Abstract

Introduction Autoimmune diseases include a diverse and complex group of pathologies with a broad clinical spectrum due to the production of autoantibodies, which generates multisystemic compromise. Therapeutic plasma exchange (TPE) is a good additive treatment for immunosuppression due to its action over the autoantibodies. Objectives To describe the main clinical characteristics and outcomes of patients with systemic lupus erythematosus and other systemic autoimmune diseases managed with TPE. Methodology This descriptive retrospective study enrolled patients with systemic autoimmune diseases who received TPE. Results In total, 66 patients with a median age of 33.5 years (24-53 years) were included; the majority were females [n=51 (77.27%)]. Forty (60.61%) patients were diagnosed with systemic lupus erythematosus. In these cases, the main indication for TPE was diffuse alveolar hemorrhage (DAH; n=20, 30.3%) and neurolupus (n=9, 13.6%). No TPE-related deaths occurred, and the main complication was hemorrhage, without significant differences among the four types of TPE solutions used. The overall outcome was improvement in 41 (62.12%) patients. Conclusion TPE is safe and effective in patients with severe manifestations of autoimmune diseases.

Highlights

  • Autoimmune diseases include a diverse and complex group of pathologies with a broad clinical spectrum due to the production of autoantibodies, which generates multisystemic compromise

  • Among the group of autoimmune diseases with severe multisystemic compromise, systemic lupus erythematosus (SLE); antiphospholipid syndrome (APS); ANCA-positive vasculitis, such as granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis; autoimmune hemolytic anemia (AHAI); idiopathic thrombocytopenic purpura (ITP); and systemic sclerosis are well described. All these entities are treated with different immunosuppressive agents, each with different levels of evidence and outcomes, when there is severe compromise, positive outcomes have been shown following the removal of autoantibodies with therapeutic plasma exchange (TPE) procedure in critical patients [2,3,4]

  • There are approximately 179 indications in which Therapeutic plasma exchange (TPE) has been used, with different outcomes and levels of evidence according to the American Society for Apheresis (ASFA) guidelines [4]

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Summary

Introduction

Autoimmune diseases include a diverse and complex group of pathologies with a broad clinical spectrum due to the production of autoantibodies, which generates multisystemic compromise. Among the group of autoimmune diseases with severe multisystemic compromise, systemic lupus erythematosus (SLE); antiphospholipid syndrome (APS); ANCA-positive vasculitis, such as granulomatosis with polyangiitis (previously known as Wegener’s granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (previously known as ChurgStraus syndrome); autoimmune hemolytic anemia (AHAI); idiopathic thrombocytopenic purpura (ITP); and systemic sclerosis (scleroderma) are well described All these entities are treated with different immunosuppressive agents, each with different levels of evidence and outcomes, when there is severe compromise, positive outcomes have been shown following the removal of autoantibodies with therapeutic plasma exchange (TPE) procedure in critical patients [2,3,4]. The American Society for Apheresis (ASFA) guidelines of 2016, define the term plasmapheresis as “a procedure in which blood of the patient or the donor is passed through a medical device which separates plasma from other

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