[A Case of Sjogren's Syndrome Complicated with Cryoglobulinemia, Nephrogenic Diabetes Insipidus, and Renal Tubular Acidosis] (Japanese)

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Title: [A Case of Sjogren's Syndrome Complicated with Cryoglobulinemia, Nephrogenic Diabetes Insipidus, and Renal Tubular Acidosis] (Japanese)
Authors: Koura, T.; Nishinarita, S.; Matsukawa, Y.; Kobayashi, Tatsuya; Shimada, H.; Takei, M.; Tomita, Y.; Baba, M.; Hayama, T.; Hashimoto, S.
Publisher: Nihon Rinsho Meneki Gakkai Kaishi
Date Published: April 01, 1995
Reference Number: 73
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A 68-year-old woman had been complained of xerostomia since she was 30 years old. Further symptoms of polyuria, polyposia and insomnia had been developed since she was 35 years old. The biopsy material from a minor salivary gland demonstrated the infiltration of lymphocytes into mesenchyme which was compatible with Sjogren's syndrome. She admitted to our hospital because of myalgia in bilateral gastrocnemius and petechiae in both lower extremities in addition to the complaints described above. Complete blood cell counts on admission revealed hemoglobin 9.7 g/dl, platelet count 12.5 x 10(4)/microliters, and white blood cell count 3,300/microliters. Marked polyuria, polyposia (more than 5,000 ml/day, respectively) and low urine gravity (1.005) were observed, although the serum creatinine level showed normal value. Serologic examination showed that the elevation of total serum protein concentration (9.5 g/dl) with marked elevation of serum IgG level (6,190 mg/dl). Her immunoglobulins contained cryoglobulin (cryocrit 20%), and immunoelectrophoresis demonstrated the existence of IgG-kappa monoclonal protein. A positive anti-nuclear antibody at 1:320 dilution, a positive rheumatoid factor and a positive antibody to SS-A (Ro) were also observed. The serial studies of blood gas analysis could not demonstrated the presence of metabolic acidosis. Together with the result of elevated plasma antidiuretic hormone level and results of vasopressin test, Fishberg's concentrating test and the tests of the overload of NH4Cl or bicarbonate, she was diagnosed Sjogren's syndrome with both diabetes insipidus and subclinical renal tubular acidosis. She was initially medicated with prednisolone (40 mg/day, orally), then she was given six courses of intravenous cyclophosphamide (750 mg/body/month).