Low-dose rituximab as an adjuvant therapy in pemphigus

• Published in: Indian journal of dermatology, venereology, and leprology Vol. 83; no. 3; pp. 317 - 325
• Main Authors: Gupta, Jaya, Raval, Ranjan C, Shah, Arti N, Solanki, Rekha B, Patel, Dhara D, Shah, Kaksha B, Badheka, Ami D, Shah, Keyur B, Aggarwal, Neetish K, Ravishankar, Vaaruni
• Format: Journal Article
• Language: English
• Published: India Medknow Publications and Media Pvt. Ltd 01.05.2017; Scientific Scholar
• Subjects: Adult; Aged; Antineoplastic Agents, Immunological - administration & dosage; Arthritis; Autoimmune diseases; Chemotherapy, Adjuvant - methods; Child; Dermatology; Dosage and administration; Dose-Response Relationship, Drug; Drug Administration Schedule; Drug therapy; Female; Hepatitis; Hospitals; Humans; Immunosuppressive agents; Immunotherapy; Infections; Lymphoma; Male; Middle Aged; Monoclonal antibodies; Neutropenia; Pemphigus; Pemphigus - diagnosis; Pemphigus - drug therapy; Rituximab - administration & dosage; Skin diseases; Targeted cancer therapy; Urticaria; Viral infections; India
• ISSN: 0378-6323; 0973-3922; 1998-3611
• DOI: 10.4103/ijdvl.IJDVL_1078_14

Pemphigus is a chronic autoimmune blistering disease where systemic steroids and immunosuppressants are the mainstay of therapy, but long-term treatment with these agents is associated with many side effects. Rituximab, a chimeric monoclonal anti-CD20 antibody, in low doses has shown efficacy as an adjuvant to reduce the dose of steroids. To study the clinical efficacy and safety of low-dose rituximab as an adjuvant therapy in pemphigus. Fifty patients with extensive pemphigus were selected, who either had recalcitrant pemphigus, were steroid dependent, had relapsed after pulse therapy, had anti-desmoglein levels >20, had contraindications to conventional treatment or wanted to avoid conventional treatment and its side effects. Two doses of rituximab (500 mg) were given 2 weeks apart and patients were regularly followed up every 2 weeks for 3 months and then monthly upto 2 years. Complete blood counts, liver function tests, renal function tests, skin biopsy, direct immunofluorescence and desmoglein levels were checked before and after rituximab administration. Pre-rituximab chest X-ray and electrocardiograph were also obtained. At 3 months, 41 (82%) patients showed complete remission. Nine (18%) patients had partial remission. After 6-12 months, 20 (40% of enrolled patients) continued to be in remission and were off all systemic therapy and the remaining 19 (38%) were continuing to take low doses of steroids with or without other adjuvant immunosuppressants and 2 (4%) had to be given another 2 doses of rituximab and subsequently could be managed with low-dose steroids. Of the 9 patients in partial remission at 3 months, after 6-12 months 5 (10% of the total) were completely off treatment and went into complete remission and 4 (8%) were on additional treatment out of which 2 (4%) had to be given 2 additional doses of rituximab and were in partial remission with low-dose therapy at the end of 12 months. One patient developed urticaria as a side effect. Another developed herpes zoster. Our results show that low-dose rituximab is a well-tolerated and beneficial adjuvant therapy in recalcitrant pemphigus which helps reduce both the severity of disease as well as the dose of steroids and immunosuppressants.