Therapeutic Plasma Exchange in Systemic Vasculitis
Therapeutic Plasma Exchange in Systemic Vasculitis
Purpose of review: Therapeutic plasma exchange has been long proposed as a potentially useful modality to treat several systemic vasculitis conditions. This review summarizes the available evidence for the effectiveness of plasma exchange in systemic vasculitis.
Recent findings: Therapy with plasma exchange, most often combined with immunosuppressive agents, has been found effective for antiglomerular basement membrane disease, antineutrophil cytoplasmic antibody-associated vasculitis (AAV), Henoch–Schönlein purpura, cryoglobulinemic vasculitis, hepatitis B virus-associated polyarteritis nodosa and Kawasaki disease. The most common indications are life-threatening or organ function-threatening manifestations, particularly advanced renal dysfunction and disease refractory to traditional therapy. Thus, most of the available evidence favoring plasma exchange in these circumstances is from small observational studies or expert consensus. Recent advances in findings include results strengthening the notion of a small beneficial effect on preserving renal function with adjunct plasma exchange therapy in AAV with renal failure and observational data suggesting plasma exchange as a promising effective salvage option for children with Kawasaki disease not responding to standard therapy with intravenous immunoglobulins.
Summary: Evidence from case reports and case series and a few randomized controlled trials continues to support plasma exchange as a major rescue-treatment modality for several systemic vasculitis diseases. These studies offer some guidance for use of plasma exchange in systemic vasculitis, but additional data from controlled trials are needed for more accurate assessment of the indications, practical modalities, benefits and shortcomings of this treatment approach.
Systemic vasculitis is a family of diseases of mostly unknown cause that are characterized by bloodvessel inflammation occurring in multiple organs or tissues. The site of predominant vessel inflammation, associated histopathological features and underlying pathogeneses vary greatly across the systemic vasculitis diseases, which results in substantial demographic, clinical and prognostic heterogeneity. Immunosuppressive agents represent the mainstay of treatment, but therapeutic modalities differ across conditions and sometimes even within given diseases for subgroups of distinct severity.
The potential utility of therapeutic plasma exchange for treating systemic vasculitis has been discussed for more than three decades. The general principle of plasma exchange relies on the removal from blood of various substances (e.g. antibodies, immune complexes, paraproteins or cholesterol) that cannot be eliminated by hemofiltration or hemodialysis because their molecular weight is greater than that of albumin. In addition, the purification by plasma exchange of smaller molecules, such as cytokines or activated complement components, may be beneficial. In systemic vasculitis, use of plasma exchange is based on the understanding that many of the diseases are immune-complex-mediated or autoimmune diseases, but plasma exchange has also been used for diseases not clearly within these categories. Because plasma exchange essentially has a symptomatic effect, plasma exchange in systemic vasculitis is a sound choice for self-limited diseases, acute life-threatening or function-threatening manifestations requiring rapid therapeutic intervention, or as adjunct escalating therapy for disease unresponsive to conventional treatment.
In this review, we briefly revisit the basic elements of the plasma exchange technique and summarize the current knowledge of the clinical utility of plasma exchange for treating systemic vasculitis. Emphasis is placed on the most recent findings. The considered diseases are separated into two categories according to whether or not their pathogenesis is immune-complex or autoimmune mediated.
Abstract and Introduction
Abstract
Purpose of review: Therapeutic plasma exchange has been long proposed as a potentially useful modality to treat several systemic vasculitis conditions. This review summarizes the available evidence for the effectiveness of plasma exchange in systemic vasculitis.
Recent findings: Therapy with plasma exchange, most often combined with immunosuppressive agents, has been found effective for antiglomerular basement membrane disease, antineutrophil cytoplasmic antibody-associated vasculitis (AAV), Henoch–Schönlein purpura, cryoglobulinemic vasculitis, hepatitis B virus-associated polyarteritis nodosa and Kawasaki disease. The most common indications are life-threatening or organ function-threatening manifestations, particularly advanced renal dysfunction and disease refractory to traditional therapy. Thus, most of the available evidence favoring plasma exchange in these circumstances is from small observational studies or expert consensus. Recent advances in findings include results strengthening the notion of a small beneficial effect on preserving renal function with adjunct plasma exchange therapy in AAV with renal failure and observational data suggesting plasma exchange as a promising effective salvage option for children with Kawasaki disease not responding to standard therapy with intravenous immunoglobulins.
Summary: Evidence from case reports and case series and a few randomized controlled trials continues to support plasma exchange as a major rescue-treatment modality for several systemic vasculitis diseases. These studies offer some guidance for use of plasma exchange in systemic vasculitis, but additional data from controlled trials are needed for more accurate assessment of the indications, practical modalities, benefits and shortcomings of this treatment approach.
Introduction
Systemic vasculitis is a family of diseases of mostly unknown cause that are characterized by bloodvessel inflammation occurring in multiple organs or tissues. The site of predominant vessel inflammation, associated histopathological features and underlying pathogeneses vary greatly across the systemic vasculitis diseases, which results in substantial demographic, clinical and prognostic heterogeneity. Immunosuppressive agents represent the mainstay of treatment, but therapeutic modalities differ across conditions and sometimes even within given diseases for subgroups of distinct severity.
The potential utility of therapeutic plasma exchange for treating systemic vasculitis has been discussed for more than three decades. The general principle of plasma exchange relies on the removal from blood of various substances (e.g. antibodies, immune complexes, paraproteins or cholesterol) that cannot be eliminated by hemofiltration or hemodialysis because their molecular weight is greater than that of albumin. In addition, the purification by plasma exchange of smaller molecules, such as cytokines or activated complement components, may be beneficial. In systemic vasculitis, use of plasma exchange is based on the understanding that many of the diseases are immune-complex-mediated or autoimmune diseases, but plasma exchange has also been used for diseases not clearly within these categories. Because plasma exchange essentially has a symptomatic effect, plasma exchange in systemic vasculitis is a sound choice for self-limited diseases, acute life-threatening or function-threatening manifestations requiring rapid therapeutic intervention, or as adjunct escalating therapy for disease unresponsive to conventional treatment.
In this review, we briefly revisit the basic elements of the plasma exchange technique and summarize the current knowledge of the clinical utility of plasma exchange for treating systemic vasculitis. Emphasis is placed on the most recent findings. The considered diseases are separated into two categories according to whether or not their pathogenesis is immune-complex or autoimmune mediated.
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