Autoantibodies and Their Role in Scleroderma Clinical Care

Domsic, Robyn T.; Medsger, Thomas A.

doi:10.1007/s40674-016-0050-y

Cited by 25 publications

(23 citation statements)

References 57 publications

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“…Indeed, in many patients, skin thickness may regress during the follow-up, even without treatment. 24 Finally, the frequency of joint contractures was significantly higher in anti-RNAP3+ patients than in anti-RNAP3in the EUSTAR registry (46% vs 30%, respectively). 10 Association with SRC.…”

Section: Do We Need To Identify Anti-rnap3 In Definite Ssc Patients?mentioning

confidence: 85%

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Anti-RNA polymerase III antibodies in patients with suspected and definite systemic sclerosis: Why and how to screen

Lazzaroni

Airò

2018

Journal of Scleroderma and Related Disorders

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Anti-RNA Polymerase III antibodies are the most frequent anti-nuclear antibodies in systemic sclerosis, after anticentromere and anti-Topoisomerase I. Considering their specificity for systemic sclerosis, they have been included in 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for systemic sclerosis. They were first identified in 1993 using an immunoprecipitation method; the subsequent diffusion of commercial assays, based on the enzyme-linked immunosorbent assay or multiplex line immunoblot techniques, has allowed an increasing number of systemic sclerosis patients to be tested for this autoantibody; nevertheless, the diffusion of this test in systemic sclerosis patients is probably still sub-optimal. Anti-RNA Polymerase III antibodies have been associated with important clinical manifestations: rapid and diffuse cutaneous involvement, joint contractures, scleroderma renal crisis, gastric antral vascular ectasia and malignancies synchronous to systemic sclerosis onset. Moreover, other possible clinical associations, including pulmonary hypertension, still need confirmation. Since the correct approach for screening for anti-RNA Polymerase III antibodies in patients with suspected or definite systemic sclerosis is still debated, possible strategies are proposed here. Moreover, issues that are still controversial are discussed, including the interpretation of multiple simultaneous positivity for anti-RNA Polymerase III antibodies and other autoantibodies in line immunoassay, and the possible relevance of anti-RNA Polymerase III antibodies titre.

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Section: Do We Need To Identify Anti-rnap3 In Definite Ssc Patients?mentioning

confidence: 85%

“…Indeed, in many patients, skin thickness may regress during the follow-up, even without treatment. 24…”

Section: Introductionmentioning

confidence: 99%

Anti-RNA polymerase III antibodies in patients with suspected and definite systemic sclerosis: Why and how to screen

Lazzaroni

Airò

2018

Journal of Scleroderma and Related Disorders

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“…However, their utility in ILD patients has different values. Anticentromere Antibody (ACA, also named CENtromere Protein B, CENP-B) and anti-Scl70 are associated with SSc [93], but the former is protective for ILD, while the latter is associated with severe forms [94]. Anti-Sm and Anti-RNP can be present in several CTDs, but are specific for SLE, while anti-U1-RNP is specific for MCTD [95].…”

Section: First-line Autoimmunity Examsmentioning

confidence: 99%

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Sambataro

Pignataro³

et al. 2020

Diagnostics

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The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD.

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“…A point of interest comes from patient 1837, who was not included in our ACA analysis due to lack of an ACA confirmatory test. However, since patient 1837 has confirmed expression of RNAIII autoantibodies, we can reasonably infer the absence of ACA (Domsic and Medsger, 2016). Furthermore, the ATAC-seq profile of subject 1837 clustered independently with the ACA negative patients, suggesting that chromatin accessibility profiles may predict autoantibody expression.…”

Section: Serum Autoantibody Profiles Are Correlated With Th2-related mentioning

confidence: 81%

Distinct T cell chromatin landscapes in scleroderma subtypes

Dou

Zhao

Abe

et al. 2021

Preprint

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Systemic sclerosis (SSc; scleroderma) is a poorly understood autoimmune rheumatic disease that primarily affects women. The clinical hallmark is hardening of the skin, but internal organ dysfunction is the leading cause of death. Diagnosis and treatment are complicated by heterogeneity within the disease including variable lethality, fibrosis severity, serum autoantibody production, and internal organ involvement. Important gaps remain in our knowledge of the exact molecular and cellular pathways underlying distinct SSc subtypes. Herein, we identify genome-wide chromatin accessibility profiles of peripheral CD4+ T cells to distinguish and better understand the observed heterogeneity in SSc patients. We identify a link between serum anticentromere autoantibody (ACA) subtype and elevated levels of T helper 2 (Th2) cells and increased chromatin access at gene loci encoding fibrosis-driving Th2 cytokines IL4, IL13, and IL4 receptor. Biological sex followed by autoantibody subtype are the predominant variables associated with differences in CD4+ T cell epigenomic profiles, while mycophenolate mofetil treatment appeared to have no effect. These results suggest new mechanistic basis and therapeutic strategies to address SSc, especially the ACA+ subtype that is associated with pulmonary arterial hypertension.

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Autoantibodies and Their Role in Scleroderma Clinical Care

Cited by 25 publications

References 57 publications

Anti-RNA polymerase III antibodies in patients with suspected and definite systemic sclerosis: Why and how to screen

Anti-RNA polymerase III antibodies in patients with suspected and definite systemic sclerosis: Why and how to screen

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Distinct T cell chromatin landscapes in scleroderma subtypes

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