JAK Inhibitors' Efficacy in Bullous Pemphigoid

NCT06561256 | Completed

• 1 other identifier: FirstAHFujian123
• Study Type: observational
• Target: 120
• Locations: 1 country
• Sites: 1

Brief 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.

Conditions

Bullous Pemphigoid; Janus Kinase Inhibitor; Corticosteroids

Outcome Measures

Primary Outcomes (3)

• 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 Outcomes (4)

• The scores of BPDAI

  2022.01.01-2024.07.31

• The scores of itching Numeric Rating Scale (NRS)

  2022.01.01-2024.07.31

• Adverse events

  2022.01.01-2024.07.31

• Clinical remissions

  2022.01.01-2024.07.31

Interventions

Janus Kinase Inhibitor DRUG

Janus Kinase Inhibitor in combination with corticosteroids

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

All enrolled patients will be followed for a duration of 48 weeks. This study will include approximately 300 patients with moderate to severe BP. The Primary outcomes were the time to cease of new lesions, time to achieve minimal therapy, the cumulative amount of corticosteroids (at the time of achieving minimal therapy). The Secondary outcomes were the scores of BPDAI (week 0, week 2, and week 4). the scores of itching Numeric Rating Scale (NRS) (week 0, week 2, and week 4). the level of IgE (week 0, week 2) and eosinophil percentage (Eos%) (week 0, week 2). the clinical remissions at week 24 and week 48. Adverse events: all treatment-related adverse events (AEs) were assessed.

You may qualify if:

• Diagnosis of Bullous pemphigoid;
• Moderate to severe bullous pemphigoid； The disease severity was measured using the Bullous Pemphigoid Disease Area Index (BPDAI) score and was classified into mild (BPDAI ≤ 19), moderate (20 ≤ BPDAI ≤ 56), and severe (BPDAI ≥ 57).
• Moderate to severe BP received JAK Inhibitors combination with corticosteroids therapy；

You may not qualify if:

• Patients diagnosed with malignant neoplasms
• Previous administration with biologics within 3 months

Sponsors & Collaborators

• Chao Ji: lead

Study Sites (1)

The First Affiliated Hospital of Fujian Medical University

Fujian, Fuzhou, 350000, China

Location

MeSH Terms

Conditions

Pemphigoid, Bullous

Interventions

Janus Kinase Inhibitors

Condition Hierarchy (Ancestors)

Skin Diseases, Vesiculobullous; Skin Diseases; Skin and Connective Tissue Diseases; Autoimmune Diseases; Immune System Diseases

Intervention Hierarchy (Ancestors)

Protein Kinase Inhibitors; Enzyme Inhibitors; Molecular Mechanisms of Pharmacological Action; Pharmacologic Actions; Chemical Actions and Uses

Study Officials

• Chao Ji, PhD

  First Affiliated Hospital of Fujian Medical University

  STUDY CHAIR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Clinical Professor

Study Record Dates

• First Submitted: August 15, 2024
• First Posted: August 20, 2024
• Study Start: January 1, 2022
• Primary Completion: July 31, 2024
• Study Completion: July 31, 2024
• Last Updated: September 5, 2024

Record last verified: 2024-08

Data Sharing

• IPD Sharing: Will not share

Locations

CN (1)