FDA approves the first treatment option for generalized pustular psoriasis flares in adults

• More than half of patients treated with SPEVIGO^® (spesolimab-sbzo) injection, for intravenous use showed no visible pustules one week after receiving treatment
• Spesolimab is a monoclonal antibody that inhibits interleukin-36 (IL-36) signaling

RIDGEFIELD, Conn., Sept. 1, 2022 /PRNewswire/ -- Boehringer Ingelheim announced today the U.S. Food and Drug Administration has approved SPEVIGO, the first approved treatment option for generalized pustular psoriasis (GPP) flares in adults. SPEVIGO is a novel, selective antibody that blocks the activation of the interleukin-36 receptor (IL-36R), a key part of a signaling pathway within the immune system shown to be involved in the cause of GPP.

"GPP flares can greatly impact a patient's life and lead to serious, life-threatening complications," said Mark Lebwohl, M.D., lead investigator and publication author, and Dean for Clinical Therapeutics, Icahn School of Medicine at Mount Sinai, Kimberly and Eric J. Waldman Department of Dermatology, New York. "The approval of SPEVIGO is a turning point for dermatologists and clinicians. We now have an FDA-approved treatment that may help make a difference for our patients who, until now, have not had any approved options to help manage GPP flares."

Distinct from plaque psoriasis, GPP is a rare and potentially life-threatening neutrophilic skin disease, which is characterized by flares (episodes of widespread eruptions of painful, sterile pustules). In the United States, it is estimated that 1 out of every 10,000 people has GPP. Given that it is so rare, recognizing the signs and symptoms can be challenging and consequently lead to delays in diagnosis.

"This important approval reflects our successful efforts to accelerate our research with the aim to bring innovative treatments faster to the people most in need," said Carinne Brouillon, Member of the Board of Managing Directors, responsible for Human Pharma, Boehringer Ingelheim. "We recognize how devastating this rare skin disease can be for patients, their families and caregivers. GPP can be life-threatening and until today there have been no specific approved therapies for treating the devastating GPP flares. It makes me proud that with the approval of SPEVIGO we can now offer the first U.S. approved treatment option for those in need." 

In the 12-week pivotal Effisayiltm 1 clinical trial, patients experiencing a GPP flare (N=53) were treated with SPEVIGO or placebo. After one week, patients treated with SPEVIGO showed no visible pustules (54%) compared to placebo (6%). 

In Effisayiltm 1, the most common adverse reactions (?5%) in patients that received SPEVIGO were asthenia and fatigue, nausea and vomiting, headache, pruritus and prurigo, infusion site hematoma and bruising, and urinary tract infection.

"GPP can have an enormous impact on patients' physical and emotional wellbeing. With the FDA approval of this new treatment, people living with GPP now have hope in knowing that there is an option to help treat their flares," said Thomas Seck, M.D., Senior Vice President, Medicine and Regulatory Affairs, Boehringer Ingelheim. "SPEVIGO represents Boehringer Ingelheim's commitment to delivering meaningful change for patients living with serious diseases with limited treatment options."

About SPEVIGO
SPEVIGO is indicated for the treatment of GPP flares in adults. SPEVIGO is contraindicated in patients with severe or life-threatening hypersensitivity to spesolimab-sbzo or to any of the excipients in SPEVIGO. Reactions have included drug reaction with eosinophilia and systemic symptoms (DRESS).

What is SPEVIGO? 
SPEVIGO is a prescription medicine used to treat generalized pustular psoriasis (GPP) flares in adults. It is not known if SPEVIGO is safe and effective in children.

Important Safety Information

Do not receive SPEVIGO if you have had a severe or life-threatening allergic reaction to spesolimab-sbzo or any of the ingredients in SPEVIGO.

What is the most important information I should know about SPEVIGO?

SPEVIGO may cause serious side effects, including:

• Infections. SPEVIGO may lower the ability of your immune system to fight infections and may increase your risk of infections. Your healthcare provider should check you for infections and tuberculosis (TB) before starting treatment with SPEVIGO and may treat you for TB before you begin treatment with SPEVIGO if you have a history of TB or have active TB. Your healthcare provider should watch you closely for signs and symptoms of TB after treatment with SPEVIGO. Tell your healthcare provider right away if you have an infection or have symptoms of an infection, including:
• fever, chills or sweats
• muscle aches
• cough
• shortness of breath
• blood in your phlegm (mucus)
• burning when you urinate
• urinating more often than normal
• Allergic reactions and infusion-related reactions. Serious allergic reactions may happen during or after your infusion of SPEVIGO. If you have a serious allergic reaction, your healthcare provider will stop treatment with SPEVIGO. If you have an infusion-related reaction, your healthcare provider will stop your SPEVIGO infusion and treat your symptoms and may restart SPEVIGO at a slower infusion rate. Tell your healthcare provider or get emergency medical help right away if you get any of the following symptoms during or after your infusion of SPEVIGO:
• feeling faint, dizzy, or lightheaded
• swelling of your face, eyelids, lips, mouth, tongue, or throat
• trouble breathing or throat tightness
• fever
• mouth sores
• chest tightness
• hives or skin rash that is different than the rash from generalized pustular psoriasis (GPP)
• itching
• swollen lymph nodes

Before you receive SPEVIGO, tell your healthcare provider about all of your medical conditions, including if you:

• have an infection that does not go away or that keeps coming back.
• have TB or have been in close contact with someone with TB.
• have recently received or are scheduled to receive an immunization (vaccine). You should not receive live vaccines after treatment with SPEVIGO.
• are pregnant or plan to become pregnant. It is not known if SPEVIGO can harm your unborn baby.
• are breastfeeding or plan to breastfeed. It is not known if SPEVIGO passes into your breast milk. Talk to your healthcare provider about the best way to feed your baby during treatment with SPEVIGO.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

What are the most common side effects of SPEVIGO:

• feeling tired or weak
• nausea and vomiting
• headache
• itching or itchy bumps
• a collection of blood under the skin at the infusion site or bruising
• urinary tract infection

These are not all of the possible side effects of SPEVIGO. Call your doctor for medical advice about side effects.

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 more information, please see Prescribing Information and Medication Guide.

CL-SPG-100001 09.01.2022

About generalized pustular psoriasis (GPP)
GPP is a rare, heterogenous and potentially life-threatening neutrophilic skin disease, which is clinically distinct from plaque psoriasis. GPP is caused by neutrophils (a type of white blood cell) accumulating in the skin, resulting in painful, sterile pustules all over the body. The clinical course varies, with some patients having a relapsing disease with recurrent flares, and others having a persistent disease with intermittent flares. While the severity of GPP flares can vary, if left untreated they can be life-threatening due to complications such as sepsis and multisystem organ failure. This chronic, systemic disease has a substantial quality of life impact for patients and increased healthcare burden. GPP has a varied prevalence across different geographical regions and more women are affected than men.

GPP flares can lead to hospitalization with serious complications, including heart failure, renal failure and sepsis, and the unpredictability and severity of these flares greatly affect a person's quality of life.

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Boehringer Ingelheim
Boehringer Ingelheim is working on breakthrough therapies that improve the lives of humans and animals. As a leading research-driven biopharmaceutical company, the company creates value through innovation in areas of high unmet medical need. Founded in 1885 and family-owned ever since, Boehringer Ingelheim takes a long-term perspective. Around 52,000 employees serve more than 130 markets in the three business areas, Human Pharma, Animal Health, and Biopharmaceutical Contract Manufacturing. Learn more at www.boehringer-ingelheim.com.

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