Sjögren’s syndrome: History, clinical and pathological features

Parke, Anne; Ww, Buchanan

doi:10.1007/s10787-998-0012-6

Cited by 14 publications

(4 citation statements)

References 62 publications

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“…Although the primary clinical features of this disease were described by W.B. Hadden in 1888, the sicca syndrome as a systemic disease was first appreciated in 1933 by a Swedish ophthalmologist Henrik Samuel Conrad Sjögren [7]. In his thesis, Dr. Sjögren clarified that keratoconjunctivitis sicca, coined by himself, had no relation to xerophthalmia resulting from vitamin A deficiency.…”

Section: Sjogren’s Syndromementioning

confidence: 99%

Mouse Models of Primary Sjogren’s Syndrome

Park¹,

Gauna²,

Cha

2015

CPD

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Sjögren’s syndrome (SjS) is a chronic autoimmune disorder characterized by immune cell infiltration and progressive injury to the salivary and lacrimal glands. As a consequence, patients with SjS develop xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). SjS is the third most common rheumatic autoimmune disorder, affecting 4 million Americans with over 90% of patients being female. Current diagnostic criteria for SjS frequently utilize histological examinations of minor salivary glands for immune cell foci, serology for autoantibodies, and dry eye evaluation by corneal or conjunctival staining. SjS can be classified as primary or secondary SjS, depending on whether it occurs alone or in association with other systemic rheumatic conditions, respectively. Clinical manifestations typically become apparent when the disease is relatively advanced in SjS patients, which poses a challenge for early diagnosis and treatment of SjS. Therefore, SjS mouse models, because of their close resemblance to the human SjS, have been extremely valuable to identify early disease markers and to investigate underlying biological and immunological dysregulations. However, it is important to bear in mind that no single mouse model has duplicated all aspects of SjS pathogenesis and clinical features, mainly due to the multifactorial etiology of SjS that includes numerous susceptibility genes and environmental factors. As such, various mouse models have been developed in the field to try to recapitulate SjS. In this review, we focus on recent mouse models of primary SjS and describe them under three categories of spontaneous, genetically engineered, and experimentally induced development of SjS-like disease. In addition, we discuss future perspectives of SjS mouse models highlighting pros and cons of utilizing mouse models and demands for improved models.

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Section: Sjogren’s Syndromementioning

confidence: 99%

Mouse Models of Primary Sjogren’s Syndrome

Park¹,

Gauna²,

Cha

2015

CPD

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“… 33 , 34 As expected, the most common diagnosis was KCS, as it has been reported. 32 , 35–37 Similarly, other ophthalmologic diagnoses had been described. Akpek et al reported the following ocular involvement in a group of SS patients: corneal perforation (3.1%), corneal ulcer (0.6%), corneal scarring (4.3%), papillary conjunctivitis (7.4%), follicular conjunctivitis (2.5%), uveitis (1.2%), scleritis/episcleritis (0.6%), optic neuropathy (1.8%), and orbital inflammation (1.8%).…”

Section: Discussionmentioning

confidence: 91%

Ocular Manifestations in Colombian Patients with Systemic Rheumatologic Diseases

Uribe-Reina¹,

Muñoz-Ortíz²,

Cifuentes-González³

et al. 2021

OPTH

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Purpose To establish the prevalence of ocular involvement in a Colombian population with rheumatologic diseases. Design Observational cross-sectional study. Methods We included a probabilistic sample size of 797 patients who attended a rheumatologic disease center in Bogotá, Colombia. Statistical analysis with descriptive measures and Chi-square independence test between rheumatologic diseases and ophthalmological symptoms and diseases was performed. Results Eighty-four percent of the population were women, and the mean age was 54.61± 15.64 years. The most common condition was rheumatoid arthritis (33.37%), followed by fibromyalgia (22.71%), Sjögren Syndrome (19.72%), and systemic lupus erythematosus (9.91%). Almost 7% of the patients presented polyautoimmunity. Thirty-five percent of the patients reported one or more ophthalmological symptoms, being dry eye sensation the most common (30.86%), followed by ocular pain (2.76%), red-eye, and decreased visual acuity (both 2.63%). Similarly, 21.45% of the patients presented one or more ophthalmological diagnoses, being keratoconjunctivitis sicca the most common (15.93%), followed by cataract, uveitis (1.38% each), and scleritis (1.25%). Conclusion Almost a third of the patients reported any ocular involvement. It is crucial to be aware of the most common ophthalmic manifestations among the different rheumatologic diseases in our population, to offer early specialist referral and timely treatment.

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“…Sjogren's syndrome (SS) is a chronic, progressive, and systemic autoinflammatory disease, with exocrine (mainly salivary and lacrimal) glands as the main target organs, leading to the development of sicca symptoms [1,2]. They are the main clinical feature of the disease.…”

Section: Introductionmentioning

confidence: 99%

Perspective Chapter: Pulmonary System and Sjogren’s Syndrome

Ehtesham¹,

Tiwari²,

George³

et al. 2022

Idiopathic Pulmonary Fibrosis

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Sjogren’s syndrome (SS) is a connective tissue disease targeting the exocrine glands with subsequent sicca symptoms mainly in eyes and mouth. Respiratory symptoms may be the most frequent extraglandular manifestation following fatigue and pain. Mucosal dysfunction may affect the upper and lower airways, being the small airways more frequently involved. Parenchymal disease carries most of the morbidity and mortality. Nonspecific interstitial pneumonia (NSIP) is the most common radiographic feature, whereas the fibrotic NSIP type is the most reported finding in biopsies. Pulmonary lymphoma may arise from bronchial-associated lymphoid tissue lesions, and although rare, it is prevalent in SS. Chronic hypertrophic bronchial wall changes may ascribe to the various cystic lesions. Under their presence, possible lymphocytic interstitial pneumonia, amyloidosis, and lymphoma should be explored. Pulmonary arterial hypertension may present as frequently as in lupus, especially in Asian populations. Advanced knowledge in the pathogenesis has helped in understanding the various presentations within the respiratory system, contrasting with the scarce therapeutic options to treat both the airway and parenchymal disease. Anti-fibrotic parenchymal lung therapy offers promising outcomes. The pulmonary involvement in SS may associate with a decline in quality of life and reduced life expectancy. Subsequently, clinicians should know these facts for a timely intervention.

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Sjögren’s syndrome: History, clinical and pathological features

Cited by 14 publications

References 62 publications

Mouse Models of Primary Sjogren’s Syndrome

Mouse Models of Primary Sjogren’s Syndrome

Ocular Manifestations in Colombian Patients with Systemic Rheumatologic Diseases

Perspective Chapter: Pulmonary System and Sjogren’s Syndrome

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