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Choroidopathy of systemic lupus erythematosus

Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Harvard Medical School, Boston, MA, USA; Schepens Retina Foundation, Schepens Eye Research Institute, Department of Ophthalmology, Harvard Medical School, Boston, MA, USA

H S Uy

E K Akpek

S L Harper

D N Zacks

Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Harvard Medical School, Boston, MA, USA

C S Foster

Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA. Tel: (/1) 617 573 3968; Fax: (/1) 617 573 3181; E-mail: fosters{at}helix.mgh.harvard.edu

Purpose: To describe the ocular and systemic manifestations associated with systemic lupus erythematosus (SLE) choroidopathy.

Methods: Three new cases of choroidopathy in patients with active SLE were described. Twentyfive published cases of lupus choroidopathy were summarized.

Results: There have been 28 cases of lupus choroidopathy (47 involved eyes) that have been reported in the English literature since 1968, including the three current cases. Only two of the patients were male. The choroidopathy was bilateral in 19 patients (68%). All 28 patients (100%) had active systemic vascular disease at the onset of their choroidopathy; 18 (64%) had nephropathy and 10 (36%) had central nervous system (CNS) lupus vasculitis. All but one of the patients had a known diagnosis of SLE at the onset of choroidopathy. 30 of the 47 involved eyes had presenting visual acuity of 20=40 or better; 14 eyes showed improvement in visual acuity with therapy. 23 patients (82%) had resolution of their choroidopathy when their systemic disease was brought under control. Despite treatment, 4 of the 28 patients (14%) died from complications of SLE.

Conclusions: Although less known than retinopathy, lupus choroidopathy may be more common than generally appreciated. It usually serves as a sensitive indicator of lupus activity. The presence of SLE choroidopathy is generally indicative of coexistent (although sometimes occult) nephropathy, CNS vasculitis, and other SLE visceral lesions. Immunomodulation of the systemic disease can lead to improvement and resolution of the systemic vasculitis as well as the choroidopathy.

Key Words: choroidopathy • retinopathy • central nervous system (CNS) • nephropathy • systemic lupus erythematosus (SLE) • vasculitis • scleritis

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