Health Canada approves first treatment option for generalized pustular psoriasis flares in adults

-  Generalized pustular psoriasis differs significantly from plaque psoriasis in both its disease mechanism and severity.¹
-  In the EFFISAYIL^®-1 trial, over half of the SPEVIGO^® (spesolimab)-treated patients were free of pustules, one week after receiving a single dose.²

BURLINGTON, ON, March 23, 2023 /CNW/ - Health Canada has granted marketing authorization for SPEVIGO^® (spesolimab for injection) for the treatment of flares in adult patients with generalized pustular psoriasis (GPP), Boehringer Ingelheim (Canada) Ltd. has announced today.³ Spesolimab is a novel, selective antibody that blocks the activation of the interleukin-36 receptor (IL-36R), a signaling pathway within the immune system shown to be involved in the pathogenesis of GPP.^2,4,5

Distinct from plaque psoriasis, GPP is a rare dermatological condition, characterized by the sudden appearance of multiple small blisters filled with sterile pus on large areas of the body referred to as a flare.⁶ A flare can be triggered by certain medications, steroid withdrawal, stress, excessive sun exposure, infections, and pregnancy.^7,8 Flares greatly affect a person's quality of life and can cause additional symptoms such as fever, chills, malaise, nausea and pain, or even life-threatening complications that may require emergency medical treatment.^2,5,6 Given that it is so rare, recognizing the symptoms can be challenging and consequently lead to delays in diagnosis.¹

"GPP flares can greatly impact a patient's life and lead to serious, life-threatening complications," said Dr. Hélène Veillette, MD, FRCPC, associate clinical professor, director of dermatology, CHU de Québec-Université Laval and Principal Investigator at Diex Research. "The approval of spesolimab is a turning point for dermatologists and patients. We now have a GPP flare treatment that resolves GPP flares quickly and is approved specifically for GPP, based on randomized controlled clinical trials."

This approval is based on results from the pivotal EFFISAYIL^®-1 Phase II clinical trial. In the 12-week trial, patients experiencing a GPP flare were treated with spesolimab or placebo.² Most patients at the outset of the trial had a high, or very high, density of pustules, and impaired quality of life.² After one week, 54% of patients treated with spesolimab showed no visible pustules compared to placebo (6%).²

"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 Dr. Gabriel Kim, Vice President, Medical and Regulatory Affairs, Boehringer Ingelheim (Canada) Ltd. "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. Today's news marks the first of what we hope will be a number of new treatment options from our accelerated late-stage portfolio with the potential to transform the lives of people for generations to come."

About spesolimab
Spesolimab is a novel, humanized, selective antibody that blocks the activation of the interleukin-36 receptor (IL-36R), a signaling pathway within the immune system shown to be involved in the pathogenesis of several autoinflammatory diseases, including GPP.^2,4,5 It is the first investigational treatment to specifically target the IL-36 pathway for the treatment of GPP flares that has been evaluated in a statistically powered, randomized, placebo-controlled trial.

About the EFFISAYIL^®-1 clinical trial
EFFISAYIL^®-1 (NCT03782792) was a 12-week Phase II trial investigating patients with a GPP flare (N=53), randomly assigned 2:1 to a single 900 mg intravenous dose of spesolimab or placebo.¹

After one week, 54% of patients treated with spesolimab showed no visible pustules, compared to 6% of patients treated with placebo.¹ After 12 weeks more than 4 out of 5 patients (84.4%) had no visible pustules and clear/almost clear skin (81.3%).¹

Infections were reported by 17% and 6% of patients in the spesolimab and placebo groups respectively (at week one).¹

About generalized pustular psoriasis (GPP)
GPP is a rare, heterogenous and potentially life-threatening neutrophilic skin disease, which is clinically distinct from plaque psoriasis.^1,5 GPP is caused by neutrophils (a type of white blood cell) accumulating in the skin, resulting in painful, sterile pustules all over the body.⁹ The clinical course varies, with some patients having a relapsing disease with recurrent flares, and others having a persistent disease with intermittent flares.⁹ 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.⁵ This chronic, systemic disease has a substantial quality of life impact for patients and increased healthcare burden.¹⁰ GPP has a varied prevalence across different geographical regions and more women are affected than men.^5,11,12,13

About Boehringer Ingelheim (Canada) Ltd.  
Boehringer Ingelheim is working on breakthrough therapies that transform lives, today and for generations to come. 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. More than 52,000 employees serve over 130 markets in the three business areas, Human Pharma, Animal Health, and Biopharmaceutical Contract Manufacturing. The Canadian headquarters of Boehringer Ingelheim was established in 1972 in Montreal, Quebec and is now located in Burlington, Ontario. Boehringer Ingelheim employs approximately 500 people across Canada. Learn more at www.boehringer-ingelheim.ca. 

REFERENCES

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¹ Puig L, et al. Global consensus on the clinical course, treatment and management of generalized pustular psoriasis (GPP). Presented at the European Academy of Dermatology and Venereology (EADV) Congress, Milan, Italy, 7–10 September 2022: P1203.B.

² Bachelez H et al. Trial of Spesolimab for Generalized Pustular Psoriasis. NEJM. 2021;385:2431-40.

³ SPEVIGO Product Monograph, March 22, 2023

⁴ Furue K, et al. Highlighting Interleukin-36 Signalling in Plaque Psoriasis and Pustular Psoriasis. Acta Derm Venereol. 2018;98:5–13.

⁵ Crowley JJ, et al. A brief guide to pustular psoriasis for primary care providers, Postgrad Med. 2021;133(3):330-344.

⁶ Melinda J. Gooderham, Abby S. Van Voorhees & Mark G. Lebwohl (2019) An update on generalized pustular psoriasis, Expert Review of Clinical Immunology, 15:9, 907-919, DOI: 10.1080/1744666X.2019.1648209.

⁷ Strober B, Kotowsky N, Medeiros R, et al. Unmet medical needs in the treatment and management of generalized pustular psoriasis flares: evidence from a survey of Corrona registry dermatologists. Dermatol Ther (Heidelb). 2021;11(2):529-541. doi:10.1007/s13555-021- 00493-0.

⁸ Kharawala S, Golembesky AK, Bohn RL, Esser D. The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review. Expert Rev Clin Immunol. 2020;16(3):239-252. doi:10.1080/1744666X.2019.1708193.

⁹ Navarini AA, et al. European consensus statement on phenotypes of pustular psoriasis. JEADV. 2017;31:1792-1799.

¹⁰ Hanna M, et al. Economic burden of generalized pustular psoriasis and palmoplantar pustulosis in the United States. Curr Med Res Opin. 2021. 37(5):735-742.

¹¹ Ohkawara A et al. Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background. Acta Derm Venereol. 1996 Jan;76(1):68–71.

¹² Augey F, et al. Generalized pustular psoriasis (Zumbusch): a French epidemiological survey. Eur J Derm. 2006; 16(6):669-673.

¹³ Jin H, et al. Clinical features and course of generalized pustular psoriasis in Korea. J Dermatol. 2015; 42(7):674-678.

SOURCE Boehringer Ingelheim (Canada) Ltd.

Anne Coffey, Human Pharma Communications Manager, Boehringer Ingelheim (Canada) Ltd. , Email: [email protected]