Rs. Lai et al., CHURG-STRAUSS-SYNDROME PRESENTING WITH PULMONARY CAPILLARITIS AND DIFFUSE ALVEOLAR HEMORRHAGE, Scandinavian journal of rheumatology, 27(3), 1998, pp. 230-232
Churg-Strauss syndrome (CS) is a rare disorder characterized by the hi
stopathological triad of systemic necrotizing vasculitis, extravascula
r granuloma, and eosinophilic infiltrate, occurring in individuals wit
h asthma or a history of allergy. We report a case of CSS with rare pr
esentation of diffuse pulmonary hemorrhage. A transbronchial lung biop
sy showed necrotizing vasculitis, pulmonary capillaritis, and prominen
t eosinophilic infiltrate. At autopsy, necrotizing granuloma and diffu
se alveolar hemorrhage were found in the lungs. Laboratory data showed
hypereosinophilia, disseminated intravascular coagulopathy, and posit
ive anti-proteinase 3 antineutrophil cytoplasmic antibody (ANCA). This
case emphasize that CSS should be considered in the differential diag
nosis of ANCA-positive vasculitis with diffuse alveolar hemorrhage.