Agranulocytosis in Sjögren's syndrome: Two case reports and analysis of 11 additional reported cases

2002 ◽  
Vol 31 (5) ◽  
pp. 338-345 ◽  
Author(s):  
J. Friedman ◽  
A. Klepfish ◽  
E.B. Miller ◽  
V. Ognenovski ◽  
R.W. Ike ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Case Reports ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome in the light of current research – literature review and selected case reports

2014 ◽  
Vol 67 (3) ◽  
pp. 409-422
Author(s):  
Maria Panaś ◽  
Maciej Rączkiewicz ◽  
Małgorzata Zaleska ◽  
Tomasz Kaczmarzyk ◽  
Konrad Kisielowski
Keyword(s):  
Literature Review ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Case Reports ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Research Literature ◽  
Sjögren‘S Syndrome

scholarly journals Anti-Ro/SAA Autoantibodies Might Represent the Antineuronal Antibodies Responsible for Cerebellar Degeneration in Sjögren's Syndrome 

2020 ◽  
Author(s):  
Syuichi Tetsuka ◽  
Tomohiro Suzuki ◽  
Tomoko Ogawa ◽  
Ritsuo Hashimoto ◽  
Hiroyuki Kato
Keyword(s):  
Sjögren’S Syndrome ◽  
Purkinje Cells ◽  
Sjögren's Syndrome ◽  
Case Reports ◽  
Cerebellar Atrophy ◽  
Central Nervous System Involvement ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Cerebellar Degeneration ◽  
Sjögren‘S Syndrome

Abstract Background: Neurologic disorders are one of the most common extraglandular manifestations of Sjögren's syndrome (SjS). Central neurologic symptoms can appear in about 5% of patients with SjS. However, only a few reports of cerebellar degeneration have been reported, and the clinical features and pathologic mechanisms of cerebellar degeneration associated with SjS are unclear.Patients and methods: We recently treated cerebellar degeneration in a patient with SjS. We analyzed the serum and cerebrospinal fluid (CSF) to detect anti-Ro/anti-SjS-related antigen A (SSA) and anti-La/anti-SjS-related antigen B (SSB) antibodies. We also searched the literature for previous case reports on SjS to evaluate the characteristics of cerebellar degeneration in patients with SjS and examined whether the Ro/SSA (Ro52/tripartite motif protein [TRIM]21) protein was expressed in murine cerebellum using immunohistochemistry.Results: Although all patients were positive for anti-Ro/SSA antibodies, some patients were negative for anti-La/SSB antibodies. Anti-Ro/SSA antibodies were observed in both serum and CSF. Anti-Ro/SSA antibodies were negative in the CSF of SjS patients without central nervous system involvement. Cerebellar atrophy was observed, and sequelae remained in the majority of the patients. Autopsy findings indicated a selective loss of Purkinje cells. Ro52/TRIM21 expression was detected throughout murine brains, including the hippocampus, cerebral cortex, and cerebellum. High Ro52/TRIM21 expression was observed in Purkinje cells.Conclusions: We described the characteristics of cerebellar degeneration in patients with SjS and Ro52/TRIM21 expression in Purkinje cells of murine cerebellar tissue sections. Thus, these outcomes indicated that anti-Ro/SSA autoantibodies were likely responsible for cerebellar degeneration in SjS.

scholarly journals Cognitive Function in Primary Sjögren’s Syndrome: A Systematic Review

2019 ◽  
Vol 9 (4) ◽  
pp. 85 ◽  
Author(s):  
Ciro Manzo ◽  
Eva Martinez-Suarez ◽  
Melek Kechida ◽  
Marco Isetta ◽  
Jordi Serra-Mestres
Keyword(s):  
Systematic Review ◽  
Cognitive Impairment ◽  
Cognitive Function ◽  
Sjögren’S Syndrome ◽  
Unmet Needs ◽  
Sjögren's Syndrome ◽  
Case Reports ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

Background: Cognitive disorders are reported to be common in patients with primary Sjogren’s syndrome (pSS). In some cases, they are the first clinical manifestation, preceding the diagnosis of pSS by two years on average. Aim: A systematic review was conducted to explore cognitive impairment in pSS, with reference to diagnostic methods and their relationship with laboratory data and clinical manifestations. Materials and Methods: According to the PRISMA 2009 checklist, we carried out a comprehensive literature search in the three main bibliographic databases: MEDLINE, EMBASE, and PsycINFO (NICE HDAS interface). The following main search terms were used: primary Sjogren syndrome, neurological manifestations, fatigue, cognitive functions, psychiatric manifestations, mild cognitive impairment, dementia, and neurocognitive disorder. The search was made on 14 September, 2018. References from all selected studies were also examined. Inclusion criteria were: all studies and case-reports published in any language from 2002 that assessed the association of pSS (according to classification criteria proposed by the 2002 American/European collaborative group (AECG)) with all types of cognitive impairment (including dementia). Exclusion criteria were: reviews, abstracts, secondary Sjögren’s syndrome (SS), and all articles in which other classification criteria were used. Results: The initial search yielded 352 articles, of which 253 were excluded based on the title and abstract review. A total of 54 articles underwent a full-length review, and 32 articles were excluded. Data were extracted from 18 studies and three case-reports involving a total of 6196 participants. In most cases, cognitive dysfunction was a brain fog or a mild cognitive impairment (MCI). Occasionally, an autoimmune dementia was present. The relationship between pSS and degenerative dementias, such as Alzheimer’s disease (AD), was a controversial issue, even if some investigators hypothesized that pSS could be a risk factor. Several unmet needs were highlighted. First, some of the included studies had not reported the severity of pSS; hence, few correlations between disease severity and cognitive function were possible. Secondly, the evaluation of the pathogenetic role of comorbid diseases was often absent. The lack of information on the type of dementia represented a third critical point in the majority of the included studies. Conclusions: This systematic review confirmed that adequate studies on cognitive function in pSS are scarce, mostly performed on small-sized samples, and often conflicting. The routine assessment of cognitive function in patients with pSS seems advisable and it will help to elucidate some of the unmet needs highlighted by this review in future appropriately designed studies.

