Exploratory Study of IFX-1 in Patients With Pyoderma Gangrenosum

Open Label Exploratory Phase IIa Trial to Investigate the Safety and Efficacy of IFX-1 in Treating Patients With Pyoderma Gangrenosum (OPTIMA)

The purpose of this study is to determine whether vilobelimab (development name: IFX-1) is safe and effective in the treatment of pyoderma gangrenosum.

Neutrophilic dermatoses are a spectrum of inflammatory disorders characterized by skin lesions resulting from a neutrophil-rich inflammatory infiltrate in the absence of infection. Pyoderma gangrenosum is associated with a neutrophilic leukocytosis, which is likely to be triggered by C5a. This study is set up based on the hypothesis that vilobelimab might be able to block C5a induced pro-inflammatory effects such as neutrophil activation and cytokine generation, potentially contributing to the local skin inflammation and tissue damage.

Inclusion Criteria:

• Diagnosis of an ulcerative form of pyoderma gangrenosum confirmed by the investigator

In addition, the subject must fulfill at least 3 of the following 6 criteria at screening:

History of

• Pathergy (ulcer occurring at the sites of trauma)
• Personal history of inflammatory bowel disease or inflammatory arthritis
• History of papule, pustule or vesicle that rapidly ulcerated

Clinical examination (or photographic evidence) of

• Peripheral erythema, undermining border, and tenderness at site of ulceration
• Multiple ulcerations (at least 1 occurring on the lower leg)
• Cribriform or "wrinkled paper" scar(s) at sites of healed ulcers

Subject has a minimum of 1 evaluable ulcer (≥2 cm2) on the lower extremity at screening

Exclusion Criteria:

• Pyoderma gangrenosum target ulcer for more than 3 years before screening
• Surgical wound debridement within the previous 2 weeks before screening
• Use of intravenous antibacterials, antivirals, anti-fungals, or anti-parasitic agents within 30 days before screening
• Any drug treatment for pyoderma gangrenosum including corticosteroids (>10 mg), intralesional steroids, cyclosporine A, biologicals and immunosuppressives (with the exception of antibiotics for wound superinfection) used within a time of 5 half-lives of the drug before screening