Extended matching item (EMI)
Tristan Barrett, Nadeem Shaida, Ashley Shaw, Adrian K. Dixon in Radiology for Undergraduate Finals and Foundation Years, 2018
Match the clinical scenarios / questions below to the above list options. A patient with known osteoarthritis complains of hard, painful swellings over a number of distal interphalangeal joints. Subsequent X-rays confirm bony outgrowths in these regions. What is the eponymous name of this finding?A young patient suspected of having rheumatoid arthritis is found on examination to have a salmon-pink rash on her upper left arm, hepatosplenomegaly and lymphadenopathy. What is the likely unifying diagnosis?A 25-year-old man presents with lower back pain and episodes of shortness of breath. A CXR shows mild biapical fibrosis, an X-ray of the lumboscaral spine shows bilateral sacroiliitis, with fusion of the lower lumbar vertebrae. Which major histocompatibility complex is most likely to be associated?A patient presents systemically unwell. History reveals difficulty in swallowing and a history of Raynaud’s phenomenon. On examination there is telangiectasisa around her mouth. An auto-antibody screen is positive for anti-centromere antibodies only. Hand X-rays are requested; list the two features that are most likely to be present.
Respiratory
Kristen Davies, Shadaba Ahmed in 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.
Causes and Assessment of Dysphagia and Aspiration
John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford in 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.
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
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.
Related Knowledge Centers
- Antinuclear Antibody
- Autoantibody
- Centromere
- Crest Syndrome
- Immunofluorescence
- Kinetochore
- Primary Biliary Cholangitis
- Pulmonary Fibrosis
- Scleroderma
- Autoimmune Disease