Churg-Strauss Syndrome Presenting with Diffuse Alveolar Hemorrhage and Rapidly Progressive Glomerulonephritis
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概要
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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.
著者
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SHIBUYA Kazutoshi
Department of Surgical Pathology, Toho University School of Medicine
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Sugino Keishi
Department of Respiratory Medicine, Toho University Omori Medical Center
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Kikuchi Naoshi
Department of Respiratory Medicine, Toho University Omori Medical Center
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Muramatsu Yoko
Department of Respiratory Medicine, Toho University Omori Medical Center
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Sano Go
Department of Respiratory Medicine, Toho University Omori Medical Center
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Kusano Emiko
Department of Respiratory Medicine, Toho University Omori Medical Center
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Isobe Kazutoshi
Department of Respiratory Medicine, Toho University Omori Medical Center
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Sakamoto Susumu
Department of Respiratory Medicine, Toho University Omori Medical Center
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Takai Yujiro
Department of Respiratory Medicine, Toho University Omori Medical Center
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Hasegawa Chikako
Department of Pathology, Toho University Omori Medical Center
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Hatanaka Kazuhito
Department of Pathology, Labor Health and Welfare Organization Kanto Rosai Hospital
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Uekusa Toshimasa
Department of Pathology, Labor Health and Welfare Organization Kanto Rosai Hospital
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Homma Sakae
Department of Respiratory Medicine, Toho University Omori Medical Center
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Sano Go
Department Of Respiratory Medicine Toho University Medical Center
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Sugino Keishi
東邦大学医療センター大森病院 呼吸器内科
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Sugino Keishi
Department Of Respiratory Medicine Toho University Medical Center
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Uekusa Toshimasa
Department Of Laboratory Medicine Kanto Rousai Hospital
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Hasegawa Chikako
Department Of Pathology Omori Hospital Toho University School Of Medicine
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Muramatsu Yoko
Department Of Geophysics Kyoto University
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Hatanaka Kazuhito
Department Of Forensic Medicine Graduate School Of Medicine University Of Tokyo
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Homma Sakae
Department Of Respiratory Medicine Respiratory Center
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Sakamoto Susumu
Department Of Cardiology Akashi Medical Center
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Shibuya Kazutoshi
Department Of Clinical Pathology School Of Medicine Toho University
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Takai Yujiro
Department Of Respiratory Medicine Toho University Omori Medical Center
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Isobe Kazutoshi
Department Of Respiratory Medicine Toho University Omori Medical Center
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