Fact-sheet: Progressive systemic sclerosis - scleroderma
Updated on 09/17/2021 at 12:16 PM
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Definition
Systemic sclerosis is a systemic disease of unknown etiology,
predominantly affecting adults, combining cutaneous, osseous, and visceral manifestations, particularly pulmonary or digestive.
Cutaneous scleroderma is a sclerotic induration of the skin and may be isolated or part of systemic sclerosis.
CREST syndrome = Calcinosis + Raynaud's + Esophageal dysmotility (dyskinesia, achalasia) + Sclerodactyly + Telangiectasia.
Laboratory findings
Antinuclear antibodies (anti-topoisomerase 1 "anti-Scl 70", anti-centromere, anti-U1-RNP, anti-U3-RNP, anti-RNA polymerase).
Absence of hypereosinophilia
Radiography
Osseous abnormalities: acro-osteolysis, soft tissue calcifications, rare upper cervical involvement mimicking hydroxyapatite deposition disease or rheumatoid arthritis. Possible rib and mandibular resorption.
Cardiothoracic involvement: diffuse infiltrative lung disease of NSIP type, fibrosis, cancer. Possible pleural thickening.
Digestive (motility) involvement: pseudo-achalasia and esophageal dyskinesia, gastroparesis, megaduodenum, sacculations and pseudodiverticula of the small bowel and colon.
Ultrasound
Echocardiography-Doppler: screening for myocardial involvement.
CT
Thoracic involvement: pulmonary fibrosis, NSIP-type interstitial lung disease, pulmonary neoplasia (bronchioloalveolar). Possible signs of pericarditis and pleural thickening.
Digestive involvement: esophageal pseudo-achalasia presenting as a patulous esophagus, intestinal pseudo-obstruction (dilated fluid-filled small bowel loops). Exceptionally: calcifying pancreatitis, colonic telangiectasias.