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Scleritis and sclerokeratitis associated with IgA vasculitis: A case series

  • Hernanz, I.1
  • Larque, A.B.2
  • Quintana, L.F.3, 4, 5
  • Espigol-Frigole, G.6, 4
  • Espinosa, G.6, 4, 5
  • Adan, A.1, 4, 5
  • Sainz-de-la-Maza, M.1, 4
  • 1 Clinical Institute of Ophthalmology, Hospital Clinic of Barcelona, Barcelona, Spain
  • 2 Department of Pathology, Hospital Clinic of Barcelona, Barcelona, Spain
  • 3 Department of Nephrology and Renal Transplantation, Centro de Referencia en Enfermedad Glomerular Compleja del Sistema Nacional de Salud (CSUR), Hospital Clinic of Barcelona, Barcelona, Spain
  • 4 August Pi i Sunyer Biomedical Research Institute (IDIBAPS), Barcelona, Spain
  • 5 Department of Medicine, University of Barcelona, Barcelona, Spain
  • 6 Department of Autoimmune Diseases, Hospital Clinic of Barcelona, Barcelona, Spain
Published Article
American Journal of Ophthalmology Case Reports
Publication Date
Apr 22, 2021
DOI: 10.1016/j.ajoc.2021.101100
PMID: 33997470
PMCID: PMC8093897
PubMed Central
  • Case Report


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.

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