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Indications for plasma exchange in the treatment of polyarteritis nodosa, Churg-Strauss syndrome and other systemic vasculitides

, : Indications for plasma exchange in the treatment of polyarteritis nodosa, Churg-Strauss syndrome and other systemic vasculitides. Transfusion Science. 17(2): 211-223

Since 1977, plasma exchanges (PE) have been proposed to treat immune-mediated disease, especially when an immune complex-induced mechanism is suspected, including vasculitis and particularly polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS). The rationale for the use of PE is to remove circulating pathogenic factors, such as immune complexes and their components (antigens and antibodies). The indications of PE in the treatment of PAN without hepatitis B virus (HBV) infection and CSS have recently been clarified by two prospective therapeutic studies. There is presently no argument to support the systematic prescription of PE at the time of diagnosis of PAN without HBV infection and CSS, even for patients with factors of poor prognosis. PE may be useful as a second-line treatment in patients with refractory vasculitis and sometimes allowed, in association with corticosteroids and immunosuppressive drugs, recovery from PAN in some clinically severely compromised patients. HBV-related PAN is the best indication of PE in the treatment of PAN and is, in combination with antiviral therapy, the first-line treatment of this particular form of vasculitis. Among 41 patients with HBV-related PAN treated with PE and antiviral therapy (vidarabine in 35, interferon-alpha 2b in 6), the 7 year survival rate was 83%. HBeAg/HBeAb seroconversion was obtained in 51.2% and total virus clearance (HBeAg/HBeAb and HBsAg/HBsAb seroconversion was seen in 24.4%. These results are much better than those obtained under corticosteroids +- cyclophosphamide +- PE, with which seroconversion was rare. There are presently no convincing data to support the prescription of PE in Wegener's granulomatosis and microscopic polyangiitis, even in the more severe forms, except in dialysis-dependent patients and further prospective trials are needed to determine the therapeutic value of PE, especially for severe glomerulonephritis in anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. Side effects of PE in the treatment of PAN and CSS became more rare with technological improvements. In our experience of 182 patients with PAN and CSS treated with PE, none died during a session and the side effects and complications due to PE were usually mild and transient.


DOI: 10.1016/0955-3886(96)00013-6

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