ABSTRACT
Renal involvement in primary Sjögren's syndrome occurs in 10-60% of cases. Tubulointerstitial nephritis with distal renal tubular acidosis (DRTA) is the main type of involvement. It's generally asymptomatic and revealed by complications of DRTA. We report 4 cases of GJS complicated by nephrocalcinosis, tetapresic hypokaliemia and osteomalacia. In 2 cases, nephrocalcinosis was diagnosed simultaneously with the GJS. The 2 other cases were diagnosed 3 and 15 years after primary GJS. All patients were treated by coticosteroids at the dose of 0.5 mg/Kg/jour, alkaline solution, K Cl in 2 cases and vitamin D in 2 cases. After a mean follow up of 8.7 years, the renal function remain stable in the 3 cases of nephrocalcinosis and in a patient with osteomalacia, bone lesions progressed. In conclusion, DRTA must be detected by acidification tests in patients with primary Sjögren's syndrome because of their latency and to prevent severe complications.
