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Sjögren's syndrome with acute renal failure. 1998

Y J Kwon, and J H Park, and S W Kim, and S Y Han, and H J Pyo, and N H Won
Department of Internal Medicine, Korea University, Seoul. toitoi@hitel.net.

We experienced a 65-year-old woman with Sjögren's syndrome who presented with acute renal failure, hypergammaglobulinemia with monoclonal gammopathy, and hypocomplementemia. She improved with steroid pulse therapy (methylprednisolone 0.5 g/day for 3 days). This patient had also sensorineural hearing loss, symmetric sensory polyneuropathy of legs, and interstitial lung disease. Ten months after recovery from acute renal failure, low-dose oral prednisolone (0.1 mg/kg/day) was withdrawn. On the third month of steroid withdrawal, acute renal failure recurred with hypergammaglobulinemia, hyperamylasemia, and autoimmune cholangitis-like biochemical derangements, which also responded to steroid pulse therapy (methylprednisolone 0.3 g/day for 3 days). When we would withdraw steroid in a patient with visceral involvement of Sjögren's syndrome, we should consider multiple clinical and laboratorial variables, including erythrocyte sedimentation rate, serum levels of IgG, total protein, C3/C4, CRP, amylase, lipase, and alkaline phosphatase. We report this case which exhibited various unusual manifestations with a review of literature.

UI MeSH Term Description Entries
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012859 Sjogren's Syndrome Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis. Sicca Syndrome,Sjogren Syndrome,Sjogrens Syndrome,Syndrome, Sicca,Syndrome, Sjogren's
D058186 Acute Kidney Injury Abrupt reduction in kidney function. Acute kidney injury encompasses the entire spectrum of the syndrome including acute kidney failure; ACUTE KIDNEY TUBULAR NECROSIS; and other less severe conditions. Acute Kidney Failure,Acute Kidney Insufficiency,Acute Renal Failure,Acute Renal Injury,Acute Renal Insufficiency,Kidney Failure, Acute,Kidney Insufficiency, Acute,Renal Failure, Acute,Renal Insufficiency, Acute,Acute Kidney Failures,Acute Kidney Injuries,Acute Kidney Insufficiencies,Acute Renal Failures,Acute Renal Injuries,Acute Renal Insufficiencies,Kidney Failures, Acute,Kidney Injuries, Acute,Kidney Injury, Acute,Kidney Insufficiencies, Acute,Renal Failures, Acute,Renal Injuries, Acute,Renal Injury, Acute,Renal Insufficiencies, Acute

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