クリオグロブリン血症, 腎性尿崩症, 潜在性尿細管性アシどーシスを合併したシェーグレン症候群の1例 [in Japanese] A case of Sjogren's syndrome complicated with cryoglobulinemia, nephrogenic diabetes insipidus, and renal trbular acidosis [in Japanese]
Access this Article
Search this Article
症例は68歳,女性.約40年間,口腔乾燥を認め,耳鼻科での精査によりシェーグレン症候群の診断を得た.さらに多飲多尿,腓腹筋痛,下腿皮下出血が出現したため入院となった.入院時,汎血球減少,赤沈亢進, LDH高値,血清総蛋白高値,高ガンマグロブリン血症, IgG-κ型M蛋白,クリオグロブリン血症が認められた.また,フィッシュバーグテスト,バゾプレッシンテスト, NH<sub>4</sub>C<sub>l</sub>負荷試験などの結果,腎性尿崩症,潜在性尿細管性アシドーシスの合併を認めた.入院後,血漿交換,プレドニン40mg/日の投与を開始したところ,血清総蛋白,ガンマグロブリンは低下し,出血症状は改善した.しかし,尿量,尿比重は改善しなかったため,サイクロフォスファミド大量療法(700mg/body)を行ったが,検査異常所見ならびに尿崩症の改善は認めなかった.本症例で認められた腎間質と糸球体の障害は,シェーグレン症候群とクリオグロブリンの両者によるものであると考えられた.
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 Sjören'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 9.7g/d<i>l</i>, platelet count 12.5×10<sup>4</sup>/μ<i>l</i>, and white blood cell count 3, 300/μ<i>l</i>. Marked polyuria, polyposia (more than 5, 000m<i>l</i>/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 (9.5g/d<i>l</i>) with marked elevation of serum IgG level (6, 190mg/d<i>l</i>). Her immunoglobulins contained cryoglobulin (cryocrit 20%), and immunoelectrophoresis demonstrated the existence of IgG-κ 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 NH<sub>4</sub>Cl or bicarbonate, she was diagnosed Sjögren's syndrome with both diabetes insipidus and subclinical renal tubular acidosis. She was initially medicated with prednisolone (40mg/day, orally), then she was given six courses of intravenous cyclophosphamide (750mg/body/month). These treatments, however, did not change her complaints nor change the laboratory examination. It is generally accepted that interstitial nephritis induced by the infiltration of lymphocytes may contribute to the renal tubular acidosis and diabetes insipidus seen in Sjören's syndrome. Taking to the resistance of these treatments, the large amount of cryoglobulin in her serum might responsible for the pathogenesis of renal disorders as well as the infiltration of lymphocytes.
- Japanese Journal of Clinical Immunology
Japanese Journal of Clinical Immunology 18(2), 221-227, 1995-04-30
The Japan Society for Clinical Immunology