Comparison of Guidelines for Management of Pemphigus: a Review of Systemic Corticosteroids, Rituximab, and Other Immunosuppressive Therapies

• Published in: Clinical reviews in allergy & immunology Vol. 61; no. 3; pp. 351 - 362
• Main Authors: Zhao, Wenzhe, Wang, Jingying, Zhu, Haiqin, Pan, Meng
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.12.2021; Springer; Springer Nature B.V
• Subjects: Adrenal Cortex Hormones - therapeutic use; Allergology; Autoimmune diseases; Azathioprine; B cells; Care and treatment; CD20 antigen; Comparative analysis; Corticosteroids; Humans; Immunology; Immunosuppression Therapy; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Immunotherapy; Internal Medicine; Lymphocytes B; Medicine; Medicine & Public Health; Monoclonal antibodies; Mucosa; Mycophenolate mofetil; Mycophenolic acid; Pemphigus; Pemphigus - drug therapy; Practice Guidelines as Topic; Rituximab; Rituximab - therapeutic use; Skin; Skin diseases; Steroids; Rituximab; Corticosteroids; Pemphigus; Guidelines
• ISSN: 1080-0549; 1559-0267
• DOI: 10.1007/s12016-021-08882-1

Pemphigus is a severe autoimmune bullous dermatosis that affects the skin and/or mucosa, and it may be life-threatening without proper treatment. The guidelines and/or consensus statements for treatment vary widely between groups. We selected 6 guidelines and consensus statements established by different associations about the management of pemphigus vulgaris (PV) and/or pemphigus foliaceus (PF) to review, compare, and contrast the similarities and differences of these recommendations and provide optimal management suggestions to physicians. Corticosteroids remain a first-line therapy for pemphigus, but there are many differences in initial dose, tapering schedule, and management of relapse between different guidelines. Rituximab is a monoclonal antibody targeting CD20-positive B lymphocytes that is approved as a first-line therapy in moderate-to-severe pemphigus. Immunosuppressive agents, such as azathioprine (AZA) and mycophenolate mofetil (MMF), are also widely used as corticosteroid-sparing drugs, but the adjuvant applications and dosage regimens of different recommendations are not standardized. We attribute these differences to the clinical scoring adopted, the standards for disease severity evaluation, the publication year of each guideline, and local and regional healthcare differences.