title: Churg-Strauss syndrome presenting with diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis.
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.
authors.affiliation: Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo. keishi_sugino@ybb.ne.jp
authors.forename: Keishi
authors.initials: K
authors.lastname: Sugino
authors.forename: Naoshi
authors.initials: N
authors.lastname: Kikuchi
authors.forename: Yoko
authors.initials: Y
authors.lastname: Muramatsu
authors.forename: Go
authors.initials: G
authors.lastname: Sano
authors.forename: Emiko
authors.initials: E
authors.lastname: Kusano
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Isobe
authors.forename: Susumu
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Yujiro
authors.initials: Y
authors.lastname: Takai
authors.forename: Chikako
authors.initials: C
authors.lastname: Hasegawa
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Shibuya
authors.forename: Kazuhito
authors.initials: K
authors.lastname: Hatanaka
authors.forename: Toshimasa
authors.initials: T
authors.lastname: Uekusa
authors.forename: Sakae
authors.initials: S
authors.lastname: Homma
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D003937:Diagnosis, Differential; D018450:Disease Progression; D005921:Glomerulonephritis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D006470:Hemorrhage / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011650:Pulmonary Alveoli / Q000473:pathology*
chemical_list: D000305:Adrenal Cortex Hormones