China
Africa
Explore chapters and articles related to this topic
Respiratory
Published in Kristen Davies, Shadaba Ahmed, Core Conditions for Medical and Surgical Finals, 2020
Systemic sclerosis: Connective tissue disease characterised by hardened, sclerotic skin. Can occur with skin tightening alone (scleroderma) or with systemic involvement (limited or diffuse). Limited cutaneous systemic sclerosis is associated with Raynaud phenomenon, predominant face and limb involvement and positive anti-centromere antibodies. CREST syndrome (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) is a subtype of limited cutaneous systemic sclerosis. Diffuse cutaneous systemic sclerosis affects the trunk and proximal limbs predominantly, as well as the lungs and kidneys. Associated with positive scl-70 antibodies.
Pathogenesis
Published in Aparna Palit, Arun C. Inamadar, Systemic Sclerosis, 2019
Several population-based studies have established the association of ethnicity with development and severity of SSc. The disease occurrence is higher in Choctaw Native Americans and Blacks. There is a younger age of onset, higher frequency of diffuse skin involvement, pulmonary disease, and overall worse prognosis in Black individuals as compared to Whites. The Hispanic and Native Americans develop a more severe disease than Whites. Anti-centromere antibodies are commonly found in Whites, and anti-ribonucleoprotein and anti-fibrillarin antibodies are common in Blacks. Anti-Scl70 antibodies are found in equal frequency in all ethnic groups.5
Causes and Assessment of Dysphagia and Aspiration
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Scleroderma and ‘CREST’ syndrome (calcinosis, Raynaud’s, oesophageal involvement, sclerodactyly, telangiectasis) are progressive disorders of connective tissue. Smooth muscle cells are replaced by collagen fibres, resulting in fibrosis. Anti-nuclear antibodies and anti-centromere antibodies are raised in this condition. The lower oesophagus is often affected, resulting in poor peristalsis, severe GORD with stricture formation and Barrett’s oesophagus.
Recent innovations in the screening and diagnosis of systemic sclerosis-associated interstitial lung disease
Published in Expert Review of Clinical Immunology, 2023
Ashima Makol, Vivek Nagaraja, Chiemezie Amadi, Janelle Vu Pugashetti, Elaine Caoili, Dinesh Khanna
Autoantibodies represent the serologic hallmark of SSc, well-studied as diagnostic and prognostic markers of disease-related complications. They have also been shown to be associated with ILD in patients with known SSc. Among these, the autoantibody most often associated with the presence of ILD is ATA [34,63–66]. Additionally, anti Th/To ribonucleoprotein antibodies and anti PM/Scl have also been shown to be associated with ILD, though these antibodies are not as highly prevalent in SSc [67,68]. In two large multi-center cohorts, the Canadian Scleroderma Research Group registry and the German Network for Systemic Scleroderma Registry, the presence of Anti-SSA/Ro was found to be associated with at least a two-fold increased odds of ILD [69,70]. On the contrary, anti-centromere antibodies appear to be ‘relatively protective’ and associated with decreased likelihood of progressive ILD [34,71]. While these antibodies carry an association with the presence of ILD, they are not unique to lung-specific disease activity and also correlate with extrapulmonary SSc complications. Therefore, the use of blood-based biomarkers alone cannot replace gold-standard HRCT to evaluate for ILD. While we recommend that all patients with SSc be screened for ILD, there still exists some variation in practice pattern, with some clinicians and patients choosing to defer HRCT imaging. In these situations, elevated levels of these ILD-associated autoantibodies can be used as a risk stratification tool and when present should signal a higher risk of ILD in patients with SSc prompting HRCT acquisition.
Transferrin isoforms analysis by capillary electrophoresis in systemic lupus erythematosus and systemic sclerosis
Published in Scandinavian Journal of Clinical and Laboratory Investigation, 2020
Lech Chrostek, Ewa Gindzieńska-Sieśkiewicz, Ewa Gruszewska, Otylia Kowal-Bielecka, Bogdan Cylwik
The study was carried out on the sera of 81 patients (mean age: 46 years, range: 19–71) admitted to the Department of Rheumatology and Internal Diseases in University Hospital of Bialystok and included 38 patients with SLE (mean age: 38 years, range: 23–70) and 43 patients with SSc (mean age: 46 years, range: 19–71). The duration of SLE was from 1 to 21 years and SSc from 3 months to 15 years. The diagnosis of the disease was made on the basis of criteria given by the American College of Rheumatology [9,10]. In 23 patients with SSc was recognized as a limited form of SSc and 20 as having diffuse form of SSc. About 16 of 23 patients with limited SSc presented lung fibrosis (56%). All of the patients with limited form of SSc exhibited anti-centromere antibodies and 9 of them also anti-Ro-52. In nine patients with diffuse form of SSc were present anti-Scl-70 (anti-tropoisomerase I antibodies) and in one patients with limited form. All of the patients with SLE presented antinuclear antibodies (ANA test) and anti-double stranded DNA antibodies (anti-dsDNA test). The patients were taking non-steroidal anti-inflammatory drugs (NSAIDs) and disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate and sulfasalazine.
Association between Skin Thickness Measurements with Corneal Biomechanical Properties and Dry Eye Tests in Systemic Sclerosis
Published in Ocular Immunology and Inflammation, 2019
Ziya Ayhan, Mahmut Kaya, Taylan Ozturk, Gul Arikan, Merih Birlik
Scleroderma is an autoimmune progressive chronic disorder of unknown aetiology; it is characterised by tightening and thickening of the skin associated with widespread microangiopathy and multi-organ fibrosis.1–3 It can be classified as limited cutaneous scleroderma (lcSSc) or diffuse cutaneous scleroderma (dcSSc) according to extent of skin involvement shown to influence the patient’s daily functions and survival.4,5 In lcSSc, the fibrosis is mainly restricted to the hands, arms and face, and Raynaud’s phenomenon is present for several years before fibrosis appears; pulmonary hypertension is frequent, and anti-centromere antibodies occur in 50–90% of those patients. Diffuse cutaneous scleroderma is a rapidly progressing disorder that affects large areas of the skin and compromises internal organs. Progressive fibrosis in the skin and lungs accounts for the significant morbidity and mortality of patients with SSc.6