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

Autoimmun Rev. 2011 Sep;10(11):679-84. doi: 10.1016/j.autrev.2011.04.028. Epub 2011 May 1.

Abstract

Introduction: Therapeutic 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.

Objective: To 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.

Methods: We 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.

Results: A 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.

Conclusions: TPE 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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantibodies / blood
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / physiopathology
  • Autoimmune Diseases / therapy*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Plasma Exchange*
  • Recurrence
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Autoantibodies