Primary Sjögren's syndrome complicated with cryoglobulinemic glomerulonephritis, myocarditis, and multi-organ involvement.

ABSTRACT

Glomerulonephritis in primary Sjögren's syndrome is rarely reported. Cryoglobulinemic glomerulonephritis with the presence of cryoglobulin deposition in the glomerular capillary lumen in primary Sjögren's syndrome is extremely rare. A 51-year-old woman with primary Sjögren's syndrome for > 10 years complained of fever, hypertension, and proteinuria. In addition, novel manifestations, including myocarditis with heart failure, pericardial effusion, and polyneuropathy (sensory motor neuropathy) were also noted. Cryoglobulinemia test was positive, and kidney biopsy results were consistent with cryoglobulinemic glomerulonephritis. There were no symptoms associated with systemic lupus erythematosus or other connective tissue disease. Treatment with monthly methylprednisolone and cyclophosphamide pulse therapy for 6 months resulted in resolution of proteinuria, heart failure, and neurologic symptoms.