[A case of primary Sjögren's syndrome with severe pulmonary hypertension and glomerular damage].

Abstract

We report a case of 58 years old female with primary Sjögren's syndrome who accompanied pulmonary hypertension and glomerular damage. Renal biopsy revealed interstitial nephritis and glomerular damage. Pulmonary perfusion scintigram revealed diffusely decreased pulmonary perfusion, but the defect was not observed. Immunocomplex positive indicated that immune disorder would damage her lung and kidney. Proteinuria and pulmonary hypertension were improved by high dose of prednisolone and low dose of oral cyclophosphamide treatment. No previous reports had shown pulmonary hypertension and glomerular damage complicated with primary Sjögren's syndrome in same patients at same time. But some reports had suggested immune disorder had caused pulmonary hypertension or glomerulonephritis in patients of primary Sjögren's syndrome. Our patient showed immune disorder, and it might cause pulmonary hypertension and glomerular damage.