Sjögren's syndrome is a chronic
autoimmune disease, characterized by specific autoimmune
antibodies anti-Ro and anti-La, and it can involve multiple organs, such as the kidneys, lungs, muscles, and nervous system. The most common renal complication of Sjögren's syndrome is
tubulointerstitial nephritis, and
glomerulonephritis is relatively uncommon. We report the case of an 86-year-old man presenting with recurrent
fever, poor appetite, decreased salivary secretion, and
body weight loss. Laboratory investigation revealed that serum
creatinine was 4.2 mg/dL,
proteinuria was 3+, and there was microscopic
hematuria. Positive perinuclear
anti-neutrophil cytoplasmic antibody, anti-Ro, and anti-La
antibodies were detected. Renal biopsy showed crescentic
glomerulonephritis with scanty
immune complex deposition. The patient was diagnosed with primary Sjögren's syndrome complicated with rapidly progressive
glomerulonephritis with positive
anti-neutrophil cytoplasmic antibody. Unlike the patients of other case reports, our patient's renal function did not recover after
immunosuppressant treatment, and he finally received long-term
hemodialysis. Pauci-immune
glomerulonephritis is a rare renal complication of Sjögren's syndrome, and progress to
renal failure in such patients is possible.