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Connective Tissue Disorders
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Laura Atzori, Caterina Ferreli, Franco Rongioletti
Laboratory studies: ANA test is positive in approximately 95% of patients with SSc. Anti-topoisomerase I (anti-Scl-70) antibodies are associated with dcSSc and a higher risk of severe inter-stitial lung disease. Anti-centromere antibody is typically associated with limited cutaneous lcSSc. Antibodies to RNA polymerase III are found in patients with dcSSc and are generally associated with rapidly progressive skin involvement, as well as an increased risk for a renal crisis.
Scleroderma and associated complications
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Yasmeen Jabeen Bhat, Safiya Bashir
The sensitivity of these tests is however quite low, being 30% and 40%, respectively, for ACA and anti-ScL-70 antibodies. Anti-RNA polymerase III antibodies are commonly associated with diffuse cutaneous systemic sclerosis and are predictive of scleroderma renal crisis or development of malignancy in systemic sclerosis [54,55]. Other autoantibodies that may be uncommonly found in systemic sclerosis are given in Table 41.5.
Dysphagia
Published in Sherif Gonem, Ian Pavord, Diagnosis in Acute Medicine, 2017
– Autoimmune screen. This should include ANA, anti-dsDNA (SLE), anti-centromere antibodies or anti-Scl-70 antibodies (systemic sclerosis), anti-RNP antibodies (mixed connective tissue disease) and anti-Jo-1 antibodies (polymyositis).
Relationship between YKL-40 and pulmonary arterial hypertension in systemic sclerosis
Published in Modern Rheumatology, 2019
Tetsuya Furukawa, Kiyoshi Matsui, Masayasu Kitano, Yuichi Yokoyama, Masahiro Sekiguchi, Naoto Azuma, Yasutomo Imai, Seiichi Hirota, Kiyofumi Yamanishi, Hajime Sano
The study group consisted of 78 patients with SSc who were referred to our institution for treatment from August 2014 to April 2017. All patients fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism Classification criteria for SSc [20]. Our patient group included 10 men and 68 women, with a mean age of 62.8 ± 13.4 years (range: 18–83 years) and a mean disease duration of 10.1 ± 9.9 years (range: 0.33–42 years), considering Raynaud’s phenomenon as the onset manifestation of disease, and the modified Rodnan total skin thickness score (mRSS) of 6.5 ± 7.1. SSc-specific antibody showed anti-Scl-70 antibodies in 20 patients, anti-centromere antibodies in 39 patients, and anti-RNA polymerase III antibodies in 2 patients. In 17 patients, the screen for antibodies was negative. Seventy-six patients were antinuclear antibody (ANA) positive and 2 patients were ANA negative. Diffuse cutaneous SSc (dcSSc) was identified in 19 patients, with limited cutaneous SSc (lcSSc) identified in the other 59 patients. Cases confirmed by examination even after steroid treatment (prednisolone, PSL) and/or immunosuppressant were included. Among the 41 patients who had received prior treatment, 35 were treated using PSL, 4 using cyclophosphamide, 3 using mizoribine, 1 using methotrexate, 2 using cyclosporine, 3 using tacrolimus, 12 using salazosulfapyridine, and 6 using D-penicillamine.
Systemic sclerosis complicated by arrhythmogenic right ventricular cardiomyopathy in a Chinese middle-aged female
Published in Modern Rheumatology Case Reports, 2018
Shi-Kun Ma, Chao-Xia Lu, Wei Wu, Yong-Tai Liu, Xue Zhang
A 59-year-old woman from Northern China was admitted to the department of Cardiology at our hospital in March, 2014. She had shortness of breath after exertion, pitting oedema of the lower extremities and Raynaud’s phenomenon (Figure 1, Panel A). Her mother died of heart failure. One of her younger sister had chest tightness after exertion and abnormally enlarged ventricular septum (14.5 mm as shown in echocardiogram), but no more information for diagnosis was available. Careful clinical evaluations as well as auxiliary examinations were performed on this patient. Strongly positive ANA and anti-Scl-70 antibodies were found in serologic tests. Echocardiogram showed huge right ventricle (48 mm, basal diameter in apical 4-chamber view) and enlarged right atrium (53 × 49 mm in apical four-chamber view) (Figure 1, Panel B), as well as severe systolic dysfunction of both ventricles (left ventricular ejection fraction: 30% and tricuspid annular plane systolic excursion: 8 mm), estimated systolic pulmonary artery pressure was 20 mm Hg according to the velocity of the tricuspid valve regurgitation. Cardiac magnetic resonance showed diffused late gadolinium enhancement in both ventricular walls (Figure 1, Panel C).
Disease staging and sub setting of interstitial lung disease associated with systemic sclerosis: impact on therapy
Published in Expert Review of Clinical Immunology, 2018
Peter M. George, Athol U. Wells
SSc is typified by the presence of autoantibodies and antinuclear antibodies are found in 90% of affected patients. Anti-topoisomerase 1 autoantibodies, more commonly known as anti-scleroderma-70 (anti-Scl 70) antibodies, are present in approximately 20% of SSc patients. Over 85% of anti-Scl 70-positive patients develop pulmonary fibrosis [6]. Anti-centromere antibodies (ACA) (found in 20–30% of patients) and anti-Scl 70 antibodies are mutually exclusive and ACA is, by contrast, associated with lcSSc and a low prevalence of SSc-ILD but a higher prevalence of PAH [7]. It is worth noting that a large proportion of SSc patients (approximately 80%) and SSc-ILD patients (60%) [8] are anti-Scl 70 antibody negative.