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CRST Syndrome (Thibierge-Weissenbach Syndrome;
Calcinosis, Raynaud Phenomenon,
Sclerodactyly, and Telangiectasia)
General: Scleroderma variant; possible autosomal dominant transmission; resembles Rendu-Osler- Weber disease; well-established association with primary biliary cirrhosis; prevalent in females; average age of onset 45 years; reported only in whites.
Ocular: Conjunctival hyperemia; keratitis; profuse tear lysozyme; gritty, burning sensations; bilateral optic neuropathy.
Clinical: Dermal and subcutaneous calcinosis; Raynaud phenomenon; sclerodactyly; telangiectasia; calcinosis cutis; multiple intracranial aneurysms.
Boschi A, et al. Bilateral optic neuropathy associated with the crest variant of scleroderma. Eur J Ophthalmol 1993; 3:219-222.
Frayha RA, et al. Familial CRST syndrome with sicca complex. J Rheumatol
Ortiz JR, et al. CREST-associated multiple intracranial aneurysms and bilateral optic neuropathies. J Clin Neuro- Ophthalmol 1991; 11:233-240.
Thibierge G, Weissenbach RJ. Concretions Calcaires Souscutanees et Scleroderme. Ann Derm Syph (Paris) 129-l55.
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