Churg-Strauss syndrome presenting with pulmonary capillaritis and diffuse alveolar hemorrhage

Churg-Strauss syndrome (CSS) is a rare disorder characterized by the histopathological triad of systemic necrotizing vasculitis, extravascular granuloma, and eosinophilic infiltrate, occurring in individuals with asthma or a history of allergy. We report a case of CSS with rare presentation of diffu...

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Bibliographic Details
Published in:Scandinavian journal of rheumatology 1998, Vol.27 (3), p.230-232
Main Authors: LAI, R.-S, LIN, S.-L, LAI, N.-S, LEE, P.-C
Format: Article
Language:English
Subjects:
Adult
Antibodies, Antineutrophil Cytoplasmic - blood
Autoantigens - blood
Biological and medical sciences
Capillaries - pathology
Churg-Strauss Syndrome - blood
Churg-Strauss Syndrome - complications
Churg-Strauss Syndrome - pathology
Hemorrhage - etiology
Hemorrhage - pathology
Humans
Lung - blood supply
Lung - pathology
Lung Diseases - etiology
Lung Diseases - pathology
Male
Medical sciences
Myeloblastin
Pulmonary Alveoli - pathology
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Serine Endopeptidases - blood
Vasculitis - etiology
Vasculitis - pathology
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Summary:Churg-Strauss syndrome (CSS) is a rare disorder characterized by the histopathological triad of systemic necrotizing vasculitis, extravascular granuloma, and eosinophilic infiltrate, occurring in individuals with asthma or a history of allergy. We report a case of CSS with rare presentation of diffuse pulmonary hemorrhage. A transbronchial lung biopsy showed necrotizing vasculitis, pulmonary capillaritis, and prominent eosinophilic infiltrate. At autopsy, necrotizing granuloma and diffuse alveolar hemorrhage were found in the lungs. Laboratory data showed hypereosinophilia, disseminated intravascular coagulopathy, and positive anti-proteinase 3 antineutrophil cytoplasmic antibody (ANCA). This case emphasize that CSS should be considered in the differential diagnosis of ANCA-positive vasculitis with diffuse alveolar hemorrhage.
ISSN:0300-9742
1502-7732
DOI:10.1080/030097498440877