Analysis of the characteristics of juvenile primary Sj(?)gren's syndrome with renal damage / 中华风湿病学杂志

Objective To study the renal damage of patients with juvenile primary Sjgren's syndrome (pSS)and its clinical manifestations,pathologic characteristics with biopsy,treatment and prognosis.Methods Ten patients with juvenile pSS complicated with renal impairments were retrospectively analyzed.Data of these 10 patients were compared with those without renal impairments.Results Ten patients complicated with renal impairments in 24 patients with juvenile pSS,9 of them presented with type I renal tubular acidosis(RTA), 5 with hypokalemia paralysis,3 with calcification of the renal tissue,3 with positive urine protein.1 with dia- betes insipidus.There was no significant difference between patients with renal impairments and those without. Three patients underwent kidney biopsy that showed chronic interstitial nephritis(CIN)with extensive lymp- hoplasmie cell infiltration.Two patients had glomerular lesions and one of them was diagnosed as pSS over- laped with systemic lupus erythematosus(SLE).Steroid and immunosuppressive agents had significantly alle- viated symptoms and the hypergammaglobulinemia was significantly improved.Conclusion Renal impairment may be the major complication in juvenile pSS.The major clinical manifestations are RTA and the glomeruli are involved occasionally.Treatment with steroid anti immunnsuppressive agents should be given to those who have evidence of systemic involvement.