P083 Primary Goujerot- Sjögren Syndrome: a pediatric case report

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Djohra Hadef ◽  
Samy Slimani ◽  
Yamina Ouchen
Keyword(s):  
Autoimmune Disease ◽  
Clinical Signs ◽  
Lymphocytic Infiltration ◽  
Initial Therapy ◽  
Sjögren Syndrome ◽  
Lower Limbs ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Organ Systems ◽  
One Year

Abstract Background Gourgerot-Sjögren syndrome (GSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to dryness of the mucous membranes. It may also involve other organs and organ systems. GSS can be primary (pGSS) or secondary due to other autoimmune diseases. pSGS mainly affects women over 40 years old, but can occur at any age. GSS is rare in children and is often secondary to other diseases. We report the case of a 12-year-old girl with primary Gougerot-Sjögren Syndrome Methods and results A 12-year-old girl from Batna (Algeria) presented to her pediatrician in 2016 for a rash with arthralgia. She had no relevant past medical history and is the child of a non-consanguineous marriage. Clinical examination found a well-nourished child with a purpuric rash on her lower limbs. She complained of arthralgia without clinical signs of arthritis. The child also complained of a sensation of a foreign body and burning in both eyes. The eye exam was normal except for a positive Schirmer's test. There were no signs of parotitis. Laboratory tests showed an erythrocyte sedimentation rate (ESR) of 84 mm the first h and a negative C-reactive protein (CRP). She was also Leukopenic. Serological testing resulted in a positive Anti SS-A and Anti SS-B. The abdominal pelvic ultrasound was normal. The patient was diagnosed with primary Gougerot-Sjögren Syndrome. Artificial tears and hydroxychloroquine were started as initial therapy. One year later we added an immunosuppressant (Methotrexate) due to the persistence of clinical signs. Conclusion Our case is particular in that primary Gougerot-Sjögren Syndrome is rare in children. Routine follow- up with this patient is important to determine whether it is indeed pGSS with pediatric onset or GSS secondary to another autoimmune disease.

scholarly journals Cutting the “Gordian Knot” — Cardiac Involvement in Primary Sjögren Syndrome

2021 ◽  
pp. jrheum.201171
Author(s):  
George Markousis-Mavrogenis ◽  
Sophie I. Mavrogeni
Keyword(s):  
Myocardial Infarction ◽  
Cardiovascular Disease ◽  
Autoimmune Disease ◽  
Cardiac Involvement ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Exocrine Glands ◽  
Primary Sjögren Syndrome ◽  
Increased Risk

Sjögren syndrome (SS) is a systemic autoimmune disease typically characterized by inflammatory involvement of the exocrine glands1. The association of SS with an increased risk of cardiovascular disease (CVD) including manifestations such as stroke and myocardial infarction has been demonstrated by numerous previous studies1.

scholarly journals Lung complications of Sjogren syndrome

2020 ◽  
Vol 29 (157) ◽  
pp. 200021
Author(s):  
Fabrizio Luppi ◽  
Marco Sebastiani ◽  
Nicola Sverzellati ◽  
Alberto Cavazza ◽  
Carlo Salvarani ◽  
...  
Keyword(s):  
Lymphocytic Infiltration ◽  
Airway Disease ◽  
Pulmonary Involvement ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Recent Trial ◽  
Risk Of Death ◽  
Primary Sjögren Syndrome ◽  
Related Quality

Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations, including those regarding the lung. Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders.Patients with pSS-ILD report impaired health-related quality of life and a higher risk of death, suggesting the importance of early diagnosis and treatment of this type of pulmonary involvement. In contrast, airway disease usually has little effect on respiratory function and is rarely the cause of death in these patients.More rare disorders can be also identified, such as pleural effusion, cysts or bullae.Up to date, available data do not allow us to establish an evidence-based treatment strategy in pSS-ILD. No data are available regarding which patients should be treated, the timing to start therapy and better therapeutic options. The lack of knowledge about the natural history and prognosis of pSS-ILD is the main limitation to the development of clinical trials or shared recommendations on this topic. However, a recent trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various ILDs, including those in pSS patients.

