Systemic manifestations of Sjögren’s syndrome

Abstract

Sjögren’s syndrome (SS) is a heterogeneous disease which, in the majority of cases, includes a mild clinical course. However, in some patients it takes on a severe form with numerous systemic manifestations and results in an adverse outcome. Systemic manifestations occur in approximately 25% of patients with primary Sjögren’s syndrome (pSS). The clinical presentation of systemic manifestations of SS is very diverse and can involve any organ system. Systemic manifestations can occur due to lymphocytic infiltration of organs or proliferation of B lymphocytes and deposition of immune complexes. fatigue is the most common systemic manifestation. The most significant cutaneous manifestations of the disease are palpable purpura, ulcerations, urticarial vasculitis and leukocytoclastic vasculitis. musculoskeletal manifestations can range from arthralgias to erosive arthritis. Pulmonary involvement may include symptoms such as nonspecific interstitial pneumonia with fibrosis and tracheobronchial disease. renal changes are observed in 10% to 30% of patients with SS. tubulointerstitial nephritis, type 1 renal tubular acidosis and nephrogenic diabetes insipidus can develop as a consequence of lymphocytic infiltration. Less often, the inflammatory process affects the glomeruli which leads to glomerulonephritis. Liver diseases are found in approximately 20% of patients with SS and their symptoms usually include inflammation of intrahepatic bile ducts resembling primary biliary cirrhosis. The manifestations of peripheral nervous system involvement include sensorimotor axonal polyneuropathy, mononeuritis multiplex, neuropathies and radiculopathies. optic neuropathy, hemiparesis, movement disorders, cerebellar syndromes, transient ischemic attacks, transverse myelitis (less commonly), and progressive myelopathy have been described as central nervous system changes. Symptoms of vasculitis can range from mononeuritis multiplex to intestinal ischemia and dysfunction of the affected organs. The development of non-Hodgkin’s B-cell lymphoma is a major complication of the disease which occurs in 5%–7% of patients with SS.