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Central nervous system involvement in Sjogren's syndrome

2004; BMJ; Volume: 63; Issue: 6 Linguagem: Inglês

10.1136/ard.2003.019497

1468-2060

Fotini Soliotis,

Systemic Lupus Erythematosus Research

Still an unresolved issue—multicentre studies are needed Sjogren’s syndrome (SS) is a relatively common autoimmune disorder, affecting 2–3% of the adult population. It is characterised by lymphocytic infiltration and destruction of the exocrine glands. The salivary and lachrymal glands are primarily affected, leading to a dry mouth and dry eyes, the hallmark of the disease. Other exocrine glands, which may be affected, include those of the pancreas, bronchial tree, and gastrointestinal tract. SS may occur alone (primary) or in association with other connective tissue diseases (secondary). The spectrum of clinical manifestations of SS is wide, ranging from mucosal dryness, directly related to exocrine dysfunction, to more systemic complaints, affecting mainly the musculoskeletal, pulmonary, renal, and vascular systems.1 Peripheral nervous system disease, manifested commonly as peripheral sensory neuropathy or more rarely as mononeuritis multiplex, is a well established feature of the disease, occurring in approximately a quarter of patients.2 However, involvement of the central nervous system (CNS) in patients with SS is a matter of serious controversy between different research groups, with a prevalence ranging from “undetectable” to “quite common”. This discrepancy was previously attributed possibly to differences in the diagnostic criteria, selection of populations, special interests of different teams of investigators, or referral bias.3,4 The prevalence of CNS disease in SS is a controversial topic. Tables 1 and 2 list the prevalence of CNS manifestations in patients with SS reported since 1985. The numbers quoted in publications range between 0 and 100%.2,5–21 View this table: Table 1 CNS involvement in SS: reported prevalence View this table: Table 2 Psychiatric involvement in SS: reported prevalence Why is there is such a huge variability in the reported prevalence of CNS disease in SS? Firstly, there is no consensus on the definition of CNS involvement. Some authors include psychiatric disease, but some do not. Also there …