Primary Sjogren's syndrome with mesangial proliferative glomerulonephritis and IgA deposits in a child

摘要

Sjogren's syndrome is very rare in children. Here, we introduce the reader to a case of primary Sjogren's syndrome (pSS) with mesangial proliferative glomerulonephritis and IgA deposits, an uncommon renal manifestation in boys. Although we thought his prognosis was not good, his response to steroid therapy was good regarding renal problems of pSS.An 11-year-old boy was admitted to the hospital for intermittent gross hematuria and mild proteinuria, which he had had for the past year. He complained of dry eyes and dry mouth, and this had been aggravated 6 months previously. He had no family history of any kidney disease or autoimmune disease. The vital signs were stable and the physical examination revealed no abnormalities, including the lack of parotid gland swelling. Laboratory findings were as follows: hemoglobin 12.2 g/dL, white blood cell count 7,100/|iL, platelet count 327,000/|xL, blood urea nitrogen (BUN) 10 mg/dL, creatinine 0.7 mg/dL, C3 134 mg/dL, C4 15.4 mg/dL, Clq 15.5 mg/dL, CH50 46.2 U/mL, IgA 196 mg/dL, IgG 1,230 mg/dL, and IgM 71.9 mg/dL. The blood gas results showed mild metabolic acidosis. The electrolytes, liver function tests, protein, albumin, anti-neutrophil antibody, and the glomerular filtration rate were also unremarkable.