Parkinsonism and Sjögren’s Syndrome: A Fortuitous Association or a Shared Immunopathogenesis?

Abstract

Background. The Sjögren Syndrome (SS) can include various manifestations of central nervous system impairment. Extrapyramidal signs are known to be very rare and unusually discovered on early onset in this pathology. Observation. A 46-year-old woman with a history of progressive Parkinsonism for 6 years and a normal brain magnetic resonance imaging was partially improved with levodopa therapy. The later discovery of a sicca syndrome led to performing of further investigations, which revealed the presence of anti-SSA antibodies and a sialoadenitis of grade 4 according to Chisholm's classification on labial salivary gland biopsy. The diagnosis of primary SS was established and the adjunction of corticotherapy has remarkably improved Parkinson's signs without use of other immunosuppressive agents. Conclusion. Based on these findings, we discuss the hypothesis of either a causal link between SS and Parkinsonism or a fortuitous association of two distinct pathologies with or without a shared immunopathogenesis.

Impact of Sjogren's syndrome on Parkinson’s disease: A nationwide case-control study

“…Several studies have reported that SS was associated with PD, and their results were well correlated with ours [ 17 , 18 ]. Our study involved a total of 143 patients who were suffering from both SS and PD.…”

“…It was mentioned by some studies that autoimmune diseases, for instance rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), psoriasis, osteoarthritis and vasculitis, were well-recognized risk factors of PD because of the neurologic abnormalities caused by immune damage [ 11 – 16 ]. In addition to these autoimmune factors, few studies introduced that Sjogren’s syndrome (SS) was connected with PD nowadays [ 17 , 18 ].…”

“…Although several studies have investigated the association between SS and PD, the sample sizes of these studies were too small to gain a convincing result [ 17 , 18 ]. Therefore, the purpose of this study is to explore whether SS is associated with the generation of PD by conducting a case-control study based on a nationwide longitudinal population-based database.…”

Movement disorders in systemic autoimmune diseases: Clinical spectrum, ancillary investigations, pathophysiological considerations

“…Epidemiology Median age at onset was 62.5 years (range, 40 -74 years); 90% were female. Parkinsonism preceded other PSS manifestations in 70% of cases [51,53,54,56,57].…”

“…Despite differing methodologies, all found PSS to be associated with increased risk of PD, with adjusted hazard ratios (aHR) ranging from 1.23 to 1.56 [48]. Despite this, reports of parkinsonism in patients with PSS are limitedonly ten met our criteria for analysis [51][52][53][54][55][56][57][58].…”