Spevigo (spesolimab-sbzo) / Boehringer Ingelheim - LARVOL DELTA

A Study to Test Whether Spesolimab Helps People With a Skin Disease Called Netherton Syndrome (clinicaltrials.gov) - P2/3 | N=43 | Active, not recruiting | Sponsor: Boehringer Ingelheim | Trial completion date: Jan 2028 ➔ Jul 2025

Trial completion date • Dermatology

Lymphomatoid Papulosis Following Spesolimab Treatment for Generalised Pustular Psoriasis. (PubMed, Australas J Dermatol) - No abstract available

Journal • Dermatology • Hematological Malignancies • Immunology • Lymphoma • Oncology • Psoriasis • Pustular Psoriasis

IL-36 signaling as a drug target in Crohn's disease patients with IL36RN mutations. (PubMed, EMBO Mol Med) - "Administration of the IL-36R-blocking antibody spesolimab to this patient resulted in a reduction of intestinal inflammation and alterations in immune cell composition and function. Our findings indicate that pathogenic IL36RN mutations may contribute to the pathogenesis of Crohn's disease in a subset of patients and that inhibiting IL-36 signaling could offer a personalized therapeutic approach for these patients."

Journal • Crohn's disease • Gastroenterology • Gastrointestinal Disorder • Immunology • Inflammation • Inflammatory Bowel Disease • CD123

Spesolimab in generalized pustular psoriasis complicated by acrodermatitis continua of Hallopeau: a case report and mechanistic insights. (PubMed, Front Immunol) - "While conventional treatments failed to achieve significant improvement, the patient received two doses of spesolimab, and the effect was remarkable. No adverse reactions were observed in the follow-up period."

Journal • Dermatitis • Dermatology • Immunology • Psoriasis • Pustular Psoriasis

Treatment with spesolimab in patients with generalized pustular psoriasis: A review of real-world experience. (PubMed, Clin Exp Dermatol) - "Spesolimab demonstrates a low prevalence of adverse events and clinical efficacy for GPP treatment including patients with comorbidities, infections, and over 75 years of age. Its effect on plaque psoriasis remains unclear."

Journal • Real-world evidence • Dermatology • Immunology • Infectious Disease • Inflammation • Psoriasis • Pustular Psoriasis

Assessment of Conditional Listing for Highly Uncertain and High-Priced Drugs in Taiwan (ISPOR 2025) - " A total of 12 drugs were considered for conditional listing between 2022 and 2024, which included Pemazyre, Tepmetko, Qarziba, Kymriah, Vitrakvi, Blincyto, Vydamax, Takhzyro, Polivy, Velexbru, Spevigo, and Koselugo. The conditional listing opens up new opportunities for high-priced drugs. However, the 2-year real-world evidence for Pemazyre remains insufficient for the government to make a final reimbursement decision. Consequently, the results of the HTA will pose challenges in the future in Taiwan."

Generalized Pustular Psoriasis During Early Pregnancy Successfully Treated with Spesolimab: A Case Report. (PubMed, Int Med Case Rep J) - "We present a patient with Gpp at 8 weeks of gestation. The patient was finally treated with spesolimab, and her condition improved significantly after 3 days."

Journal • Dermatology • Immunology • Psoriasis • Pustular Psoriasis

Imsidolimab, a novel IgG4 IL36 receptor antagonist, was effective and well tolerated in patients with GPP (SID 2025) - "In contrast, a total of 23 cases of ADAs (23/50, 46%) were reported among patients who had received at least 1 dose of spesolimab. Imsidolimab has demonstrated effectiveness in GPP with a well tolerated and safe profile after a single dose. Targeting IL36 signaling with imsidolimab represents a promising therapeutic option."

Clinical • Late-breaking abstract • Dermatology • Immunology • Pain • Psoriasis • Pustular Psoriasis

Inflammatory markers in generalized pustular psoriasis (GPP): An immunohistochemical analysis (SID 2025) - "Antibody treatment with the IL-36R antagonist spesolimab has shown efficacy...These results indicate that compared to normal skin there is a lack IL-23 in GPP, whereas IL-36 gamma, the transcription factor Fox-P3, IL-17-F and IL-8 are the dominant cytokines in the skin of GPP. Our results provide a basis for successful treatment in generalized pustular psoriasis."