scholarly journals Isolated Arteritis of Both Lower Limbs

2018 ◽  
Vol 119 (1) ◽  
pp. 70-75
Author(s):  
Miroslav Průcha ◽  
Alena Šnajdrová ◽  
Pavel Zdráhal
Keyword(s):  
Autoimmune Disease ◽  
Lower Extremity ◽  
Lower Limb ◽  
Imaging Techniques ◽  
Rare Condition ◽  
Rapid Diagnosis ◽  
Vascular Imaging ◽  
Lower Limbs ◽  
Systemic Autoimmune Disease ◽  
Successful Therapy

Isolated arteritis of the lower limb vessels is an extremely rare condition. The use of modern vascular imaging techniques substantially facilitates and accelerates the diagnostics. In the isolated lower limb arteritis, it is always necessary to exclude Takayasu’s and giant-cell arteritis. We present the case of a female patient with an isolated lower extremity arteritis without any other symptoms of systemic vascular damage or systemic autoimmune disease. Immunosuppressive therapy is obligatory in this case. Interdisciplinary co-operation is required for rapid diagnosis and successful therapy. Our patient has consented to the publication of this report.

scholarly journals AB0947 RECIPROCAL IMPACT OF FIBROMYALGIA ON DISEASE CHARACTERISTICS AND PHYSICAL AND PSYCOLOGICAL DOMAINS IN SJOGREN SYNDROME: CROSS SECTIONAL OBSERVATIONAL STUDY.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1771.1-1772
Author(s):  
A. Capacci ◽  
P. Rubortone ◽  
V. Varriano ◽  
A. Paglionico ◽  
S. Perniola ◽  
...  
Keyword(s):  
Disease Activity ◽  
Life Style ◽  
Sjögren Syndrome ◽  
Control Group ◽  
Sjogren Syndrome ◽  
Differential Distribution ◽  
Cross Sectional ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
The Impact

Background:Sjogren Syndrome (SS) is an autoimmune exocrinopathy, resulting mainly in ocular and oral dryness, with approximately half of patients displaying symptoms from different organ systems, further adding to the heterogeneous clinical phenotype of the disease. Fatigue and pain are common systemic symptoms in patients with primary SS and fibromyalgia is a frequent condition associated with chronic diseases.Objectives:The aim of the study was to evaluate the impact of concomitant fibromyalgia in patients with Sjogren Syndrome in terms of clinical features and disease activity.Methods:50 patients with Sjogren Syndrome were enrolled in the study (100% female, age: 53.7 ± 13.2 years and disease duration: 8.7 ± 5.3 years), 25(50.0%) with concomitant fibromyalgia (SS/Fibro-group) and 25(50.0%) without (SS-group). 36 patients with primary fibromyalgia (Fibro-group) were included as control group. At study entry, demographic, educational, life-style and clinical parameters were recorded for each patient. SS was diagnosed according to the American College of Rheumatology (ACR) classification criteria (1) and fibromyalgia was diagnosed according to criteria for fibromyalgia defined by ACR (2). Moreover, each patient with fibromyalgia, with and without concomitant SS, was asked to fill a self-reported questionnaire to assess the impact of Fibromyalgia on multiple physical and psycological domains (Italian-FIQR).Results:Stratifying the study cohorts based on the demographic and life-style characteristics, no significant differences were found comparing SS-group, Fibro-group and SS/Fibro-group. However, considering the different organ involvement, SS/Fibro-group were more likely reporting arthralgia symptoms (100.0%) than SS-group (76.0% p=0.02), despite similar clinical evidence of arthritis-synovitis among the two groups (12.0% in both groups respectively, p=1.00). Moreover, SS/Fibro-group showed significantly lower ESSDAI score (2.8 ± 1.7) and higher ESSPRI score (7.0 ± 0.9) compared to SS-group (ESSDAI: 7.5 ± 3.7 p<0.001 and ESSPRI: 5.2 ± 1.4, p<0.001 respectively). Finally, analyzing the differential distribution of individual scores of physical and psycological domains of the Italian-FIQR Questionnaire, SS/Fibro-group did not differ compared to Fibro-group (p>0.05 for all the 21 questions included).Conclusion:SS is affected by concomitant fibromyalgia in terms of subjective-dependent parameters (i.e. joint complaints) however the concomitant SS does not affect the impact of fibromyalgia on physical and psycological domains, even if disease activity is higher in SS patients without fibromyalgia.References:[1]Shiboski SC et al. Arthritis Care Res, 2012[2]Wolf F. et al. Arthritis Rheum 1990Disclosure of Interests:Annunziata Capacci: None declared, Pietro Rubortone: None declared, Valentina Varriano: None declared, Annamaria Paglionico: None declared, Simone Perniola: None declared, Maria Rita Gigante: None declared, Barbara Tolusso: None declared, Stefano Alivernini: None declared, Elisa Gremese Speakers bureau: Abbvie, BMS, Celgene, Jannsen, Lilly, MSD, Novartis, Pfizer, Sandoz, UCB

