Genetics, Epigenetics, and Genomics of Systemic Sclerosis

Salazar, Gloria A.; Mayes, Maureen D.

doi:10.1016/j.rdc.2015.04.001

Cited by 30 publications

(24 citation statements)

References 99 publications

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“…In contrast to SSS or geleophysic dysplasia, SSc and localized scleroderma are two forms of acquired hardening of the skin . Systemic sclerosis is a complex autoimmune disease with progressive visceral inflammation and fibrosis of two or more organ systems commonly involving the skin . In localized scleroderma, or morphea, localized chronic fibrosis of the skin has various triggers or is idiopathic .…”

Section: Introductionmentioning

confidence: 99%

Initial characterization of stiff skin‐like syndrome in West Highland white terriers

Doelle

Linder

Boche³

et al. 2016

Veterinary Dermatology

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Section: Introductionmentioning

confidence: 99%

Initial characterization of stiff skin‐like syndrome in West Highland white terriers

Doelle

Linder

Boche³

et al. 2016

Veterinary Dermatology

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“…Based on these findings, it appears that dysregulation of the innate immune system might contribute to the activation of key effector cells such as fibroblasts and ECs, leading to production of genes implicated in SSc pathogenesis. Further genetic studies that strongly associate polymorphisms in TLRs and IFN pathways (IRF5, IRF7,IRF8 STAT4) with SSc confirm the correlation of innate immune dysregulation and IFNs with the risk of developing SSc [17,18]. The rationale for studying the role of pathogens is very attractive since the etiology of autoimmunity, as well as the origin innate immune dysregulation is largely unexplained in SSc.…”

Section: Introductionmentioning

confidence: 99%

Fresh Insights into Disease Etiology and the Role of Microbial Pathogens

Farina

2015

Curr Rheumatol Rep

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Pathogens have been implicated in the initiation and/or promotion of systemic sclerosis (scleroderma, SSc); however, no evidence was found to substantiate the direct contribution to this disease in past years. Recently, significant advances have been made in understanding the role of the innate immune system in SSc pathogenesis, supporting the idea that pathogens might interact with host innate immune-regulatory responses in SSc. In light of these findings, we review the studies that identified the presence of pathogens in SSc, along with studies on pathogens implicated in driving the innate immune dysregulation in SSc. The goal of this review is to illustrate how these pathogens, specifically viruses, may play important role both as triggers of the innate immune system, and critical players in the development of SSc disease.

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“…This probably represents the usual pattern of more advanced or severe cases in post-mortem studies. [1], left atrium [2] and the dilated right ventricle [3] and right atrium [4]. Moderate pleural effusion [5] is also demonstrated.…”

Section: Discussionmentioning

confidence: 99%

“…The clinically heterogenous disease manifests in genetically susceptible individuals with a diverse pattern of symptomatology [1]. Hence, patients with SSc represent a host of different manners of disease onset, presentation, course and prognosis.…”

Section: Introductionmentioning

confidence: 99%