title: Renal crisis due to intimal hyperplasia in a patient with mixed connective tissue disease (MCTD) accompanied by pulmonary hypertension.
abstract: We report the case of a young female patient with mixed connective tissue disease (MCTD). She had marked pulmonary hypertension (PH) without lung fibrosis. She developed renal crisis after delivery by caesarean section. Renal biopsy revealed severe renal intimal hyperplasia with mild glomerular changes. The combination of intravenous pulse high-dose corticosteroid and cyclophosphamide (CPA) infusion and subsequent corticosteroid oral administration rescued her from renal crisis. This suggests that the possibility of co-incident renal intimal hyperplasia should be considered in patients with MCTD accompanied by PH. In addition, there might be some clinical benefit in combining high-dose corticosteroid with CPA infusion in the treatment of renal crisis due to intimal hyperplasia in MCTD.
authors.affiliation: Department of Molecular Medicine and Obstetrics Osaka University Medical School, Suita.
authors.forename: T
authors.initials: T
authors.lastname: Yamaguchi
authors.forename: S
authors.initials: S
authors.lastname: Ohshima
authors.forename: T
authors.initials: T
authors.lastname: Tanaka
authors.forename: S
authors.initials: S
authors.lastname: Tsukada
authors.forename: M
authors.initials: M
authors.lastname: Matsushita
authors.forename: S
authors.initials: S
authors.lastname: Kohmo
authors.forename: T
authors.initials: T
authors.lastname: Kanzaki
authors.forename: Y
authors.initials: Y
authors.lastname: Saeki
mesh_terms: D058186:Acute Kidney Injury / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002585:Cesarean Section / Q000009:adverse effects*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006965:Hyperplasia / Q000276:immunology; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008947:Mixed Connective Tissue Disease / Q000150:complications*; D011247:Pregnancy; D012077:Renal Artery / Q000473:pathology*; D013256:Steroids; D016896:Treatment Outcome; D017539:Tunica Intima / Q000473:pathology*
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide