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

Bhutani, Tina; Farberg, Aaron S.

doi:10.1007/s13555-024-01103-5

Cited by 3 publications

(1 citation statement)

References 77 publications

(197 reference statements)

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“…A significant proportion of GPP patients, approximately 20%, develop psoriatic arthritis (PsA), suggesting a deep interconnection between cutaneous and joint manifestations in psoriasis [ 108 ]. The notable rapid progression from the onset of psoriatic arthritis to the diagnosis of GPP highlights the critical need for understanding the underlying mechanisms that link these conditions [ 109 ].…”

Section: Arthritis In Gppmentioning

confidence: 99%

Generalized Pustular Psoriasis and Systemic Organ Dysfunctions

Teshima,

Saito-Sasaki,

Sawada

2024

IJMS

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This review explores the intricate relationship between generalized pustular psoriasis (GPP) and various systemic diseases, shedding light on the broader impacts of this severe skin condition beyond its primary dermatological manifestations. GPP is identified as not only a profound contributor to skin pathology but also a significant risk factor for systemic diseases affecting cardiovascular, hepatic, renal, pulmonary, and skeletal systems, as well as associated with an increased incidence of anemia, depression, anxiety, and arthritis. The research highlights the complex interplay of cytokines, particularly IL-17 and IL-36, which are central to the pathophysiology of GPP and implicated in the exacerbation of systemic conditions. Key findings indicate a higher incidence of cardiovascular events in GPP patients compared to those with other severe forms of psoriasis, notably with a stronger correlation between myocardial infarction history and GPP development. Liver disturbances, frequently reversible upon psoriasis remission, suggest a cytokine-mediated link to hepatic health. Renal dysfunction appears elevated in GPP sufferers, with IL-17 and IL-36 potentially driving renal fibrosis. Similarly, interstitial lung disease and osteoporosis in GPP patients underscore the systemic reach of inflammatory processes initiated in the skin. The associations with anemia, depression, anxiety, and arthritis further complicate the clinical management of GPP, requiring a multidisciplinary approach. The study concludes that managing GPP effectively requires a holistic approach that addresses both the cutaneous and systemic dimensions of the disease, advocating for continued research into the mechanisms that connect GPP with broader health implications to refine therapeutic strategies.

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Section: Arthritis In Gppmentioning

confidence: 99%

Generalized Pustular Psoriasis and Systemic Organ Dysfunctions

Teshima,

Saito-Sasaki,

Sawada

2024

IJMS

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Exploring the Chronic Nature of Generalized Pustular Psoriasis [Podcast]

Elewski,

Lebwohl

2024

CCID

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Generalized pustular psoriasis (GPP) is a rare, chronic, inflammatory skin disease characterized by persistent symptoms and sudden flares of painful, sterile pustules, and may be accompanied by systemic inflammation. Ongoing symptoms of GPP can have a serious impact on patient quality of life, morbidity, and mortality, and severe flares may be life-threatening if left untreated. Guidelines have been developed for the treatment of GPP flares; however, health care professionals and patients are lacking guidance on the management of long-term, persistent symptoms of GPP. Spesolimab is the only FDA-approved treatment for GPP and is approved for use in adults and pediatric patients aged 12 years or older and weighing at least 40 kg. Spesolimab recently gained FDA approval as a subcutaneous injection to treat GPP when patients are not experiencing a flare. In this podcast episode, we discuss what is known about the chronic disease burden of GPP and how persistent symptoms affect quality of life when patients are not experiencing a flare. We address the need for treatment guidelines for chronic GPP and discuss the results of the EFFISAYIL ® 2 clinical trial, which led to the approval of the subcutaneous formulation of spesolimab to treat GPP when patients are not experiencing a flare. Finally, we discuss what can be done to improve the treatment of patients with chronic GPP, both while experiencing a flare, and while living with persistent symptoms.

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

Cited by 3 publications

References 77 publications

Generalized Pustular Psoriasis and Systemic Organ Dysfunctions

Generalized Pustular Psoriasis and Systemic Organ Dysfunctions

Exploring the Chronic Nature of Generalized Pustular Psoriasis [Podcast]

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

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