authorities; risks relating to the scope of product labels (if approved); potential market sizes; the anticipated timing for regulatory actions; the impact of pharmaceutical industry regulation and health care legislation in the Europe and the United States; and the success of future development activities. The Company expressly disclaims any intent to update any

forward-looking statements to reflect the outcome of subsequent events.

Company Contacts

Sandra Vedrick

Associate Director, Investor Relations & Human Resources

La Jolla Pharmaceutical Company Phone: 858-256-7910

Email: svedrick@ljpc.com

and

Dennis M. Mulroy

Chief Financial Officer

La Jolla Pharmaceutical Company Phone: 858-433-6839

Email: dmulroy@ljpc.com

Media Contact

Matt Middleman, M.D. LifeSci Public Relations Phone: 646-627-8384

Email: matt.middleman@lifescipublicrelations.com