scholarly journals Associations between Sjögren Syndrome, Sociodemographic Factors, Comorbid Conditions, and Scleritis in a Taiwanese Population-Based Study

2022 ◽  
Vol 12 (1) ◽  
pp. 105
Author(s):  
Ren-Long Jan ◽  
Chung-Han Ho ◽  
Jhi-Joung Wang ◽  
Sung-Huei Tseng ◽  
Yuh-Shin Chang
Keyword(s):  
Logistic Regression ◽  
Lupus Erythematosus ◽  
Population Based ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Research Database ◽  
Continuous Variables ◽  
Comorbid Conditions ◽  
Ocular Surgery

This nationwide, population-based, retrospective, matched case–control study included 111,960 newly diagnosed patients with scleritis who were identified by the International Classification of Diseases, Ninth Revision, Clinical Modification code 379.0, selected from the Taiwan National Health Insurance Research Database. Demographic characteristics, Sjögren syndrome, and comorbid conditions within 1 year before the scleritis diagnosis were examined using univariate logistic regression analyses, and a paired t-test was used for continuous variables. Adjusted logistic regression was used to compare the prognosis odds ratio (OR) of the patients with scleritis with the controls. After adjustment for confounders, patients with Sjögren syndrome were remarkably more likely to have scleritis than the controls (OR = 33.53, 95% confidence interval (CI) = 27.43–40.97, p < 0.001). Other conditions found to have increased odds of scleritis included post ocular pterygium, glaucoma, and scleral surgery (OR  =  4.01, 95% CI  =  3.64–4.43; OR  =  3.16, 95% CI  =  2.24–4.47; OR  =  6.83, 95% CI  =  5.34–8.74, respectively); systemic infections, such as syphilis, tuberculosis, and a human herpes viral infection (OR  =  4.01, 95% CI  =  2.93–5.50; OR  =  2.24, 95% CI  =  1.94–2.58; OR  =  8.54, 95% CI  =  8.07–9.03, respectively); and systemic diseases, such as rheumatoid arthritis, granulomatous vasculitis, systemic lupus erythematosus, ankylosing spondylitis, and gout (OR  =  2.93, 95% CI  =  2.66–3.23; OR  =  7.37, 95% CI  =  3.91–13.88; OR  =  3.18, 95% CI  =  2.63–3.85; OR  =  5.57, 95% CI  =  4.99–6.22; OR  =  2.84, 95% CI  =  2.72–2.96, respectively). The results strongly support an association between Sjögren syndrome, post ocular surgery, systemic infection disease, systemic autoimmune disease, and scleritis.

scholarly journals Salivary 1H-NMR Metabolomics in Primary Sjögren Syndrome. Preliminary Results of a Pilot Case-Control Study

Proceedings ◽  
2019 ◽  
Vol 35 (1) ◽  
pp. 39
Author(s):  
Setti ◽  
Sandri ◽  
Tarentini ◽  
Panari ◽  
Mucci ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
1H Nmr ◽  
Case Control Study ◽  
Case Control ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Exocrine Glands ◽  
Preliminary Results ◽  
Primary Sjögren Syndrome ◽  
Control Study

Primary Sjögren Syndrome (pSS) is a multisystem autoimmune disease which mainly involves exocrine glands, such as salivary and lacrimal. [...]

scholarly journals Cerebral Venous Thrombosis Revealing Primary Sjögren Syndrome: Report of 2 Cases