scholarly journals Immunosuppressive treatment for peripheral neuropathies in Sjogren’s syndrome – a systematic review

2020 ◽  
Vol 58 (1) ◽  
pp. 5-12 ◽  
Author(s):  
Andreea Camelia Humă ◽  
Evelyn Maria Kecskeş ◽  
Delia Tulbă ◽  
Paul Bălănescu ◽  
Cristian Băicuş
Keyword(s):  
Peripheral Neuropathy ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Case Reports ◽  
Immunosuppressive Treatment ◽  
Case Series ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Peripheral Neuropathies ◽  
Sjögren‘S Syndrome

AbstractBackground. Sjogren’s syndrome (SS) is among the most frequent autoimmune diseases and one of its most severe extraglandular manifestations is peripheral neuropathy. There is no consensus about peripheral neuropathy treatment in SS. Our aim is to identify studies proving the efficiency of immunosuppressive treatment on peripheral neuropathies in SS.Methods. The search was conducted on the PubMed (MEDLINE) database. Studies with patients diagnosed with SS and peripheral neuropathy were included. Treatment with one of the following was among inclusion criteria: glucocorticoids (GC), rituximab (RTX), azathioprine (AZA), mycophenolic acid (MMF), cyclophosphamide (CP), methotrexate (MTX), plasmapheresis or iv immunoglobulins (IV IG).Results. A total of 116 results were found and abstracts were examined. 103 papers were excluded, and the remaining 13 papers were analyzed. They were 3 case series and 10 case reports, retrospective, totalizing 62 patients of which 22 (35.5%) received IV IG, 8 (13%) received RTX, 7 (11%) CP, and 5 (8%) received only GC. Drug associations containing corticosteroids were frequent. Of those 22 treated with IV IG, 18 patients improved (82%), and 4 stabilized (18%).IV IG was useful in sensory, motor and sensorimotor neuropathies. CP had good results in mononeuritis multiplex, while autonomic neuropathies responded well to GC or RTX. AZA, RTX, MTX, MMF or plasmapheresis were not used alone. Follow-up periods were heterogenous and the evaluation of the neuropathy was not systematic.Conclusion. There is only low level evidence (retrospective case reports and case series). In most cases, IV IG treatment in patients with peripheral neuropathies and SS resulted in clinical improvement, while other therapies, such as RTX, corticosteroids and CP proved to be useful in a handful of cases.

The association of Raynaud’s phenomenon/syndrome with scleroderma and Sjögren’s syndrome – a meta-analysis

VASA ◽  
2008 ◽  
Vol 37 (Supplement 73) ◽  
pp. 26-32 ◽  
Author(s):  
Schlattmann ◽  
Höhne ◽  
Plümper ◽  
Heidrich
Keyword(s):  
Quantitative Analysis ◽  
Sjögren’S Syndrome ◽  
Mixture Model ◽  
Sjögren's Syndrome ◽  
Meta Analysis ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Raynaud’S Syndrome ◽  
Raynaud's Syndrome ◽  
Sjögren‘S Syndrome

Background: In order to analyze the prevalence of Raynaud’s syndrome in diseases such as scleroderma and Sjögren’s syndrom – a meta-analysis of published data was performed. Methods: The PubMed data base of the National Library of Medicine was used for studies dealing with Raynaud’s syndrome and scleroderma or Raynaud’s syndroem and Sjögren’s syndrom respectively. The studies found provided data sufficient to estimate the prevalence of Raynaud’s syndrome. The statistical analysis was based on methods for a fixed effects meta-analysis and finite mixture model for proportions. Results: For scleroderma a pooled prevalence of 80.9% and 95% CI (0.78, 0.83) was obtained. A mixture model analysis found four latent classes. We identified a class with a very low prevalence of 11%, weighted with 0.15. On the other hand there is a class with a very high prevalence of 96%. Analysing the association with Sjögren’s syndrome, the pooled analysis leads to a prevalence of Raynaud’s syndrome of 32%, 95% CI(26.7%, 37.7%). A mixture model finds a solution with two latent classes. Here, 38% of the studies show a prevalence of 18.8% whereas 62% observe a prevalence of 38.3%. Conclusion: There is strong variability of studies reporting the prevalence of Raynaud’s syndrome in patients suffering from scleroderma or Sjögren’s syndrome. The available data are insufficient to perform a proper quantitative analysis of the association of Raynaud’s phenomenon with scleroderma or Sjögren’s syndrome. Properly planned and reported epidemiological studies are needed in order to perform a thorough quantitative analysis of risk factors for Raynaud’s syndrome.

Sign in / Sign up

Export Citation Format

Share Document