Anti-RNA Polymerase III Antibodies in the Diagnosis of Scleroderma Renal Crisis in the Absence of Skin Disease

Bhavsar, Sankalp; Carmona, Raj

doi:10.1097/rhu.0000000000000167

Cited by 16 publications

(7 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A higher prevalence of musculoskeletal involvement, gastric antral vascular ectasia, ILD, PAH, and scleroderma renal crisis (SRC) has been reported in ARA-positive patients ( 26 , 28 , 36 , 37 ). Notably, SRC was significantly more common in ARA-positive patients compared to ARA-negative ones ( P < 0.0001).…”

Section: Classical Disease-specific Autoantibodies In Clinical Manifementioning

confidence: 99%

Classical Disease-Specific Autoantibodies in Systemic Sclerosis: Clinical Features, Gene Susceptibility, and Disease Stratification

et al. 2020

View full text Add to dashboard Cite

Systemic sclerosis (SSc) is an autoimmune disease characterized by abnormalities in microcirculation, extracellular matrix accumulation, and immune activation. Autoantibodies are markers of immune abnormalities and provide diagnostic and predictive value in SSc. Anti-topoisomerase antibodies (ATAs), anticentromere antibodies (ACAs), and anti-RNA polymerase antibodies (ARAs) are the three classical specific antibodies with the highest availability and stability. In this review, we provide an overview of the recent progress in SSc research with respect to ATAs, ACAs, and ARAs, focusing on their application in distinguishing clinical phenotypes, such as malignancy and organ involvement, identifying genetic background in human leukocyte antigen (HLA) or non-HLA alleles, and their potential roles in disease pathogenesis based on the effects of antigen-antibody binding. We finally summarized the novel analysis using ATAs, ACAs, and ARAs on more detailed disease clusters. Considering these advantages, this review emphasizes that classical SSc-specific autoantibodies are still practical and have the potential for patient and risk stratification with applications in precise medicine for SSc.

show abstract

Section: Classical Disease-specific Autoantibodies In Clinical Manifementioning

confidence: 99%

Classical Disease-Specific Autoantibodies in Systemic Sclerosis: Clinical Features, Gene Susceptibility, and Disease Stratification

et al. 2020

View full text Add to dashboard Cite

show abstract

“…In line with previous reports, the RP3 cluster in our cohort was associated with dcSSc and higher risk for renal crisis. [67][68][69][70] In their study, Patterson et al found 2 different autoantibody clusters, depending on the intensity of R3 staining (cluster RNAP III strong and cluster RNAP III weak). 21 Since RP3 titers may change over time 68 , the authors concluded, that the clusters RNAP III strong and RNAP III weak may represent different temporal stages of SSc disease.…”

Section: Discussionmentioning

confidence: 94%

Comprehensive Autoantibody Profiles in Systemic Sclerosis: Clinical Cluster Analysis

