A study to test whether spesolimab helps people with Generalized Pustular Psoriasis (GPP) who need treatment for repeated flares

Prior treatment

• Drug-triggered Acute Generalized Exanthematous Pustulosis (AGEP).
• Prior use of any other IL-36R inhibitor (e.g.imsidolimab) i.v. and/or s.c.
• Relevant chronic or acute infections including active tuberculosis, HIV infection or viral hepatitis at the time of initial treatment. For patients screened while having a flare, if Visit 1 HIV or viral hepatitis results are not available in time for dosing, these patients may receive treatment as long as the investigator has ruled out active disease based on available documented history (i.e. negative HIV and viral hepatitis test results) within 3 months prior to Visit 2. A patient can be re-screened if the patient was treated and is cured from the acute infection.
• Treatment with any drug considered likely to interfere with the safe conduct of the trial, as assessed by the investigator.
• Prior use of spesolimab s.c. or i.v. (exception: exposure to i.v. spesolimab that occurred in trials 1368-0011 or 1368-0013 only are not excluded )

Other illness

• Increased risk of infectious complications (e.g. recent pyogenic infection, any congenital or acquired immunodeficiency (e.g. HIV), past organ or stem cell transplantation), as assessed by the investigator.
• Patients with SAPHO (Synovitis–acne–pustulosis–hyperostosis–osteitis) syndrome.
• Immediate life-threatening flare of GPP or requiring intensive care treatment, according to the investigator’s judgement. Life-threatening complications mainly include, but are not limited to, cardiovascular/cytokine driven shock, pulmonary distress syndrome, or acute renal failure.

Lab values

• Severe, progressive, or uncontrolled hepatic disease, defined as >3-fold ULN elevation in AST or ALT or alkaline phosphatase, or >2-fold ULN elevation in total bilirubin.

Other

• Further exclusion criteria apply.
• Patients with primary plaque psoriasis vulgaris without presence of pustules or with pustules that are restricted to psoriatic plaques.
• Patients with primary erythrodermic psoriasis vulgaris.
• Presence of acute demyelinating neuropathy

• Patients with a GPPGA pustulation subscore of 0 or 1 and a known and documented history of GPP (per ERASPEN criteria), regardless of IL-36RN mutation status b. Patients with a GPP flare and a known and documented history of GPP (per ERASPEN criteria) regardless of IL-36RN mutation status
  • Patients must have a history of frequent GPP flares in the past: a. Patients who are not on concomitant GPP treatment at the time of screening must have had at least 2 presentations of GPP flares in the past year b. Patients who are on concomitant GPP treatment must stop this treatment at the day of initial administration of trial drug (Visit 2). These patients must have a history of flaring in case of dose reduction or discontinuation of their concomitant GPP medication.
  • Male or female patients, aged ≥18 years (if local legislation for age of consent differs, then local legislation will be followed) at screening.
  • Signed and dated written informed consent prior to admission to the trial in accordance with ICH GCP and local legislation prior to start of any screening procedures.
  • Women of childbearing potential (WOCBP) must be ready and able to use highly effective methods of birth control per ICH M3 (R2) that result in a low failure rate of less than 1% per year when used consistently and correctly. A list of contraception methods meeting these criteria and instructions on the duration of their use is provided in the participant information