Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis

Ingegnoli, Francesca; Ughi, Nicola; Mihai, Carina

doi:10.1016/j.berh.2018.08.005

Cited by 92 publications

(82 citation statements)

References 91 publications

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“…In these studies, the prevalence of scleroderma in SIgAD patients ranged from 0.3%-6.7%, with a weighted average of 0.78%, compared to a prevalence of 0.004%-0.04% in populations with normal serum immunoglobulins. [20,72,81,84,85] (Table 3) It is important to note that the largest of these studies found the lowest prevalence. This may reflect an observation bias, in which only studies which observed systemic sclerosis mentioned it, thus artificially inflating the average prevalence.…”

Section: Scleroderma/systemic Sclerosismentioning

confidence: 99%

The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency

Odineal

Gershwin

2019

Clinic Rev Allerg Immunol

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Selective Immunoglobulin A Deficiency (SIgAD) is the most common primary immunodeficiency, defined as an isolated deficiency of IgA (less than 0.07 g/L). Although the majority of people born with IgA deficiency lead normal lives without significant pathology, there is nonetheless a significant association of IgA deficiency with mucosal infection, increased risks of atopic disease, and a higher prevalence of autoimmune disease. To explain these phenomena, we have performed an extensive literature review to define the geoepidemiology of IgA deficiency and particularly the relative risks for developing Systemic Lupus Erythematosus, hyperthyroidism, hypothyroidism, type 1 diabetes mellitus, Crohn's Disease, ulcerative colitis, rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, and vitiligo; these diseases have strong data to support an association. We also note weaker associations with scleroderma, celiac disease, autoimmune hepatitis, immune thrombocytopenic purpura, and autoimmune hemolytic anemia. Minimal if any associations are noted with myasthenia gravis, lichen planus, and multiple sclerosis. Finally, more recent data provide clues on the possible immunologic mechanisms that lead to the association of IgA deficiency and autoimmunity; these lessons are important for understanding the etiology of autoimmune disease. 2 3 Introduction:

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Section: Scleroderma/systemic Sclerosismentioning

confidence: 99%

The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency

Odineal

Gershwin

2019

Clinic Rev Allerg Immunol

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“…5 The etiology of scleroderma is unknown although both genetic and environmental factors have been implicated. 6 A mutation in the mouse Fbn1 gene that encodes fibrillin-1 has been implicated in the development of SSc-like disease in the tight skin (TsK) mouse model of SSc. [7][8][9] Subsequent studies using microsatellite markers demonstrated that a 2-cM haplotype on chromosome 15q containing the FBN1 gene was strongly associated with SSc in Choctaw natives, a population with high SSc prevalence.…”

Section: Introductionmentioning

confidence: 99%

“…Systemic sclerosis (SSc) is a chronic multisystem disease characterized by widespread vascular dysfunction and progressive fibrosis of the skin and internal organs 5 . The etiology of scleroderma is unknown although both genetic and environmental factors have been implicated 6 . A mutation in the mouse Fbn1 gene that encodes fibrillin‐1 has been implicated in the development of SSc‐like disease in the tight skin (TsK) mouse model of SSc 7‐9 .…”

Section: Introductionmentioning

confidence: 99%

Co‐existence of Marfan syndrome and systemic sclerosis: A case report and a hypothesis suggesting a common link

Yap

Chong

2020

Int J of Rheum Dis

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Marfan syndrome (MFS), one of the most common inherited connective tissue disorders, is characterized by skeletal, cardiovascular, and ocular abnormalities. It was first described by Bernard Antoine Marfan in 1986. 1 MFS is inherited as an autosomal dominant trait with variable penetrance but there is no family history in at least one-quarter of MFS patients, suggesting the disease may also be caused by new mutations. 2 In 1991, the fibrillin 1 gene (FBN1) which encodes the fibrilin-1 protein, was found to be the locus for mutations that result in MFS FBN1 is located on the long arm of chromosome 15. 3 Fibrillin-1 is a major component of extracellular matrix structures known as microfibrils upon which elastin appears to be deposited. 4 Systemic sclerosis (SSc) is a chronic multisystem disease characterized by widespread vascular dysfunction and progressive fibrosis

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“…Due to its rarity, it is a disease which is uncommon to non-expert rheumatologists, which may lead to late diagnosis of the disease or its complications and jeopardisation of optimal management of the patient. [ 5 – 8 ]…”

Section: Introductionmentioning

confidence: 99%

The efficacy and safety of fecal microbiota transplantation in the treatment of systemic sclerosis

Zhang

Ren

et al. 2020

Medicine

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Background: Systemic sclerosis (SSc) is 1 of the most complex systemic autoimmune diseases.Accumulating evidence suggests that gut microbiota affect the development and function of the immune system and may play a role in the pathogenesis of autoimmune diseases. This new paradigm raises the possibility that many diseases result, at least partially, from microbiota-related dysfunction. This understanding invites the investigation of fecal microbiota transplantation (FMT) in the treatment of SSc. However, no study has specifically and systematically investigated the efficacy and safety of FMT in the treatment of SSc. Thus, this study will systematically and comprehensively appraise the efficacy and safety of FMT in the treatment of SSc. Methods: We will search the following sources without restrictions for date, language, or publication status: PubMed, Web of Science,Cochrane Central Register of Controlled Trials (CENTRAL) Cochrane Library, EMBASE and China National Knowledge Infrastructure. We will apply a combination of Medical Subject Heading (MeSH) and free-text terms incorporating database-specific controlled vocabularies and text words to implement search strategies. We will also search the ongoing trials registered in the World Health Organization's International Clinical Trials Registry Platform. Besides, the previous relevant reviews conducted on FMT for SSc and reference lists of included studies will also be searched. Results: This study will provide a reliable basis for the treatment of SSc with FMT. Conclusions: The findings will be an available reference to evaluate the efficacy and safety of FMT in the treatment of SSc. Registration number: INPLASY202060019.

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Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis

Cited by 92 publications

References 91 publications

The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency

The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency

Co‐existence of Marfan syndrome and systemic sclerosis: A case report and a hypothesis suggesting a common link

The efficacy and safety of fecal microbiota transplantation in the treatment of systemic sclerosis

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