Dermatology • Immunology • Infectious Disease • Inflammation • Psoriasis • Pustular Psoriasis • CXCL8 • CXCR3 • IL17A • IL23A • JAK3

Therapeutic insights from spatial mapping of hidradenitis suppurativa skin lesions at single cell resolution (SID 2025) - "This finding explains why Phase 2 clinical trials targeting IL36R with spesolimab reduced draining sinus tracts but not abscesses or nodules. Together, our findings advance our understanding of HS pathogenesis and highlight PCs and the IL-36 pathway as therapeutic targets in HS."

Dermatology • Hidradenitis Suppurativa • Immunology • Inflammation • CD4 • CD8

NICE Recommends Two Monoclonal Antibody Treatments (Medscape) - "Spesolimab (Spevigo, Boehringer Ingelheim) is the first targeted treatment for GPP. NICE estimates that 1300 people could benefit...NICE said spesolimab could be used to treat GPP flares in adults, but only under specific clinical criteria. These include: A GPP Physician Global Assessment (GPPGA) total score of 3 or higher. Presence of fresh pustules or worsening of existing pustules. A GPPGA pustulation subscore of 2 or more. At least 5% of body surface area covered with erythema and the presence of pustules."

NICE • Pustular Psoriasis

“Update of sections 4.4, and 4.8 of the SmPC in order to add a new warning on hypersensitivity and add hypersensitivity to the list of adverse drug reactions (ADRs) with frequency ‘not known’ based on data from clinical trials and post-marketing data sources; the Package Leaflet is updated accordingly. The RMP version 4.0 has also been submitted” (European Medicines Agency) - Pharmacovigilance Risk Assessment Committee (PRAC)-Draft agenda for the meeting on 5 - 8 May 2025: “For adoption of PRAC RMP AR, PRAC RMP assessment overview and advice to CHMP”

PRAC • Hidradenitis Suppurativa

Generalized Pustular Psoriasis with Cushing's Syndrome: A Case of Effective Spesolimab Treatment. (PubMed, Biologics) - "The patient was treated with Spesolimab, a monoclonal antibody, resulting in a substantial improvement in symptoms. This development offers novel treatment options and provides a reference for clinical medication for patients with this particular type of GPP."

Journal • Cushing’s Disease • Dermatology • Endocrine Disorders • Immunology • Inflammation • Pain • Psoriasis • Pustular Psoriasis

Update on Generalized Pustular Psoriasis. (PubMed, Acta Med Port) - "Currently, spesolimab is the only therapy specifically approved for treating GPP flares in adolescents and adults, in both Europe and the United States of America. However, the management of GPP remains complex and challenging. This narrative review provides an overview of the epidemiology, pathophysiology, clinical features, comorbidities, and evolving therapeutic strategies for GPP."

Journal • Review • Dermatology • Immunology • Infectious Disease • Inflammation • Psoriasis • Pustular Psoriasis • Septic Shock

Generalized pustular psoriasis (von Zumbusch) flares successfully treated with Spesolimab. Report of two cases and review of the literature. (PubMed, An Bras Dermatol) - No abstract available

Journal • Dermatology • Immunology • Psoriasis • Pustular Psoriasis

What is "chronic" pustular psoriasis? Identifying candidates for long-term subcutaneous spesolimab treatment. (PubMed, Expert Opin Biol Ther) - No abstract available

Journal • Dermatology • Immunology • Psoriasis • Pustular Psoriasis

Specific drugs for rare diseases in a province of eastern China under catalog management: from 2021 to 2023. (PubMed, Front Pharmacol) - "Spesolimab, Sodium Phenylbutyrate, Nitisinone and Emapalumab are currently in short supply, and the delivery rate of 16 drugs such as Selumetinib, Sirolimus (tablet), Octreotide, Dimethyl Fumarate and Lanreotide is below 80%...This indicates a generally high level of accessibility to drugs for rare diseases in China. However, attention should be given to improving the supply capacity for drugs that are in short supply and have a low delivery rate."

Journal • Rare Diseases

Medications Associated With Generalized Pustular Psoriasis Flares: A Retrospective Pharmacovigilance Study. (PubMed, J Cutan Med Surg) - No abstract available

Adverse events • Journal • Retrospective data • Dermatology • Immunology • Psoriasis • Pustular Psoriasis

Novel Small-Molecule Treatment and Emerging Biological Therapy for Psoriasis. (PubMed, Biomedicines) - "There are many types of drugs to treat psoriasis: small-molecule drugs, including corticosteroids; retinoids; vitamin D analogs; and immunosuppressants, such as glucocorticoid ointment, tretinoin cream, methotrexate tablets, etc. Macromolecular biological drugs, such as Certolizumab, Secukinumab, Guselkumab, etc., include monoclonal antibodies that target various inflammatory signaling pathways...Spesolimab is the latest biological agent used to target the interleukin-36 receptor (IL-36R) to be approved for market use, which significantly reduces the risk of general pustular psoriasis (GPP) flare by 84%...In this review, we analyze the current status of psoriasis therapeutics and discuss novel delivery systems for diverse psoriasis drugs, as well as emerging cell-based therapies. We also summarize the therapeutic effectiveness of different delivery strategies."

