CHURG-STRAUSS-SYNDROME PRESENTING WITH PULMONARY CAPILLARITIS AND DIFFUSE ALVEOLAR HEMORRHAGE

Citation
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
Citations number
14
Categorie Soggetti
Rheumatology
ISSN journal
03009742
Volume
27
Issue
3
Year of publication
1998
Pages
230 - 232
Database
ISI
SICI code
0300-9742(1998)27:3<230:CPWPCA>2.0.ZU;2-H
Abstract
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.