Sjögren's syndrome with acute renal failure

• Published in: Journal of Korean medical science 1998-12, Vol.13 (6), p.665-669
• Main Authors: Kwon, Y J, Park, J H, Kim, S W, Han, S Y, Pyo, H J, Won, N H
• Format: Article
• Language: English
• Subjects: Acute Kidney Injury - complications; Acute Kidney Injury - pathology; Aged; Female; Humans; Sjogren's Syndrome - complications; Sjogren's Syndrome - pathology
• ISSN: 1011-8934; 1598-6357
• DOI: 10.3346/jkms.1998.13.6.665

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 Ten months after recovery from acute renal failure, low-dose oral prednisolone (0.1 mg/kg/day) was withdrawn.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.