Journal • Review • Dermatology • Immunology • Psoriasis • Pustular Psoriasis

A Study to Test Long-term Treatment With Spesolimab in People With a Skin Condition Disease Called Hidradenitis Suppurativa (HS) Who Took Part in a Previous Study With Spesolimab (clinicaltrials.gov) - P2/3 | N=39 | Completed | Sponsor: Boehringer Ingelheim | Active, not recruiting ➔ Completed

Trial completion • Dermatology • Hidradenitis Suppurativa • Immunology

Multi-Criteria Decision Analysis for Evaluating Spesolimab in Generalized Pustular Psoriasis in Brazil [WITHDRAWN] (ISPOR 2025) - "This MCDA exercise established a robust framework for evaluating and prioritizing key criteria in the context of GPP treatment, emphasizing the therapeutic benefits of spesolimab. The significant weighting of health-related criteria underlines the urgency for effective interventions and supports timely access to novel treatments within the Brazilian healthcare system. Future efforts should incorporate economic assessments as more robust economic burden data becomes available, enabling a more comprehensive analysis."

Dermatology • Immunology • Inflammation • Psoriasis • Pustular Psoriasis

Successful treatment of generalized pustular psoriasis with chronic hepatitis B using spesolimab: A case report. (PubMed, Medicine (Baltimore)) - "Our study shows that spesolimab could be employed as a safe and effective therapeutic option for patients with GPP and other underlying conditions."

Journal • Dermatology • Hepatitis B • Hepatology • Immunology • Infectious Disease • Inflammation • Liver Failure • Psoriasis • Pustular Psoriasis

Effective Spesolimab Treatment for Generalized Pustular Psoriasis Masquerading as Acute Generalized Exanthematous Pustulosis: A Case Report. (PubMed, Cureus) - "Spesolimab, a monoclonal antibody targeting the interleukin 36 (IL-36) receptor, provides rapid symptom relief for GPP flares, but real-world data remain limited. We report a case of GPP complicated by overlapping features with AGEP, highlighting the importance of precise diagnosis and the efficacy of spesolimab."

Journal • Dermatology • Immunology • Psoriasis • Pustular Psoriasis

The role of interleukin inhibitors in the treatment of hidradenitis suppurativa; a systematic review of clinical trials. (PubMed, Autoimmun Rev) - "Secukinumab showed sustained hidradenitis Suppurativa Clinical Response (HiSCR) improvements, particularly in biologic-naïve patients with common adverse events (AEs). Bimekizumab was effective with biweekly dosing, while the four-week regimen had inconsistent results, with rare reports of AEs. Brodalumab and bermekimab provided rapid and sustained HiSCR responses with lesion reductions, which were well tolerated. Guselkumab and ustekinumab showed promising but statistically nonsignificant improvements with mild AEs. Risankizumab did not significantly improve HiSCR rates but showed Dermatology Life Quality Index (DLQI) benefits. Anakinra offered moderate efficacy with prolonged exacerbation-free periods but led to some treatment discontinuations. Spesolimab reduced inflammatory lesions and draining tunnels while maintaining a favorable safety profile. IL inhibitors, especially IL-17 inhibitors, have demonstrated efficacy in treating moderate-to-severe HS, while..."

Journal • Review • Dermatology • Hidradenitis Suppurativa • Immunology • Inflammation • IL17A • IL23A

Case Report: Spesolimab for pyoderma gangrenosum in an undifferentiated oligoarthritis patient receiving anti-IL-17 therapy. (PubMed, Front Immunol) - "The patient was diagnosed with pyoderma gangrenosum (PG) and achieved remission after receiving three doses of 900 mg intravenous Spesolimab every 4 weeks, along with discontinuation of Ixekizumab. The case highlights the successful use of Spesolimab (anti-IL36R) in treating PG and explores the potential "paradoxical phenomenon" linked to anti-IL-17 therapy, providing novel insights into immune dysregulation and therapeutic strategies."

Journal • Dermatology • Immunology • Infectious Disease • Inflammatory Arthritis • Pyoderma Gangrenosum • IL17A