Antimalarials in Sjogren's syndrome-the Greek experience

Sjogren's syndrome (SS) is a chronic autoimmune rheumatic disorder characterized by lymphocytic infiltration and destruction of exocrine glands, mainly of salivary and lacrimal glands, leading to dryness of mouth and eyes. It can occur either alone (primary SS) or in association with almost every systemic autoimmune rheumatic SS). Usually, SS patients have slowly progressive disease confined in exocrine glands, however, in approximately one third of primary SS patients the disorder presents a systemic and progressive course with involvement of diverse extraglandular sites and in a small but significant number of patients with lymphoid neoplasia development. Although the aetiology of SS remains unknown, chronic immune system stimulation is thought to play a central role in the pathogenesis of the disorder, as illustrated by several indices of immunological hyperactivity, including various autoantibodies, polyclonal hypergammaglobulinemia and circulating paraproteins. To date, treatment of SS remains largely empirical and symptomatic, and no clinical trial has been proved capable to change the course of the disease. Hydroxychloroquine has been successfully applied for the treatment of arthralgias, myalgias and general constitutive symptoms of SS patients. In an initial small open study, hydroxychloroquine administration has been shown to improve features of immunological hyperreactivity, such as hyper-gammaglobulinaemia and autoantibody levels. However, large prospective double-blind studies are still needed to assess the long-term efficacy of hydroxychloroquine in preventing extraglandular involvement and lymphoma development in primary SS patients.

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

Journal of Immunology Research ◽

10.1155/2018/7510174 ◽

2018 ◽

Vol 2018 ◽

pp. 1-8 ◽

Cited By ~ 14

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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MANAGEMENT OF SJOGREN'S SYNDROME THROUGH AYURVEDA : A CASE STUDY

International Journal of Research in Medical Sciences & Technology ◽

10.37648/ijrmst.v11i01.006 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Dr Raja Singla ◽

Dr Harish Kumar

Keyword(s):

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Autoimmune Disorder ◽

Lymphocytic Infiltration ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Exocrine Glands ◽

Dry Eyes ◽

Sjögren‘S Syndrome

Sjögren's syndrome is a chronic, slowly progressing autoimmune disorder characterised by lymphocytic infiltration of exocrine glands resulting in xerostomia and dry eyes (conjunctivitis). 1) The disease can present as an entity alone or in association with other auto-immune diseases like Scleroderma, RA, SLE & auto-immune thyroid disease. 2) In this article, attempt is made to understand the Sjögren's syndrome & its treatment in ayurveda by considering the concept of udakvaha srotodushti, vyadhikshamatva and vatadhikya vatrakta. Keywords:Auto-immune, Vatadhikya Vatrakta, Sjögren's syndrome, Udakvaha srotodushti

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Lymphocytic infiltration of saslivary ducts: A histopathologic lesion specific for primary Sjögren's syndrome?

Arthritis & Rheumatism ◽

10.1002/art.1780350420 ◽

1992 ◽

Vol 35 (4) ◽

pp. 481-482 ◽

Cited By ~ 6

Author(s):

Jean-Paul Leroy ◽

Yvon-Louis Pennec ◽

Guy Letoux ◽

Pierre Youinou

Keyword(s):

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Lymphocytic Infiltration ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Primary Sjögren’S Syndrome ◽

Primary Sjogren’S Syndrome ◽

Primary Sjögren's Syndrome ◽

Sjögren‘S Syndrome

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Sjogren’s Syndrome Presenting with Solely Cutaneous Features

Diagnostics ◽

10.3390/diagnostics11071260 ◽

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.

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Abnormal Histones Acetylation In Patients With Primary Sjögren’s Syndrome

10.21203/rs.3.rs-25993/v1 ◽

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.

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Sjögren’s syndrome

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0456 ◽

2020 ◽

pp. 4532-4537

Author(s):

Wan-Fai Ng

Keyword(s):

Sjögren’S Syndrome ◽

Cell Activation ◽

Immune Cell ◽

Sjögren's Syndrome ◽

Association Studies ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Exocrine Glands ◽

Lymphocytic Infiltrates ◽

Sjögren‘S Syndrome

Sjögren’s syndrome is an autoimmune connective tissue disease characterized by dryness of the eyes and mouth and lymphocytic infiltrates in the salivary, lachrymal, and other exocrine glands. Its cause is not known, but it may be primary or associated with other autoimmune diseases (secondary) and it affects women more than men (ratio 9–15:1). The aetiology of SS remains elusive, although genetic susceptibility and environmental triggers both play a role. Genome-wide association studies have identified several disease-susceptibility loci. Infections with various viruses have been associated with an SS-like syndrome. Current evidence suggests that environmental or endogenous antigen triggers immune cell activation and autoantibody production, leading to a self-perpetuating inflammatory response in genetically susceptible individuals, and resulting in destruction of exocrine glands.

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Efficacy and safety of abatacept in active primary Sjögren’s syndrome: results of a phase III, randomised, placebo-controlled trial

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-218599 ◽

2020 ◽

pp. annrheumdis-2020-218599

Author(s):

Alan N Baer ◽

Jacques-Eric Gottenberg ◽

E William St Clair ◽

Takayuki Sumida ◽

Tsutomu Takeuchi ◽

...

Keyword(s):

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Controlled Trial ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Phase Iii ◽

Open Label ◽

Double Blind ◽

Patient Reported ◽

Sjögren‘S Syndrome

ObjectivesTo evaluate efficacy and safety of abatacept in adults with active primary Sjögren’s syndrome (pSS) in a phase III, randomised, double-blind, placebo-controlled trial.MethodsEligible patients (moderate-to-severe pSS [2016 ACR/European League Against Rheumatism (EULAR) criteria], EULAR Sjögren’s Syndrome Disease Activity Index [ESSDAI] ≥5, anti-SS-related antigen A/anti-Ro antibody positive) received weekly subcutaneous abatacept 125 mg or placebo for 169 days followed by an open-label extension to day 365. Primary endpoint was mean change from baseline in ESSDAI at day 169. Key secondary endpoints were mean change from baseline in EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) and stimulated whole salivary flow (SWSF) at day 169. Other secondary clinical endpoints included glandular functions and patient-reported outcomes. Selected biomarkers and immune cell phenotypes were examined. Safety was monitored.ResultsOf 187 patients randomised, 168 completed double-blind period and 165 continued into open-label period. Mean (SD) baseline ESSDAI and ESSPRI total scores were 9.4 (4.3) and 6.5 (2.0), respectively. Statistical significance was not reached for primary (ESSDAI −3.2 abatacept vs −3.7 placebo, p=0.442) or key secondary endpoints (ESSPRI, p=0.337; SWSF, p=0.584). No clinical benefit of abatacept over placebo at day 169 was seen with other clinical and PRO endpoints. Relative to baseline, abatacept was associated with significant differences vs placebo in some disease-relevant biomarkers (including IgG, IgA, IgM-rheumatoid factor) and pathogenic cell subpopulations (post hoc analyses). No new safety signals were identified.ConclusionsAbatacept treatment did not result in significant clinical efficacy compared with placebo in patients with moderate-to-severe pSS, despite evidence of biological activity.

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