Treatment with spesolimab in patients with generalized pustular psoriasis: a review of real-world experience

Generalized pustular psoriasis (GPP) is a potentially life-threatening rare immune-mediated disease characterized by rapid onset of erythematous plaques, sterile pustules and systemic inflammation. Spesolimab, a monoclonal antibody targeting the interleukin-36 receptor, is a novel treatment for GPP. However, GPP's low incidence and limited evidence relating to treatment with spesolimab represents a challenge in determining its real-world efficacy. This review explores the clinical use of spesolimab in a real-world setting. We conducted a review on PubMed, SCOPUS, Embase, and ScienceDirect of case series and reports of patients with GPP treated with spesolimab. Review articles and clinical trials were excluded. From 33 articles, 62 patients with GPP were included. Age ranged from 4 to 88 years. Comorbidities were reported in 48 of the 62 patients; of these, 30 had plaque psoriasis. Spesolimab 900 mg intravenous was administered in one to four doses, depending on clinical response. The second dose was administered 1 week after the first, while the additional doses were given at variable intervals. Follow-up periods ranged from 2 weeks to 15 months. Complete GPP remission was observed in 35/62 (56%) patients, 21 of them achieving it within a week. Partial remission was defined in 27 of the 62 patients (44%). GPP recurrence after treatment was observed in seven patients. Among the 30 patients with concomitant plaque psoriasis reported, after spesolimab administration, plaque psoriasis recurrence was reported in 6 (20%) patients, 3 (10%) reported improvement and for 21 (70%), this outcome was not reported. Eight patients reported adverse effects, including laboratory abnormalities, infections and erythema multiforme. Spesolimab demonstrates a low prevalence of adverse events and clinical efficacy for GPP treatment, including in patients with comorbidities, infections and those > 75 years of age. Its effect on plaque psoriasis remains unclear.