The burden associated with generalized pustular psoriasis: A Canadian population-based study of inpatient care, emergency departments, and hospital- or community-based outpatient clinics

Tarride, J.E.; Prajapati, V.H.; Lynde, C.; Blackhouse, G.

doi:10.1016/j.jdin.2023.03.012

Cited by 3 publications

(1 citation statement)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Further data describing the acute and long-term burden of GPP, and the health economic pathways and burden of disease, in various populations have been published recently [ 53 , 57 – 60 ]. However, there remains an unmet need for improved guidelines for GPP diagnosis and management, improved recognition and understanding of GPP by HCPs, and multi-level support involving primary care, emergency care, and dermatologists [ 61 ].…”

Section: Discussionmentioning

confidence: 99%

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

Bhutani,

Farberg

2024

Dermatol Ther (Heidelb)

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

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

Bhutani,

Farberg

2024

Dermatol Ther (Heidelb)

View full text Add to dashboard Cite

show abstract

Review of Generalized Pustular Psoriasis: Prevalence, Diagnosis, and Treatment Options

Kozina,

Musiek,

Anadkat

2024

Journal of Psoriasis and Psoriatic Arthritis

View full text Add to dashboard Cite

Background: Generalized pustular psoriasis (GPP) is a diagnostic challenge for clinicians and is relatively rare. Prevalence is currently reported to be higher in Asian countries and among female patients; peak age of onset is variable across studies. Objective: This article provides clinicians with an overview of the global prevalence of GPP, common clinical presentation and diagnostic criteria, pathogenesis, and evolving treatment options. Discussion: Clinical presentation is characterized by onset of generalized sterile pustules with erythema, and may include systemic inflammatory signs including fever and lab abnormalities. Diagnostic criteria vary globally. European Rare and Severe Psoriasis Expert Network (ERASPEN) criteria present a clinical diagnosis while Japanese criteria include a biopsy. Advancements in understanding of pathogenesis have uncovered the role of IL-36 as a driver of disease, and this is the target of new treatments including spesolimab, an anti-IL-36 receptor antibody.

show abstract

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

Gwillim,

Nichols

2024

Front. Immunol.

View full text Add to dashboard Cite

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.

show abstract

The burden associated with generalized pustular psoriasis: A Canadian population-based study of inpatient care, emergency departments, and hospital- or community-based outpatient clinics

Cited by 3 publications

References 13 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

Review of Generalized Pustular Psoriasis: Prevalence, Diagnosis, and Treatment Options

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

Contact Info

Product

Resources

About