scholarly journals Neurological Disorders in Primary Sjögren's Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Gabriel J. Tobón ◽  
Jacques-Olivier Pers ◽  
Valérie Devauchelle-Pensec ◽  
Pierre Youinou
Keyword(s):  
Nervous System ◽  
Sjögren’S Syndrome ◽  
Neurological Disorders ◽  
Sjögren's Syndrome ◽  
Lymphocytic Infiltration ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Neurological Involvement ◽  
Autoimmune Exocrinopathy ◽  
Sjögren‘S Syndrome

Sjögren's syndrome is an autoimmune disease characterized by an autoimmune exocrinopathy involving mainly salivary and lacrimal glands. The histopathological hallmark is periductal lymphocytic infiltration of the exocrine glands, resulting in loss of their secretory function. Several systemic manifestations may be found in patients with Sjögren's syndrome including neurological disorders. Neurological involvement ranges from 0 to 70% among various series and may present with central nervous system and/or peripheral nervous system involvement. This paper endeavors to review the main clinical neurological manifestations in Sjögren syndrome, the physiopathology, and their therapeutic response.

scholarly journals Sjögren’s syndrome with nervous system injury combined with pulmonary and osseous cryptococcosis: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Liping Xu ◽  
Xinwei Song ◽  
Yan Zhang ◽  
Na Lin ◽  
Ji-An Wang
Keyword(s):  
Computed Tomography ◽  
Nervous System ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Resonance Imaging ◽  
Computed Tomography Scan ◽  
Tomography Scan ◽  
Sjögren‘S Syndrome

Abstract Background Sjögren’s syndrome is a common autoimmune disease that can involve the nervous system, but rarely both the central and peripheral. Long-term use of high-dose corticosteroids and immunosuppressants are the main risk factors for Cryptococcus infection in patients with Sjögren’s syndrome, of which pulmonary infection is the most common, while multiple bone infections are rare. Case presentation A 46-year-old Chinese woman with a 2-year history of Sjögren’s syndrome presented to our hospital with numbness of limbs, shortness of breath, and weakness. Blood immunochemistry showed that antinuclear antibody (1:640), anti-Sjögren’s syndrome-A antibodies, and anti-centromere antibodies were strongly positive. Cranial magnetic resonance imaging revealed multiple demyelinating lesions in the white matter of bilateral cerebral hemispheres. Electromyography indicated serious peripheral nerve injury, especially in lower limbs. Computed tomography scan of lumbar vertebral displayed multiple high-density shadows, and the corresponding vertebrae on magnetic resonance imaging showed abnormal low signal intensity on T1 and T2 sequences. Positron emission tomography–computed tomography showed multiple lesions with high 18F-fluorodeoxyglucose uptake in lung and vertebral bodies. Both lung and bone biopsies suggested Cryptococcus infection, with the diagnosis of Sjögren’s syndrome with nervous system injury combined pulmonary and osseous cryptococcosis. She took a reduced dose of prednisone about 10 mg/day, terminated mycophenolate mofetil, and began to take immunoglobulin of 0.4 g/kg/day intravenously for 5 days, fluconazole (400 mg/day) for 6 months. Within 3 weeks, her chest radiography showed a marked improvement, and 3 months later, the pulmonary lesions disappeared on her computed tomography scan. Conclusions This case exhibits an extremely rare condition of neural involvement in Sjögren’s syndrome combined with pulmonary and osseous cryptococcosis. This report also highlights the crucial role of detailed clinical examination, serologic markers, and biopsy in avoiding misdiagnosis. Currently, there is no guideline for this situation; in this case, we controlled the disease successfully with antifungal drugs and adequate gamma globulin, followed by an appropriate dose of corticosteroids.

scholarly journals AB0328 CLINICAL AND SEROLOGICAL DIFFERENCES BETWEEN PRIMARY SJOGREN’S SYNDROME PHENOTYPES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1189.1-1189
Author(s):  
X. E. Larco Rojas ◽  
E. Diez Alvarez ◽  
C. Moriano ◽  
A. López Robles ◽  
T. Pérez Sandoval
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Age Groups ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
The Other ◽  
Neurological Involvement ◽  
Primary Sjogren’S Syndrome ◽  
Sjögren‘S Syndrome

