title: A case of Mikulicz's disease complicated with interstitial nephritis successfully treated by high-dose corticosteroid.
abstract: A 40-year-old woman who had bilateral swelling in the eyelids and submandibular region was admitted. Clinical findings suggested she had primary Sjögren's syndrome. Laboratory data showed glucosuria, positive CRP (0.50 mg/dl), liver dysfunction (AST 53 U/l, ALT 101 U/l, gamma-GTP 241 U/l, ALP 914 U/l, LAP 496 U/l), hyperglycemia, hypergammaglobulinemia (IgG 3450 mg/dl, IgA 91 mg/dl, IgM 80 mg/dl), hypocomplementemia (C3 73 mg/dl, C4 2 mg/dl, CH50 < 19.0 U/ml), renal tubular dysfunction (urine N-acetyl-beta-D: -glucosaminidase 8.6 U/l, urine (beta2)-microglobulin 83 microg/l), and urinary concentration defect. Ammonium chloride loading test was normal. Gallium-67 scintigram indicated abnormal uptake in bilateral lacrimal glands, submandibular glands, and kidneys. A diagnosis of Mikulicz's disease and interstitial nephritis was made, since biopsy specimens of her lacrimal gland and minor salivary gland showed diffuse infiltration of lymphocytes. Renal biopsy specimens also showed severe interstitial infiltration of lymphocytes. Symptoms and laboratory data normalized in response to methylprednisolone pulse therapy and prednisolone 60 mg/day. This case of Mikulicz's disease complicated with interstitial nephritis was successfully treated by high-dose corticosteroid.
authors.affiliation: Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa, 920-0293, Japan. delph@hama-med.ac.jp
authors.forename: Kumiko
authors.initials: K
authors.lastname: Shimoyama
authors.forename: Noriyoshi
authors.initials: N
authors.lastname: Ogawa
authors.forename: Toshioki
authors.initials: T
authors.lastname: Sawaki
authors.forename: Hiromi
authors.initials: H
authors.lastname: Karasawa
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Masaki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawabata
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Fukushima
authors.forename: Yuji
authors.initials: Y
authors.lastname: Wano
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hirose
authors.forename: Hisanori
authors.initials: H
authors.lastname: Umehara
mesh_terms: D000328:Adult; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007668:Kidney / Q000473:pathology; D007765:Lacrimal Apparatus / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000008:administration & dosage*; D008882:Mikulicz' Disease / Q000150:complications / Q000188:drug therapy; D009395:Nephritis, Interstitial / Q000188:drug therapy / Q000209:etiology; D011241:Prednisone / Q000008:administration & dosage*; D012469:Salivary Glands / Q000473:pathology
chemical_list: D005938:Glucocorticoids; D011241:Prednisone; D008775:Methylprednisolone