Bilateral interstitial keratitis as the presenting manifestation of systemic lupus erythematosus in a child

Abstract

Bilateral interstitial keratitis as the presenting manifestation of systemic lupus erythematosus in a child Q1 X X Interstitial keratitis (IK) is defined as a nonsuppurative inflammation of the corneal stroma with associated cellular infiltration and vascularization commonly caused by infections or rheumatologic conditions. Although extremely rare, IK may be seen with systemic lupus erythematous (SLE). We present the first case of a child without known history or symptomatology of rheumatologic or infectious disease presenting with bilateral IK that ultimately led to a diagnosis of SLE. A 9-year-old African American girl with a medical history of asthma and no known rheumatologic or infectious disorders was referred for management of extensive bilateral corneal scarring and vascularization, mild eye pain, and photophobia. Her condition had been examined by multiple ophthalmologists Q2 X X since age 7 years when her mother noted that her eyes were becoming "blue and cloudy." She was inconsistently taking prednisolone acetate 1% 2 to 3 times per day in both eyes (OU). Review of symptoms was negative for fevers, rashes, skin lesions, arthralgias, myalgias, intestinal symptoms, oral or nasal ulcerations, foreign travel, and animal or insect bites. Uncorrected visual acuity was 20/25 right eye (OD) and 20/50 (pinhole 20/30) left eye (OS). Pupils were equal and symmetric without a relative afferent pupillary defect. Extraocular movements were full, and her intraocular pressures were 14 mm Hg OU. External examination was unremarkable. On slit-lamp examination, the conjunctiva was white and quiet without evidence of follicles, papillae, or granulomas. There was significant bilateral superficial and deep peripheral corneal stromal opacification with large vascular loops deep within the corneal stroma. The opacification extended from the peripheral cornea centrally for virtually 360 degrees, with involvement of the superior visual axis. No corneal epithelial or endothelial disease was noted. Anterior chambers were deep and quiet OU. The lenses appeared clear. Dilated examination was within normal limits. Her basic metabolic panel, liver function tests, sedimentation rate, thyroid studies, Lyme titer, tuberculin test, rapid plasma reagin, fluorescent treponemal antibody absorption, lipid panel, angiotensin converting enzyme, rheumatoid factor, anti-neutrophil cytoplasmic antibody (p and c-ANCA), hepatitis A and C antibodies, myeloperoxidase antibody, and proteinase 3 antibody were all within normal limits. Hepatitis B surface antibody was positive (patient was immunized), with the rest of the hepatitis B panel negative. EpsteinÀBarr virus IgG

Corneal stromal deposits in connective tissue disease, a case series

“… 9 Recently, Abbas et al reported a case of bilateral interstitial keratitis in a 9-year-old girl with SLE. 7 The lesions in our cases were not associated with PUK or other ocular surface or intraocular inflammation.…”

“… 5 There have been reports of acute corneal stromal infiltration and edema in systemic lupus erythematosus (SLE) patients and diffuse stromal opacification in patients with ATS, but there are no reports on peripheral corneal deposits with neovascularization, similar to that seen in Terrien's marginal degeneration (TMD). 2 , 6 , 7 , 8 , 9 , 10 In this observational case series, we report two cases of refractile, peripheral, corneal stromal deposition in patients with ATS and EDS, two closely related CTDs.…”