Background:Primary Sjogren’s Syndrome (PSS) is an autoimmune and lymphoproliferative disease with a heterogeneous presentation. It has been postulated that there may be different phenotypes, in some cases presenting a more aggressive disease with systemic manifestations and a higher risk of developing complications. This phenotype has been associated with a higher autoimmune load and an earlier age of presentation. Furthermore, the presence of anti La + has been related to an increased risk of developing Lymphoma.Objectives:To describe the phenotypic characteristics of seronegative PSS in a sample of patients from our practice. To compare the clinical and laboratory characteristics between patients with Ro + / La + and Ro + / La- antibodies. To Analyze if there are differences in patients diagnosed at an early age, compared to a later age.Methods:Clinical and serological characteristics of patients with the diagnosis of PSS were collected from the Rheumatology database of León`s Hospital between 2014-2020. All patients who met the ACR / EULAR 2016 criteria were classified as seronegative Sjogren.In the group of patients with positive autoimmunity, anti-Ro + / La + were compared with the anti-Ro + / La- patients and by age, stratifying them into the following groups: 0-49; 50-69 and> 70 years. The clinical variables analyzed were: glandular inflammation, Raynaud’s phenomenon (RP), pulmonary and neurological involvement, presence of Lymphoma and other tumours. The serological variables were: positivity of ANA, Rheumatoid Factor (RF), hypocomplementemia, hypergammaglobulinemia and B2 microglobulin.Results:72 patients were analysed, 9 were excluded because didn’t meet the criteria. Of the remaining: 90,4% were women, with a mean age of 58,7+/-15,8 years, 12,6% (8) were seronegative. In the seronegative group 25% presented lung involvement (Lymphoid Interstitial Pneumonia), 50% presented with glandular inflammation and only one patient had RP. As complications 1 patient presented Lymphoma and 1 Breast Carcinoma.58,7% (37) Ro + / La + and 28,5% (18) Ro + / La- patients were identified, no statistically significant differences were found between the two groups when comparing: glandular inflammation (8/37 vs 2/18, p = >0.05) RP (9/37 vs 4/18, p = >0.05), pulmonary involvement (5/37 vs. 6/18, p = >0.05), neurological involvement (2/37 vs. 1/18, p = >0.05), presence of Lymphoma (2/37 vs. 0 / 18, p = >0,05), other tumours (2/37 vs 3/18, p = >0.05), ANA positivity (36/37 vs 16/18, p =>0,05), Hypocomplementemia (4/37 vs 3/18, p =>0.05) and Hypergammaglobulinemia (20/37 vs 10/18, p =>0.05). But a higher frequency of positive RF linked to anti La positivity (29/37 vs 6 / 18p = 0.002) was found.When comparing by age groups, the association between RF + and La + remained in the group of 50-69 years (15/18 vs 3/18, p = 0.002) while in the other age groups there were no statistically significant differences. We also observed an increasing trend of the levels of B2microglobulin in La+ patients and later age (p=0,04)Conclusion:The presence of anti La + seems to be associated with other components of autoimmunity such as RF in patients with PSS, although this study did not show a relation with a higher frequency of complications or systemic disease. Also, the presence of La+ at older ages was associated with higher levels of B2 microglobulin. We didn’t find differences with the other described markers of B cell reactivation. Findings differ from those found in the literature, which may be largely due to sample size.References:[1]Quartuccio L., Baldini C., Bartoloni E., et al. Anti-SSA/SSB-negative Sjogren’s syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution. Autoimmunity Reviews 14 (2015) 1019–1022.[2]Quartuccio L, Isola M, Baldini C, Priori R, Bartoloni Bocci E, Carubbi F, et al. Biomarkers of lymphoma in Sjögren’s syndrome and evaluation of the lymphoma risk in prelymphomatous conditions: results of a multicenter study. J Autoimmun 2014; 51:75–80.Disclosure of Interests:None declared

scholarly journals Sjogren’s Syndrome Presenting with Solely Cutaneous Features

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1260
Author(s):  
Sneha Centala ◽  
Joyce H. Park ◽  
Diana Girnita
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Keratoconjunctivitis Sicca ◽  
Systemic Disease ◽  
Relapsing Polychondritis ◽  
Lymphocytic Infiltration ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Initial Symptom ◽  
Sjögren‘S Syndrome

Sjogren’s syndrome is classically characterized by symptoms of keratoconjunctivitis sicca and xerostomia, secondary to lymphocytic infiltration of the salivary and lacrimal glands. Cutaneous findings of this disease are infrequently discussed and thus rarely considered among patients without the typical symptomatology. However, these patients can develop xerosis, alopecia, vitiligo, papular or nodular lesions, or cutaneous vasculitis. A 56-year-old Asian female presented with intermittent cutaneous erythematous lesions of her bilateral pinna and preauricular areas. Despite initial symptom presentation causing concern for tumid lupus versus cutaneous T cell lymphoma versus relapsing polychondritis, extensive serologic and histopathologic workup eventually indicated a likely diagnosis of Sjogren’s syndrome. This case brings to light that Sjogren’s syndrome is truly a multi-systemic disease and can present with primarily extra glandular cutaneous symptoms. When approaching the workup of a new patient, it is absolutely vital to maintain a broad differential and keep in mind that overlap syndromes among multiple autoimmune diseases do exist as well.

