JAK Inhibitors' Efficacy in Bullous Pemphigoid

Plain-language summary

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease that predominantly affects the elderly population, with rare cases reported in children and adolescents. BP is characterized by circulating antibodies that target the hemidesmosomal proteins bullous pemphigoid Antigen 1 (BPAg1 or BP230) and bullous pemphigoid Antigen 2 (BPAg2 or BP180/collagen XVII) located at the dermal-epidermal junction. The first-line treatment of BP includes topical or systemic use of corticosteroids. For refractory cases or when corticosteroids are contraindicated or to minimize adverse effects, adjunct therapies such as immunosuppressants (e.g. azathioprine, cyclosporine), dapsone, tetracycline antibiotics combined with niacinamide, intravenous immunoglobulins (IVIG), and plasma exchange are employed. Recent research has explored the use of biologic agents to treat refractory BP, including anti-CD20 monoclonal antibodies, anti-IgE monoclonal antibodies, and anti-interleukin monoclonal antibodies, showing promising outcomes. Nevertheless, effectively managing BP remains a challenge. The Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway has been implicated as a key driver in many inflammatory diseases. Recently, a class of drugs targeting this pathway, known as JAK inhibitors, has been developed. Some studies have investigated the efficacy and safety of JAK inhibitors combination with corticosteroids in BP treatment. In this retrospective study, the investigators conducted a comparative analysis to evaluate the efficacy and safety of JAK inhibitors combination With corticosteroids for patients diagnosed with moderate-to-severe bullous pemphigoid.

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