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Pallor, cyanosis, and erythema of the hands
7%
18/255
Calcium deposits on digits
11%
27/255
Blanching vascular abnormalities
6%
16/255
Hypercoagulable state
54%
137/255
Heartburn and regurgitation
18%
45/255
Select Answer to see Preferred Response
Anti-centromere antibodies are specific for limited systemic scleroderma (CREST). They are rarely found in other autoimmune disorders. Hypercoagulable state is not a feature of CREST, though it is a feature of autoimmune diseases such as anti-phospholipid antibody syndrome. Systemic sclerosis is an idiopathic connective tissue disease characterized by microvascular damage and overactive collagen deposition in skin and internal organs, with several subsets. CREST syndrome is a variant of limited cutaneous systemic sclerosis that includes Calcinosis of digits; Raynaud phenomenon; Esophageal dysfunction; Sclerodactyly; and Telangectasias. Anti-centromere antibodies are particularly associated with limited cutaneous sclerosis (including CREST). Anti-DNA-topoiomerase I(anti-Scl-70) antibodies are associated with diffuse sclerosis. Hinchcliff et al. describe the typical patient workup, to include bloodwork, pulmonary function tests, echocardiogram, and chest CT in order to establish the diagnosis and extent of visceral involvement. Spencer-Green et al. discuss the test characteristics of both anti-centromere antibodies and anti-DNA topoisomerase (anti-Scl-70) antibodies and conclude that both are highly specific for limited subsets of scleroderma but neither is sensitive enough to function as a screening test since 40% of systemic sclerosis patients will have neither antibody present. Illustration A shows an example of calcinosis of digits, specifically calcinosis cutis. Illustration B shows the pallor, cyanosis, and erythema of Raynaud phenomenon. Illustration C shows sclerodactyly. Illustration D shows telangiectasias distributed across the upper lip. Incorrect Answers: Answer 1: Pallor, cyanosis, and erythema of the hands describes Raynaud phenomenon, a component of CREST. Answer 2: Calcium deposits on the digits and extensor tendons are a feature of CREST. Answer 3: This describes telangiectasias found in CREST. Answer 5: Heartburn and regurgitation are symptoms of esophageal dysfunction in CREST.
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