Scleritis and sclerokeratitis associated with IgA vasculitis: A case series

Abstract

Purpose: To describe a case series of scleritis associated with IgA vasculitis (IgAV) at a tertiary referral center.

Observations: Three men with scleritis associated with IgAV were identified: one with anterior scleritis alone, one with anterior scleritis and peripheral ulcerative keratitis (sclerokeratitis), and one with anterior and posterior scleritis. Visual acuity was preserved except from the patient who developed posterior scleritis. Ocular pain was the main symptom at presentation. All patients had a previous history of palpable purpura, but only one was aware of his underlying IgAV. Laboratory results revealed microhematuria and proteinuria with normal urinary β₂ microglobulin levels and negative serum ANCAs. Skin or kidney biopsy demonstrated leukocytoclastic vasculitis or glomerulonephritis with dominant IgA immune deposits.

Conclusions and importance: Although uncommon, IgAV should be included in the differential diagnosis of anterior scleritis alone or associated with peripheral ulcerative keratitis or posterior scleritis, even in systemically asymptomatic patients. Urinalysis should not be underestimated in assessment of scleritis to detect early stages of glomerular disease. Scleritis may be the first manifestation whose study may lead to the diagnosis of IgAV. Multidisciplinary approach is necessary to prevent irreversible organ damage such as renal failure.

Keywords: Anterior scleritis; Chronic kidney disease; Complement activation; IgA deposits; IgA vasculitis; Immunofluorescence; Posterior scleritis; Proteinuria; Sclerokeratitis; Swept-source optical coherence tomography.