JAK Inhibitors' Efficacy in Bullous Pemphigoid
NCT Number: NCT06561256
Status: COMPLETED
Study Type: OBSERVATIONAL
Conditions: Bullous Pemphigoid|Janus Kinase Inhibitor|Corticosteroids
Interventions: DRUG: Janus Kinase Inhibitor
Sponsor: Chao Ji
Sex: ALL
Age: CHILD, ADULT, OLDER_ADULT
Enrollment: 120
Study Design: Observational Model: |Time Perspective: p
Start Date: 2022-01-01
Completion Date: 2024-07-31
Locations: The First Affiliated Hospital of Fujian Medical University, Fujian, Fuzhou, 350000, China

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.

Primary Outcome Measures: Time to cease of new lesions, The time from baseline to cease of new blister formation., 2022.01.01-2024.07.31|Time to achieve minimal therapy., Minimal therapy defined as ≤ 0.1 mg/kg/d of prednisone (or equivalent) or 20 g/wk of clobetasol propionate and/or minimal adjuvant or maintenance therapy, 2022.01.01-2024.07.31|The cumulative amount of methylprednisolone (at the time of achieving minimal therapy)., The cumulative amount of methylprednisolone from baseline to the time of achieving minimal therapy, 2022.01.01-2024.07.31
Secondary Outcome Measures: The scores of BPDAI, the Bullous Pemphigoid Disease Area Index (BPDAI) scores and was classified into mild (BPDAI ≤ 19), moderate (20 ≤ BPDAI ≤ 56), and severe (BPDAI ≥ 57). This study included patients with moderate to severe BP., 2022.01.01-2024.07.31|The scores of itching Numeric Rating Scale (NRS), The patient is asked to rate their current level of itching on this 0-10 scale. 0 - No itching 1-3 - Mild itching 4-6 - Moderate itching 7-10 - Severe itching. This NRS score for itching can help healthcare providers assess the severity of the patient's itching, track changes over time, and guide appropriate treatment interventions., 2022.01.01-2024.07.31|Adverse events, All treatment-related adverse events (AEs) were assessed., 2022.01.01-2024.07.31|Clinical remissions, Partial remission on minimal therapy: presence of transient new lesions that heal within 1 week while patient is receiving minimal therapy for at least 2 months Complete remission on minimal therapy: absence of new or established lesions or pruritus while patient is receiving minimal therapy for at least 2 months Partial remission off therapy: presence of transient new lesions that heal within 1 week without treatment while patient is off all BP therapy for at least 2 months.

Complete remission off therapy: absence of new or established lesions or pruritus while patient is off all BP therapy for at least 2 months., 2022.01.01-2024.07.31