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
A. Mercurio ◽  
M. Altieri ◽  
V. M. Saraceni ◽  
T. Paolucci ◽  
G. L. Lenzi
Keyword(s):  
Nervous System ◽  
Venous Thrombosis ◽  
Cerebral Venous Thrombosis ◽  
Lymphocytic Infiltration ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Exocrine Glands ◽  
Neurologic Complications ◽  
Primary Sjögren Syndrome ◽  
Syndrome Report

Sjögren syndrome (SS) is an autoimmune disease of the exocrine glands, characterized by focal lymphocytic infiltration and destruction of these glands. Neurologic complications are quite common, mainly involving the peripheral nervous system (PNS). The most common central nervous system (CNS) manifestations are myelopathy and microcirculation vasculitis. However, specific diagnostic criteria for CNS SS are still lacking. We report two cases of primary SS in which the revealing symptom was cerebral venous thrombosis (CVT) in the absence of genetic or acquired thrombophilias.

Prognostic markers of lymphoma development in primary Sjögren syndrome

Lupus ◽  
2019 ◽  
Vol 28 (8) ◽  
pp. 923-936 ◽  
Author(s):  
S Retamozo ◽  
P Brito-Zerón ◽  
M Ramos-Casals
Keyword(s):  
Risk Factors ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Low Grade ◽  
Sjogren Syndrome ◽  
Close Link ◽  
Systemic Involvement ◽  
Primary Sjögren Syndrome

Sjögren syndrome is a systemic autoimmune disease that principally affects women between the fourth and sixth decades of life who present with sicca symptomatology caused by dryness of the main mucosal surfaces. The clinical spectrum of Sjögren syndrome extends from dryness to systemic involvement. Since 1978, Sjögren syndrome has been closely associated with an enhanced risk of lymphoma, one of the most severe complications a patient may develop. Primary Sjögren syndrome patients have a 10–44-fold greater risk of lymphoma than healthy individuals, higher than that reported for systemic lupus erythematosus and rheumatoid arthritis. The close link between lymphoma and Sjögren syndrome is clearly exemplified by the very specific type of lymphoma arising in Sjögren syndrome patients, mainly low-grade B-cell lymphomas (predominantly a marginal zone histological type) with primary extranodal involvement of the major salivary glands (overwhelmingly parotid), with a primordial role of cryoglobulinemic-related markers (both clinical and immunological). The most recent studies support a higher number of risk factors detected in an individual leads to a higher lymphoma risk. A close follow-up of high-risk groups with longitudinal assessments of all known risk factors, including cryoglobulin-related markers and EULAR Sjögren's syndrome disease activity index measurement in particular, is mandatory.

scholarly journals Sjögren Syndrome Complicated with Cystic Lung Disease and Pulmonary Amyloidosis

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Koichiro Takahashi ◽  
Hironori Sadamatsu ◽  
Shinsuke Ogusu ◽  
Kazutoshi Komiya ◽  
Tomomi Nakamura ◽  
...  
Keyword(s):  
Lung Disease ◽  
Respiratory Symptoms ◽  
Lymphocytic Infiltration ◽  
Japanese Woman ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Laboratory Findings ◽  
Cystic Lung ◽  
Cystic Lung Disease ◽  
Pulmonary Amyloidosis

A 72-year-old Japanese woman was noted to have multiple cystic lung shadows and infiltrates on chest radiography and computed tomography (CT). She complained of dryness of the mouth and eyes, but she did not have respiratory symptoms, such as cough, sputum production, and dyspnea. Her laboratory findings showed high titers of anti-SSA/Ro and anti-SSB/La antibodies. Surgical lung biopsy was performed and demonstrated pathologic findings of amyloid light-chain deposition and bronchiolitis with lymphocytic infiltration. Taken altogether, she was diagnosed as Sjögren syndrome with bronchiolitis and pulmonary amyloidosis. Since then, she has been carefully followed up without treatment. After 6 years, the cystic lung lesions on CT gradually enlarged and increased in number, but she remained to have no respiratory symptoms and no manifestations of lymphoma. Here, we report a rare case of Sjögren syndrome complicated with cystic lung disease and pulmonary amyloidosis.

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