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

Mégevand, Pierre; Chizzolini, Carlo; Chofflon, Michel; Roux‐Lombard, Pascale; Lalive, Patrice H.; Picard, Fabienne

doi:10.1159/000098469

Cited by 21 publications

(15 citation statements)

References 47 publications

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“…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] .…”

Section: Cerebrospinal Fluid Findings Inmentioning

confidence: 99%

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

Pars

Pul²,

Schwenkenbecher³

et al. 2016

Eur Neurol

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Background: Sjögren's syndrome is a chronic autoimmune-mediated disease that can cause a variety of neurological manifestations. Methods: This study investigated characteristics of clinical and cerebrospinal fluid (CSF) features in patients with neurological diseases associated with Sjögren's syndrome. Eighty-two patients were examined separately according to the presence of Sjögren's syndrome alone or in combination with other autoimmune diseases. Results: In the 47 patients with primary Sjögren's syndrome, peripheral neuropathy (57%) was found most frequently, followed by the involvement of the central nervous system (CNS; 17%), cranial neuropathy (15%), and myalgia (11%). These patients did not display consistent signs of inflammation in the CSF. Slight pleocytosis of 8-107 cells/µL was found in patients with peripheral neuropathy (9%), cranial neuropathy (20%), and CNS involvement (25%). Oligoclonal bands indicating intrathecal IgG synthesis occurred in 26% of patients with peripheral neuropathy, 20% of patients with cranial neuropathy, and 25% of patients with CNS involvement. Conclusions: In patients with Sjögren's syndrome and neurological manifestations, inflammatory CSF changes were rarely found and did not show a characteristic pattern irrespective of peripheral or central genesis of neurological deficits. Analysis of the CSF presents therefore an important diagnostic procedure to exclude other autoimmune and infectious diseases.

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Section: Cerebrospinal Fluid Findings Inmentioning

confidence: 99%

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

Pars

Pul²,

Schwenkenbecher³

et al. 2016

Eur Neurol

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“…Die Differenzierung zur MS ist nicht immer einfach. Hierbei hilft die Bestimmung der oligoklonalen Banden oder anti-Ro-Antikörper im Liquor [18]. Zur Abgrenzung gegenüber einer Neuromyelitis optica (NMO, bzw.…”

Section: Extraglanduläre Manifestationenunclassified

Das Sjögren-Syndrom

et al. 2017

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ZusammenfassungDas Sjögren-Syndrom ist eine chronische Autoimmunerkrankung mit Beteiligung der Tränen- und Speicheldrüsen. Durch lymphozytäre Infiltration und sekundäre Destruktion des exokrinen Drüsengewebes kommt es zur Sicca-Symptomatik. Es handelt sich um eine der 3 häufigsten Autoimmunerkrankungen mit einer Prävalenz von 0,03–2,1%, abhängig von Alters- und Populationsgruppen sowie der angewandten Diagnostik-Kriterien, und betrifft deutlich häufiger Frauen (ca. 10:1) 1 2 3. Neben der charakteristischen Sicca-Symptomatik leiden viele Patienten unter systemischen Manifestationen wie z. B. Arthralgien, seltener Hautvaskulitis, interstitieller Lungenerkrankung oder Nierenbeteiligung.Nicht-steroidale Antirheumatika, Kortikosteroide und Immunsuppressiva werden zur Therapie dieser Komplikationen angewandt während die Sicca-Symptomatik meist lokal-symptomatisch behandelt wird. Das Risiko der Entwicklung eines malignen Lymphoms ist beim Sjögren-Syndrom erhöht. Ein Großteil der Patienten leidet außerdem unter einer ausgeprägten Abgeschlagenheit (Fatigue) 4, die das subjektiv schwerwiegendste Symptom darstellen kann. Die Prävalenz von depressiven Störungen ist ebenfalls erhöht 5. Auch wenn neue therapeutische Angriffspunkte und B-Zell-gerichtete Therapien, die sich derzeit in klinischer Testung befinden, auf eine Verbesserung der Behandlungsmöglichkeiten hoffen lassen, bleibt die Heterogenität des Krankheitsbildes und der Symptome eine Herausforderung in Klinik und Forschung.

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“…For patients reporting memory deficit, dementia or cognitive dysfunction, these white matter lesions are always present [ 32 , 43 – 48 ]. In one case of dementia, an Electroencephalogram (EEG) agreed with the SPECT findings above, identifying in the parietal lobes short runs of theta activities; symptoms improved with treatment of corticosteroids [ 49 ].…”

Section: Brain Lesionsmentioning

confidence: 99%

“…This same treatment for another patient worked and others in the study (treated with 5 to 10 mg daily of Donepezil) also saw improvement and ceased cognitive decline [ 45 ]. Treatment with hydroxychloroquine was also effective against memory and concentration deficits [ 48 ]; contrarily, L-dopa has not improved patient symptoms [ 47 ]. In this study, 12 patients (out of 20 pSjS patients) were found to have dementia- the majority of which had parietal lobe lesions by SPECT, but this statistic is skewed drastically from its occurrence in the average population, as the patients were chosen at a memory clinic.…”

Section: Brain Lesionsmentioning

confidence: 99%

Beyond the Glands: An in-Depth Perspective of Neuropathy in Sjögren’s Syndrome

Voigt

Sukumaran

Nguyen³

2014

Rheumatology (Sunnyvale)

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Primary Sjögren’s Syndrome (pSjS) is an autoimmune disease characterized by sicca (xerophthalmia or xerostomia) symptoms, anti-SS-A (Ro) or anti-SS-B (La) autoantibodies, and lymphocytic infiltrates in the exocrine glands. Disease incidence is estimated to be 0.1–3% of the general population with 0.4–3.1 million individuals in the US with women being nine times more likely to be afflicted with SjS than men. The frequency continues to rise accompanied with the multi-factorial etiology making it a challenging disease to manage and treat. Treatment of this disease remains problematic due to the lack of therapeutic treatments relying on replacement therapies such as artificial saliva and eye lubricants or immunosuppressive agents. To further complicate the management of the disease, there are number of multi-systemic manifestations specifically peripheral neuropathy associated with later stage of disease onset. Increasingly, there is mounting evidence that suggests the involvement of central nervous system. It remains to be determined the underlying cause and effect of the dysregulated immune response and the neuropathy associated with SjS. In this review, we provided an in-depth look at key neurological dysfunctions documented to occur in pSjS. Specifically, we discussed the prevalence, symptomology, and current treatments.

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

Cited by 21 publications

References 47 publications

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

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

Das Sjögren-Syndrom

Beyond the Glands: An in-Depth Perspective of Neuropathy in Sjögren’s Syndrome

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