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Progressive systemic sclerosis (scleroderma; systemic scleroderma)


Progressive Systemic Sclerosis (Scleroderma; Systemic Scleroderma)

General: Chronic connective tissue disease of unknown etiology; chronic and usually progressive 858h79i disorder; typical onset is in third to fifth decade; ratio of women to men is 4:l; primary sites of pathology are the arterioles and capillaries of affected organs.

Ocular: Marginal corneal ulcers; shortened fornices of the conjunctiva; ptosis; cotton-wool patches of retina; papilledema; retinal hemorrhages; cicatrization of conjunctiva and cornea; blepharitis; blepharospasm; thready, tenacious yellow-white conjunctival discharge; hypertrophy of lacrimal gland; episcleritis; ocular myositis; Sjgren syndrome; uveitis; vitreal haze; keratitis sicca; decreased corneal sensation; iritis; ischemic choroidopathy; iris sectorial atrophy; blepharophimosis; heterochromia; keratoconus; central retinal vein occlusion; branch retinal vein occlusion.

Clinical:

Vascular insufficiency; Raynaud phenomenon; malaise; weight loss; stiffness; fever; polyarticular arthritis; diffuse edema of the hands; calcinosis; esophageal involvement; sclerodactyly; telangiectasis; esophageal stricture; renal failure; diffuse interstitial fibrosis.

Collins JF. Handbook of Clinical Ophthalmology. New York: Masson, 1982.

David R, Irvy M. Focal chorioretinitis and iridocyclitis associated with scleroderma. Ann Ophthalmol 1976; 8: 199-202.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Reddy CV, Foster CS. Scleroderma. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. V. Philadelphia: WB Saunders, 1994:2919-2924.

Saari KM et al. Bilateral central retinal vein occlusion in a patient with scleroderma. Ophthalmologica 1981; 182: 7-l2.




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