CREST syndrome is an autoimmune condition which is associated with atrophy and fibrosis of the oesophageal musculature resulting in dysphagia and reflux-type symptoms. Which of the following is not a feature of CREST syndrome?
Correct Answer: Erythematous malar rash
Description: The scleroderma spectrum of disorders are a group of connective tissue diseases inclusive of localized scleroderma (affects skin only), Raynaud's (vasospastic involvement of the fingers) and systemic sclerosis which is itself divided into diffuse cutaneous systemic sclerosis (DCSS) and limited cutaneous systemic sclerosis (also known as CREST syndrome). The two conditions vary in their symptom pattern and onset, but both involve the internal organ systems, including the renal tract and lungs (causing failure and pulmonary fibrosis). Skin involvement in DCSS is severe and widespread, but organ involvement is maximal at around 3 years and then typically improves. In CREST skin involvement is confined to the face and extremities; however, organ involvement tends to be progressive and more severe. CREST syndrome is typified by the following features: Calcinosis, Raynaud's, esophageal disorders, sclerodactyly and telangiectasia. Diagnosis is clinical and backed by identification of ceain auto-antibodies. Anti-nuclear antibodies are usually present, anti-Scl-70 is positive in 40% of scleroderma, the presence of anti-centromere antibody occurs in 80%-90% of CREST and is suggestive of the diagnosis. However, auto-antibody testing is non-specific, and either antibody or both antibodies may occur in both conditions.
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