Renal vasculitis: microscopic polyarteritis and Wegener's granuloma.
Thus, although the prognosis for small vessel vasculitis affecting the kidney has improved from almost inevitable death 30 years ago, through a 30-35% 5-year survival 10-15 years ago, many problems remain. Although it seems likely that the more intense immunosuppression used in most units over the past 10-15 years has improved immediate survival, a number of these elderly and often frail patients die directly as a result of these treatment regimens; in our present series, 5/16 deaths (31%) could be related directly to immunosuppression. In some patients extrarenal complications of the vasculitis, particularly in the gastrointestinal tract, still may lead to early death. The relative roles (if any) of methylprednisolone and/or plasma exchange in treatment of renal vasculitis are not clear yet, although a recent controlled trial in patients with crescentic nephritis, mainly the result of small vessel renal vasculitis, showed a modest benefit from plasma exchange in addition to prednisolone and cyclophosphamide, but only in those patients requiring dialysis. In the longer term, we do not know for how long, with what agent and with what intensity immunosuppression must be maintained. In our series, in which most patients were maintained on modest immunosuppression for many years, relapse of the vasculitis was almost absent, suggesting some merit in this strategy. We used azathioprine rather than intermittent intravenous cyclophosphamide, as others have advocated; there are no data to choose between these regimens at the moment, although even in this relatively elderly population long-term oral cyclophosphamide is better avoided because of risks to the bladder, gonadal toxicity and oncogenesis.(ABSTRACT TRUNCATED AT 250 WORDS)
Guy's Campus UMDS, London, UK.
J S
JS
Cameron
D000368:Aged; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010488:Polyarteritis Nodosa / Q000473:pathology / Q000628:therapy*