Höppner

Tabeling

Casteleyn

et al. 2022

Preprint

View full text Add to dashboard Cite

Background: Systemic sclerosis (SSc) belongs to the group of connective tissue diseases and is associated with the occurrence of disease-specific autoantibodies. Although it is still controversial whether these antibodies contribute to pathogenesis, there are new insights into the development of these specific antibodies and their possible pathophysiological properties. Interestingly, they are associated with specific clinical manifestations, but for some rarer antibodies this association is not fully clarified. The aim of this study is a comprehensive analysis of the serum autoantibody status in patients with SSc followed by correlation analyses of autoantibodies with the clinical course of the disease. Methods: Serum from SSc patients was analyzed using a line blot (EUROLINE, EUROIMMUN AG) for SSc-related autoantibodies. Autoantibodies to centromere, Topo-1, antimitochondrial antibodies (AMA) M2 subunit, angiotensin II type 1 receptors (AT1R) and endothelin-1 type-A-receptors (ETAR) were also determined by ELISA. We formed immunological clusters and used principal components analysis (PCA) to assign specific clinical characteristics to these clusters. Results: A total of 372 SSc patients were included. 95.3% of the patients were antinuclear antibody positive and in 333 patients at least one SSc specific antibody could be detected. Four immunological clusters could be found by PCA. Centromere, Topo-1 and RP3 all formed own clusters, which are associated with distinct clinical phenotypes. We found that patients with an inverted phenotype, such as limited cutaneous SSc patients within the Topo-1 cluster show an increased risk for ILD compared to ACA positive patients. Anti-AT1R and anti-ETAR autoantibodies were measured in 176 SSc patients; no association with SSc disease manifestation was found. SSc patients with AMA-M2 antibodies showed an increased risk of cardiovascular events. Conclusion: In our in large cluster analysis, which included an extended autoantibody profile, we were able to show that serologic status of SSc patients provides important clues to disease manifestation, co-morbidities and complications. Line blot was a reliable technique to detect autoantibodies in SSc and detected rarer autoantibodies in 42% of our patients.

show abstract

“…Skin sclerosis was absent at SRC onset, but the two patients eventually developed diffuse and rapidly progressive skin thickening. 66 Conversely, ILD that requires aggressive therapy and severe peripheral ulcers/gangrene rarely occurred in this subgroup. The prognosis of patients with anti-RNAP III Abs was the worst in all SSc patients until angiotensin-converting enzyme (ACE) inhibitors were developed.…”

Section: Ssc-specific Autoabsmentioning

confidence: 97%

Anti-nuclear autoantibodies in systemic sclerosis : News and perspectives

Hamaguchi

Takehara

2018

Journal of Scleroderma and Related Disorders

View full text Add to dashboard Cite

Systemic sclerosis is a connective tissue disorder characterized by microvascular damage and excessive fibrosis of the skin and internal organs. One hallmark of the immunological abnormalities in systemic sclerosis is the presence of anti-nuclear antibodies, which are detected in more than 90% of patients with systemic sclerosis. Anti-centromere antibodies, anti-DNA topoisomerase I antibodies, and anti-RNA polymerase III antibodies are the predominant anti-nuclear antibodies found in systemic sclerosis patients. Other systemic sclerosis-related anti-nuclear antibodies include those targeted against U3 ribonucleoprotein, Th/To, U11/U12 ribonucleoprotein, and eukaryotic initiation factor 2B. Anti-U1 ribonucleoprotein, anti-Ku antibodies, anti-PM-Scl, and anti-RuvBL1/2 antibodies are associated with systemic sclerosis overlap syndrome. Anti-human upstream binding factor, anti-Ro52/TRIM21, anti-B23, and anti-centriole antibodies do not have specificity to systemic sclerosis, but are sometimes detected in sera from patients with systemic sclerosis. Identification of each systemic sclerosis-related antibody is useful to diagnose and predict organ involvement, since the particular type of systemic sclerosis-related antibodies is often predictive of clinical features, severity, and prognosis. The clinical phenotypes are largely influenced by ethnicity. Currently, an immunoprecipitation assay is necessary to detect most systemic sclerosisrelated antibodies; therefore, the establishment of an easy, reliable, and simple screening system is warranted.

show abstract

Anti-RNA Polymerase III Antibodies in the Diagnosis of Scleroderma Renal Crisis in the Absence of Skin Disease

Cited by 16 publications

References 6 publications

Classical Disease-Specific Autoantibodies in Systemic Sclerosis: Clinical Features, Gene Susceptibility, and Disease Stratification

Classical Disease-Specific Autoantibodies in Systemic Sclerosis: Clinical Features, Gene Susceptibility, and Disease Stratification

Comprehensive Autoantibody Profiles in Systemic Sclerosis: Clinical Cluster Analysis

Anti-nuclear autoantibodies in systemic sclerosis : News and perspectives

Contact Info

Product

Resources

About