Pulmonary-renal syndrome associated with antineutrophil cytoplasmic antibodies
Keywords:
ANCA, vasculitis, Wegener´s granulomatosis, microscopis polyangiitis, Churg-Strauss syndromeAbstract
The pulmonary-renal syndrome is defined by the association of
alveolar haemorrhage and rapidly progressive glomerulonephritis, resulting from several diseases and frequently associated
with serum positivity for antineutrophil cytoplasmic antibodies
(ANCA). The most common illness presenting as pulmonary-renal
syndrome is systemic vasculitis. Wegener´s granulomatosis, microscopis polyangiitis and Churg-Strauss syndrome are described
as small vessel vasculitides strongly associated with ANCA. The
diagnosis of ANCA-associated vasculitis is made on the basis of
clinical findings, by biopsy of a relevant involved organ and the
presence of ANCA. However, the application of ANCA testing as a
clinical diagnostic tool is still regarded as controversial. Accurate
understanding of the key pathogenic points of ANCA-associated
vasculitis can undoubtedly provide a more rational therapeutic
approach.
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