IgA nephropathy, non-cirrhotic portal fibrosis, and POEMS syndrome: A rare combination in the long-term follow-up of Sjögren's syndrome.

Abstract

Sjögren's syndrome is a heterogeneous autoimmune disorder that may be associated with systemic manifestations involving multiple organs. We herein reported a rare combination of immunoglobulin A nephropathy; non-cirrhotic portal fibrosis; and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome in a 15-year follow-up of a female patient initially diagnosed with Sjögren's syndrome. The patient had excessive lymphoproliferation featured by lymphadenopathy and hyperglobulinemia. The diagnoses of immunoglobulin A nephropathy and non-cirrhotic portal fibrosis were confirmed by renal and liver biopsies. She received prolonged corticosteroids and immunosuppressive drugs, which improved immunoglobulin A nephropathy but did not hinder the progression of portal fibrosis, leading to intractable variceal bleeding. The patient died of repeated hematemesis despite endoscopic variceal ligation. Valuable pathological information of multi-organ involvement as well as detailed clinical course were presented to facilitate further understanding of this rare entity. Excessive lymphoproliferation might play an important role in the progression of systemic complications in Sjögren's syndrome, which requires prolonged immunosuppression and organ-specific treatment.