F. Lhote et L. Guillevin, INDICATIONS FOR PLASMA-EXCHANGE IN THE TREATMENT OF POLYARTERITIS-NODOSA, CHURG-STRAUSS-SYNDROME AND OTHER SYSTEMIC VASCULITIDES, Transfusion science, 17(2), 1996, pp. 211-223
Since 1977, plasma exchanges (PE) have been proposed to treat immune-m
ediated disease, especially when an immune complex-induced mechanism i
s suspected, including vasculitis and particularly polyarteritis nodos
a (PAN) and Churg-Strauss syndrome (CSS). The rationale for the use of
PE is to remove circulating pathogenic factors, such as immune comple
xes 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 st
udies. There is presently no argument to support the systematic prescr
iption 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 usef
ul as a second-line treatment in patients with refractory vasculitis a
nd sometimes allowed, in association with corticosteroids and immunosu
ppressive drugs, recovery from PAN in some clinically severely comprom
ised patients. HBV-related PAN is the best indication of PE in the tre
atment of PAN and is, in combination with antiviral. therapy, the firs
t-line treatment of this particular form of vasculitis. Among 41 patie
nts with HBV-related PAN treated with PE and antiviral therapy (vidara
bine in 35, interferon-alpha 2b in 6), the 7 year survival rate was 83
%. HBeAg/HBeAb seroconversion was obtained in 51.2% and clearance (HBe
Ag/HBeAb HBsAg/HBsAb seroconversions) was seen in 24.4%. These results
are much better than those obtained under corticosteroids +/- cycloph
osphamide +/- PE, with which seroconversion was rare. There are presen
tly no convincing data to support the prescription of PE in Wegener's
granulomatosis and microscopic polyangiitis, even in the more severe f
orms, except in dialysis-dependent patients and further prospective tr
ials are needed to determine the therapeutic value of PE, especially f
or severe glomerulonephritis in anti-neutrophil cytoplasmic antibody-a
ssociated systemic vasculitis. Side effects of PE in the treatment of
PAN and CSS became more rare with technological improvements. In our e
xperience 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. Copyright (C) 1996 Elsevier Science Ltd.