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 Involvement of Aquaporins in the Pathogenesis, Diagnosis and Treatment of Sjögren’s Syndrome

2018 ◽  
Vol 19 (11) ◽  
pp. 3392 ◽  
Author(s):  
Muhammad Soyfoo ◽  
Clara Chivasso ◽  
Jason Perret ◽  
Christine Delporte
Keyword(s):  
Salivary Glands ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Keratoconjunctivitis Sicca ◽  
Lymphocytic Infiltration ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Chronic Autoimmune Disease ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands resulting in diminished production of saliva and tears. The pathophysiology of SS has not yet been fully deciphered. Classically it has been postulated that sicca symptoms in SS patients are a double step process whereby lymphocytic infiltration of lacrimal and salivary glands (SG) is followed by epithelial cell destruction resulting in keratoconjunctivitis sicca and xerostomia. Recent advances in the field of the pathophysiology of SS have brought in new players, such as aquaporins (AQPs) and anti AQPs autoantibodies that could explain underlying mechanistic processes and unveil new pathophysiological pathways offering a deeper understanding of the disease. In this review, we delineate the link between the AQP and SS, focusing on salivary glands, and discuss the role of AQPs in the treatment of SS-induced xerostomia.

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.

scholarly journals Long term course of tear gland function in patients with keratoconjunctivitis sicca and Sjogren's syndrome

1997 ◽  
Vol 81 (6) ◽  
pp. 435-438 ◽  
Author(s):  
A A Kruize ◽  
O P van Bijsterveld ◽  
R J Hene ◽  
P C M de Wilde ◽  
T E W Feltkamp ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Keratoconjunctivitis Sicca ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Gland Function ◽  
Sjögren‘S Syndrome

Beta2Microglobulin and Lymphocytic Infiltration in Sjögren's Syndrome

1975 ◽  
Vol 293 (24) ◽  
pp. 1228-1231 ◽  
Author(s):  
Joseph P. Michalski ◽  
Troy E. Daniels ◽  
Norman Talal ◽  
Howard M. Grey
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Lymphocytic Infiltration ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome
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