scholarly journals Doença Intersticial Pulmonar Grave e Mania Induzida por Corticosteróides em Doente com Lúpus Eritematoso Sistémico e Síndrome de Sjögren Secundária

2017 ◽  
Vol 30 (3) ◽  
pp. 246 ◽  
Author(s):  
Sofia Silvério Serra ◽  
Teresa Pedrosa ◽  
Sandra Falcão ◽  
Jaime Cunha Branco
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Sjögren’S Syndrome ◽  
Lupus Erythematosus ◽  
Sjögren's Syndrome ◽  
Manic Episode ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Neurological Involvement ◽  
Systemic Lupus ◽  
Sjögren‘S Syndrome

Interstitial lung disease occurs in up to 25% of patients with Sjögren’s syndrome and 2% - 8 % of patients with systemic lupus erythematosus. Corticosteroid therapy remains the main treatment for systemic lupus erythematosus. However, it can be associated with several neuropsychiatric disorders especially with prednisolone at a dose of more than 40 mg/day. We present the case of a 51-year-old patient with systemic lupus erythematosus and secondary Sjögren’s syndrome with severe pulmonary involvement four years after the diagnosis. Chest computed tomography revealed neofibrosis and ground glass appearance pattern. After increasing the dose of prednisolone to 60 mg/day, the patient presented a manic episode. There was need of hospitalization and the situation was considered to be secondary to corticosteroids at high doses. Central neurological involvement by organic disease was excluded.We introduced monthly perfusion of cyclophosphamide for six months and later started mycophenolate mofetil 2 g/day, reducing prednisolone to 10 mg/day and maintaining hydroxychloroquine 400 mg/day, with control of disease activity.

Abnormal Histones Acetylation In Patients With Primary Sjögren’s Syndrome

2020 ◽  
Author(s):  
Yan Li ◽  
Xiuying Lv ◽  
Mi Zhou ◽  
Yan He ◽  
Ying Wang ◽  
...  
Keyword(s):  
Histone Acetylation ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Histone H3 ◽  
Lymphocytic Infiltration ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Tnf Α ◽  
Spearman Test ◽  
Sjögren‘S Syndrome

Abstract Background pSS is an autoimmune disease characterized by an exocrine gland epithelium injury with dense lymphocytic infiltration, involvement of the peripheral nervous, pulmonary, blood, kidney, skin, and joint. Aberrant histone acetylation is increasingly thought to plays important role in the pathogenesis of AIDs. However, there is very little data on histone acetylation in pSS. Methods We investigated the expressions of HAT genes (p300, CREEBP and PCAF) by real time PCR in PBMCs from pSS patients. HAT activity and histone H3/ H4 acetylation activity measured by activity kit and histone H3/H4 acetylation verified by WB. Spearman test was utilized to analyze the association between levels of HAT activity and clinical parameters of patients with pSS. Results The expressions of p300, CREEBP and PCAF in PBMCs from pSS patients were decreased in mRNA comparison with HCs. HAT activity and histone H3/H4 acetylation were reduced in PBMCs from pSS patients. We found negatively correlations between the HAT activity and CRP and TNF-α, and positively correlations between the HAT activity and C4. Conclusions Histone hypoacetylation is observed in patients with pSS and involved in the disease duration of Sjogren's syndrome.

scholarly journals MicroRNA in Sjögren’s Syndrome: Their Potential Roles in Pathogenesis and Diagnosis

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
M. Reale ◽  
C. D’Angelo ◽  
E. Costantini ◽  
M. Laus ◽  
A. Moretti ◽  
...  
Keyword(s):  
B Cells ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Lymphocytic Infiltration ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Exocrine Glands ◽  
Autoantibody Production ◽  
Systemic Autoimmunity ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SS) or sicca syndrome was described by Swedish ophthalmologist Sjögren in the year 1933 for the first time. The etiology of the SS is multifunctional and includes a combination of genetic predisposition and environmental as well as epigenetic factors. It is an autoimmune disease characterized by features of systemic autoimmunity, dysfunction, and inflammation in the exocrine glands (mainly salivary and lacrimal glands) and lymphocytic infiltration of exocrine glands. In fact, the involvement of lacrimal and salivary glands results in the typical features of dry eye and salivary dysfunction (xerostomia). Only in one-third of the patients also present systemic extraglandular manifestations. T cells were originally considered to play the initiating role in the autoimmune process, while B cells were restricted to autoantibody production. In recent years, it is understood that the roles of B cells are multiple. Moreover, autoantibodies and blood B cell analysis are major contributors to a clinical diagnosis of Sjögren’s syndrome. Recently, there has been rising interest in microRNA implication in autoimmunity. Unfortunately, to date, there are only a few studies that have investigated their participation in SS etiopathogenesis. The purpose of this work is to gather the data present in the literature to clarify this complex topic.

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