Indication for plasma exchange for systemic necrotizing vasculidities

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Abstract

Plasma exchanges (PE) are a component of regimens prescribed to treat systemic necrotizing vasculidities. They are also part of the best therapeutic strategy for virus-induced vasculidities. The combination of antiviral agents and PE has proven efficacy against polyarteritis nordosa. This strategy is also effective for human immunodeficiency virus-associated vasculitis and, unlike cytotoxic agents, does not jeopardize the outcome of acquired immunodeficiency syndrome. Concerning the vasculitis seen in the context of hepatitis C virus-related cryoglobulinemia, PE contribute to better outcomes but, because of the poor efficacies of antiviral drugs, only about half of the patients achieve definitive recovery and relapses are frequent. The use of PE to treat antineutrophil cytoplasm antibody-associated vasculidities with severe renal insufficiency leads to improved renal function and thus fewer patients require dialysis. Although PE does not improve survival, their adjunction to corticosteroids and immunosuppressants for patients with alveolar hemorrhage could also limit the severity of this severe manifestation.

Introduction

Plasma exchanges (PE) are frequently included in regimens to treat vasculidities but the indications need to be codified. Results from several prospective trials conducted of the past decade have answered some questions and we are now able to specify when PE are useful during the course of vasculitis.

They are indicated in vasculidities secondary to virus infections, and those associated with antineutrophil cytoplasm antibodies (ANCA) and severe renal insufficiency. Herein, we review the current indications of PE in various systemic necrotizing vasculidities, associated or not with viral infections.

Section snippets

Virus-associated vasculidities

Viruses have been proven to be the etiological agent responsible for several vasculidities, which can affect vessels of various calibers and are usually not associated with ANCA. Two major vasculidities are the consequence of viral infection: hepatitis B virus (HBV) is responsible for classic polyarteritis nordosa (PAN) [1] and hepatitis C virus (HCV) for mixed cryoglobulinemia [2]. Other viruses can also be associated, albeit less frequently, with the occurrence of vasculitis, for example,

Anca-associated vasculidities

Combining corticosteroids and cyclophosphamide are regimens common to every case of Wegener’s granulomatosis, and indicated for the majority of patients with microscopic polyangitis. In Wegener’s granulomatosis, a consensus has not been reached concerning cyclophosphamide, even though its indication is universally accepted. Oral administration is prescribed at 2 mg/kg/d [14]. The dose can be adapted according to the therapeutic response, the occurrence of side effects, renal function and age.

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