Therapeutic plasma exchange for the management of refractory systemic autoimmune diseases: Report of 31 cases and review of the literature

IntroductionTherapeutic plasma exchange (TPE) represents a treatment option in patients with systemic autoimmune diseases because most of their clinical manifestations are related to the presence of antibodies or immune complex deposition.ObjectiveTo describe the main demographic and clinical characteristics as well as the outcome of patients with systemic autoimmune diseases treated with TPE at a tertiary care center.MethodsWe included all patients with systemic autoimmune diseases in whom the indication for treatment with TPE was a flare of the disease between 1999 and 2010. The indications for treatment, complications and outcomes were obtained from review of medical records.ResultsA total of 31 patients (18 (58%) females and 13 (42%) males) were treated with a total of 196 TPE sessions with an average of 6.3 sessions per patient. Mean age at the time of TPE was 52.9 years (range, 26.0–82.0 years). Ten (32.3%) patients had ANCA-associated vasculitides, 6 (19.4%) mixed cryoglobulinemia secondary to hepatitis C virus (HCV) infection, 5 (16.1%) essential mixed cryoglobulinemia, 4 (12.9%) catastrophic antiphospholipid syndrome, 3 (9.7%) systemic lupus erythematosus, 2(6.5%) dermatomyositis and 1 (3.1%) polyarteritis nodosa. All patients except one were receiving corticosteroids at varying doses and all received a concomitant immunosuppressive drug. Ten (32.3%) and 9 (29.0%) patients received rituximab and intravenous immunoglobulins prior to TPE, respectively.Six patients experienced catheter-related infections, 5 urinary infections and 3 patients developed hospital acquired pneumonia. Eleven patients died in spite of the TPE.ConclusionsTPE is an effective therapeutic option for treating serious manifestations of systemic autoimmune diseases and a valid option for those patients with refractory disease to conventional treatments.