P1126PLASMAPHERESIS TREATMENT FOR SEVERE PEMPHIGUS: 10 YEARS' EXPERIENCE OF A SINGLE CENTER IN CHINA

Abstract

Abstract Background and Aims Pemphigus is a potentially life-threatening autoimmune bullous disorder. The pathogenesis involves with IgG antibodies against desmogleins (Dsg) (Dsg 1 and Dsg 3) on epithelial cell surface. Systemic corticosteroid is considered as the basis of the treatment for pemphigus. As an interventional treatment, plasmapheresis was introduced for treating severe or refractory pemphigus. The literature in this field is limited. This study aimed to review our 10 years’ experience in a single center. Method We retrospectively analyzed 17 patients with severe pemphigus who were unresponsive to high-dose prednisone and received double-filtration plasmapheresis (DFPP) treatment between January 2010 and January 2020. The information on demographic characteristics, clinical and laboratory data, treatment regimens, and clinical outcomes were collected. Results 1. Clinical characteristics: Among 17 patients, 11 patients had pemphigus vulgaris (PV), 3 patients had pemphigus foliaceus (PF), 2 patients had paraneoplastic pemphigus (PNP) and 1 patient had pemphigus herpetiform (PH). The mean age of patients was 47.8±10.0 years; 8 male patients and 9 female patients. All seventeen patients were suffering from severe pemphigus and had a period of at least one week of high-dose prednisone (1∼1.5mg/kg/d), but they were unresponsive to corticosteroid and immunosuppressants treatment. 2. As an adjuvant therapy, they received DFPP treatment with 1 to 4 sessions for each patient. Most of the patients (7 patients) received 2 sessions every other day, 5 patients received 3 sessions, 4 patients just received 1 session and only one patient received 4 sessions. For each session, the exchange volume was 1∼1.5 plasma equivalent. After DFPP treatment, the titers of Dsg antibodies significantly decreased (p<0.001, Figure 1), Nikolsky’s sign became negative and no new blisters appeared. The erosions showed reepithelialization and started healing gradually. The dosage of corticosteroid could begin to taper down rapidly in 1 to 2 weeks. On discharge, the dosage of prednisone was 0.8-1mg/kg/d. 3. As to adverse events, 4 patients had transient hypotension during the procedure, but they could recover spontaneously after parameter adjustment. No major adverse events happened. Conclusion Plasmapheresis is an effective and relatively safe treatment for severe pemphigus. Plasmapheresis can also contribute to the dosage reduction of steroid to avoid more drug-related side effect.