New Onset Generalized Pustular Psoriasis Rapidly Improved with IL-36 Blockade

Burns, Meredith; Ho-Pham, Hoang; Elston, Carly; Elewski, Boni E.

doi:10.25251/skin.7.1.4

J of Skin

2023

DOI: 10.25251/skin.7.1.4

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New Onset Generalized Pustular Psoriasis Rapidly Improved with IL-36 Blockade

Meredith Burns

Hoang Ho-Pham²,

Carly Elston³

et al.

Abstract: Generalized pustular psoriasis is a relatively rare variant of psoriasis characterized by diffuse eruption of sterile pustules on an erythematous background. Untreated flares can result in life-threatening complications including sepsis and organ failure. Generalized pustular psoriasis has historically proven challenging to treat with an unpredictable clinical course and paucity of reliably efficacious treatment options. Until recently, there have been no FDA-approved therapies for generalized pustular psorias… Show more

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2024

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Cited by 3 publications

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Clinical and Disease Burden of Patients with Generalized Pustular Psoriasis: A Review of Real-World Evidence

Bhutani,

Farberg

2024

Dermatol Ther (Heidelb)

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Generalized pustular psoriasis (GPP) is a chronic, rare, and potentially life-threatening disease. There is limited understanding of patient characteristics in GPP and their correlation with disease progression or healthcare resource utilization. Our review aims to examine real-world evidence on these characteristics and the associated disease burden as related to economic and quality of life factors. Results showed that most patients with GPP experienced flares once a year, lasting from 2 weeks to 3 months, with > 80% of patients having residual disease post-flare, with/without treatment, indicating the long-term nature of GPP. The impact of GPP on patients’ daily activities was significant, even in the absence of a flare. GPP adversely affected mental health, and anxiety and depression were reported regularly. Patients with GPP had more comorbidities, were prescribed more medication, and had more inpatient and outpatient visits than in matched plaque psoriasis or general population cohorts. Improving the education of healthcare providers in diagnosing GPP, defining disease flares, and managing the disease, as well as making globally accepted clinical guidelines for GPP treatment available, could help to reduce the burden on patients with GPP. Effective therapies that control and prevent GPP flares and manage chronic disease are needed.

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Clinical and Disease Burden of Patients with Generalized Pustular Psoriasis: A Review of Real-World Evidence

Bhutani,

Farberg

2024

Dermatol Ther (Heidelb)

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Psoriasis pustuleux généralisé : spécificités physiopathologiques, cliniques et thérapeutiques

Jullien,

Misery,

Viguier

2024

Annales de Dermatologie et de Vénéréologie - FMC

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Spesolimab for generalized pustular psoriasis: a review of two key clinical trials supporting initial US regulatory approval

Gwillim,

Nichols

2024

Front. Immunol.

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Generalized pustular psoriasis (GPP) is a chronic, rare, and potentially life-threatening inflammatory disease, characterized by the rapid and widespread eruption of small, sterile pustules with surrounding skin erythema. Abnormal signaling of the interleukin-36 (IL-36) pathway appears to have a central role in GPP immunopathology, and provides a rational therapeutic target. Spesolimab is a first-in-class humanized monoclonal antibody that binds specifically to the IL-36 receptor, and antagonizes IL-36 signaling. Spesolimab obtained regulatory approval in the United States (US) in September 2022 for use in the treatment of GPP flares in adults, and was subsequently approved for GPP flare treatment in many other countries across the world. Recently, regulatory approval was granted for subcutaneous dosing of spesolimab for treatment of GPP when not experiencing a flare. Here, we review data from two key clinical trials that supported the initial US regulatory approval; namely, the phase 1 proof-of-concept trial (ClinicalTrials.gov ID, NCT02978690), and Effisayil™ 1 (NCT03782792), which remains the largest and only randomized clinical trial in patients experiencing GPP flares published to date. In the phase 1 proof-of-concept trial, a Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) score of 0 or 1 (clear or almost clear skin) was attained in 5/7 (71%) patients by week 1 and in all 7 patients by week 4; and the mean percent improvement in the Generalized Pustular Psoriasis Area and Severity Index (GPPASI) score from baseline was 59.0% at week 1, 73.2% at week 2, and 79.8% at week 4. In Effisayil™ 1, a GPPGA pustulation subscore of 0 (no visible pustules) was achieved in 19/35 (54%) patients receiving spesolimab at the end of week 1, versus 1/18 (6%) receiving placebo (difference, 49 percentage points; 95% confidence interval [CI], 21 to 67; P<0.001); and a GPPGA total score of 0 or 1 was achieved by 15/35 (43%) patients in the spesolimab group, versus 2/18 (11%) patients in the placebo group (difference, 32 percentage points; 95% CI, 2 to 53; P = 0.02). Infections at week 1 were reported in 6/35 (17%) patients receiving spesolimab and in 1/18 (6%) patients receiving placebo. These data demonstrate the efficacy and safety of spesolimab in providing rapid and sustained clinical improvement for patients with GPP flares, which translates into improved quality of life, by offering a targeted therapy for GPP.

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New Onset Generalized Pustular Psoriasis Rapidly Improved with IL-36 Blockade

Cited by 3 publications

References 9 publications

Clinical and Disease Burden of Patients with Generalized Pustular Psoriasis: A Review of Real-World Evidence

Clinical and Disease Burden of Patients with Generalized Pustular Psoriasis: A Review of Real-World Evidence

Psoriasis pustuleux généralisé : spécificités physiopathologiques, cliniques et thérapeutiques

Spesolimab for generalized pustular psoriasis: a review of two key clinical trials supporting initial US regulatory approval

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