Churg-Strauss Syndrome Presenting with Diffuse Alveolar Hemorrhage and Rapidly Progressive Glomerulonephritis

Keishi Sugino; Naoshi Kikuchi; Yoko Muramatsu; Go Sano; Emiko Kusano; Kazutoshi Isobe; Susumu Sakamoto; Yujiro Takai; Chikako Hasegawa; Kazutoshi Shibuya; Kazuhito Hatanaka; Toshimasa Uekusa; Sakae Homma

doi:10.2169/internalmedicine.48.2356

CASE REPORTS

Churg-Strauss Syndrome Presenting with Diffuse Alveolar Hemorrhage and Rapidly Progressive Glomerulonephritis

Keishi Sugino, Naoshi Kikuchi, Yoko Muramatsu, Go Sano, Emiko Kusano, Kazutoshi Isobe, Susumu Sakamoto, Yujiro Takai, Chikako Hasegawa, Kazutoshi Shibuya, Kazuhito Hatanaka, Toshimasa Uekusa, Sakae Homma

Author information

Keishi Sugino
Department of Respiratory Medicine, Toho University Omori Medical Center
Naoshi Kikuchi
Department of Respiratory Medicine, Toho University Omori Medical Center
Yoko Muramatsu
Department of Respiratory Medicine, Toho University Omori Medical Center
Go Sano
Department of Respiratory Medicine, Toho University Omori Medical Center
Emiko Kusano
Department of Respiratory Medicine, Toho University Omori Medical Center
Kazutoshi Isobe
Department of Respiratory Medicine, Toho University Omori Medical Center
Susumu Sakamoto
Department of Respiratory Medicine, Toho University Omori Medical Center
Yujiro Takai
Department of Respiratory Medicine, Toho University Omori Medical Center
Chikako Hasegawa
Department of Pathology, Toho University Omori Medical Center
Kazutoshi Shibuya
Department of Pathology, Toho University Omori Medical Center
Kazuhito Hatanaka
Department of Pathology, Labor Health and Welfare Organization Kanto Rosai Hospital
Toshimasa Uekusa
Department of Pathology, Labor Health and Welfare Organization Kanto Rosai Hospital
Sakae Homma
Department of Respiratory Medicine, Toho University Omori Medical Center

Keywords: Churg-Strauss syndrome, rapidly progressive glomerulonephritis, diffuse alveolar hemorrhage, myeloperoxidase-antineutrophil cytoplasmic antibody

JOURNAL OPEN ACCESS

2009 Volume 48 Issue 20 Pages 1807-1811

DOI https://doi.org/10.2169/internalmedicine.48.2356

Details

Abstract

A 46-year-old man with a 4-month history of bronchial asthma was admitted to our hospital complaining of progressive dyspnea, weakness of the lower extremities, multiple truncal erythematous purpura and hemoptysis. Neurological examination identified the presence of mononeuritis multiplex. Laboratory data indicated marked anemia, eosinophilia, severe renal failure with nephrotic condition and elevated serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) levels (1,050 EU). Chest computed tomography showed diffuse ground glass opacity in both lungs. Bronchoalveolar lavage fluid revealed bloody fluid with eosinophilia (81%). Microscopic findings of a transbronchial lung biopsy were consistent with alveolar hemorrhage. A skin biopsy revealed eosinophilic vasculitis consistent with Churg-Strauss syndrome (CSS). A renal biopsy specimen revealed pauci-immune crescentic necrotizing glomerulonephritis. Consequently, he was diagnosed as having CSS presenting with diffuse alveolar hemorrhage (DAH) and rapidly progressive glomerulonephritis (RPGN) with MPO-ANCA-associated systemic vasculitis. His clinical condition markedly improved with the administration of intravenous corticosteroid (CS) and cyclophosphamide (CY). Thus, we report a case of CSS presenting with the rare complication of DAH and RPGN.

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