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

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 diff...

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Bibliographic Details
Published in:Clinical reviews in allergy & immunology 2021-12, Vol.61 (3), p.351-362
Main Authors: Zhao, Wenzhe, Wang, Jingying, Zhu, Haiqin, Pan, Meng
Format: Article
Language:English
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
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Summary: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.
ISSN:1080-0549
1559-0267
DOI:10.1007/s12016-021-08882-1