Evidence of an immunopathogenic basis for central nervous system disease in primary Sjögren's syndrome

Abstract

The pathogenesis of central nervous system complications in primary Sjögren's syndrome (CNS‐SS) is unknown. In order to determine whether patients with active CNS‐SS have cerebrospinal fluid (CSF) abnormalities indicative of CNS inflammation, CSF analyses from 30 patients with active CNS‐SS (SSA) were contrasted with those from 20 SS patients without CNS involvement (SSI) and 20 patients with systemic lupus erythematosus and active CNS disease (SLEA). Elevations of total protein concentration, IgG concentration, IgG to total protein ratio, and IgG index were observed in patients with SSA, but not in those with SSI. Agarose gel electrophoresis results were abnormal, with 1 or more bands, in 25 of 29 SSA patients (86%), but in only 3 of 18 SSI patients (17%). Similar, but less striking, CSF abnormalities were seen in a minority of SLEA patients. Fifteen SSA patients (50%) had transient, mild‐to‐moderate CSF pleocytosis, while only 1 SSI patient and 2 SLEA patients had similar findings. Cytologic findings were abnormal in 18 SSA patients (60%); these included atypical mononuclear cells, lymphoblastoid cells, and plasma cells. The presence of immuno‐competent cells and evidence for the intrathecal synthesis of IgG within the CSF of SSA, but not SSI, patients provide diagnostic parameters which are indicative of active disease and which can be monitored serially during therapy.

Cerebrospinal Fluid Anti-SSA Autoantibodies in Primary Sjögren’s Syndrome with Central Nervous System Involvement

“…The blood-brain barrier is generally intact in SS-related CNS involvement [13]. All patients showed evidence of intrathecal IgG synthesis demonstrated by raised CSF IgG index or CSF-specific oligoclonal bands, which is in accordance with previously published findings [13]. This observation may indicate a specific role of intrathecally synthesized IgG in SS-related CNS manifestations.…”

Cerebrospinal Fluid Findings in Neurological Diseases Associated with Sjögren's Syndrome

“…However, data regarding CSF results in patients with Sjögren's syndrome are of a lesser volume. Indeed, one study included 30 patients with active CNS involvement [14] . However, CSF results are incomplete and the interpretation of OCBs (positive in 86% of 29 patients) underlies limitations.…”

“…Few studies reported routine CSF findings such as cell count, glucose, total protein level, and oligoclonal bands (OCBs) in case reports or small case series [4,[9][10][11][12][13] . In 1986, CSF cell count, total protein, and IgG profiles from 30 patients with CNS involvement due to Sjögren's syndrome were described [14] . Because CSF findings in patients with Sjögren's syndrome are still poorly defined, we performed a thorough evaluation of clinical and laboratory data with special emphasis on CSF.…”

An autopsied case of Sjögren's syndrome with massive necrotic and demyelinating lesions of the cerebellar white matter

“…The neurological features seen in patients with primary SjS include seizure disorders [10], aseptic meningoencephalitis [12], symptoms mimicking multiple sclerosis [11], progressive dementia [6], and paraneoplastic-like syndrome [13].…”