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Crst syndrome (thibierge-weissenbach syndrome; calcinosis, raynaud phenomenon, sclerodactyly, and telangiectasia) 


Crst syndrome (thibierge-weissenbach syndrome; calcinosis, raynaud phenomenon, sclerodactyly, and telangiectasia)                                                                                                            329

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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