Corneal vortex keratopathy in childhood-onset systemic lupus erythematosus (c-SLE)

  • Luciana Paim-Marques
  • Paula Carneiro
  • Islane Castro Verçosa
  • Simone AppenzellerEmail author
Original Article



To describe the prevalence and risk factors associated with corneal vortex keratopathy in a childhood-onset systemic lupus erythematosus (c-SLE) cohort.

Material and methods

Consecutive outpatients with c-SLE were evaluated by a pediatric ophthalmologist and pediatric rheumatologist in an outpatient clinic setting in an urban Children’s Hospital. Demographic, clinical, laboratory, and disease characteristics were documented for each patient. Cumulative drug dosage, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and Systemic Lupus Erythematosus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR-DI) scores were calculated.


A total of 76 c-SLE patients (61 (80.26%) females; mean age = 17.9 (SD ± 3.07)) were included. Ophthalmologic abnormalities were observed in 36 (47.36%) patients of which 16 (21.10%) had corneal vortex keratopathy (p = 0.03). c-SLE patients with corneal vortex keratopathy were all female. We did not observe any additional clinical, laboratory, or treatment feature associated with corneal vortex keratopathy.


We observed a high prevalence of corneal vortex keratopathy in female c-SLE. We hypothesize that this finding may be an initial, dose-related toxicity due to antimalarial use. Follow-up studies are necessary to determine if these changes are an early predictor of retinal toxicity due to antimalarial in c-SLE.

Key Points

• Corneal vortex keratopathy was frequently observed in female patients with c-SLE on a chloroquine medication.

• Corneal vortex keratopathy may be an early marker of chloroquine retinopathy.


Corneal vortex keratopathy Ocular findings Systemic lupus erythematosus 



Simone Appenzeller received grants from Fundação Apoio a Pesquisa Estado São Paulo-Brasil (Conselho Nacional Pesquisa Desenvolvimento-Brasil CNPq 304255/2015-7 and 473328/2013-5 and 401477/2016-9), Coordenação de Aperfeiçoamento de Pessoal de Nível Superior - Brasil (CAPES) Finance Code 001).

Compliance with ethical standards




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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Medical Physiopathology Program School of Medical ScienceUniversity of CampinasCampinasBrazil
  2. 2.Pediatric Rheumatology UnitAlbert Sabin Children’s HospitalFortalezaBrazil
  3. 3.Pediatric Immunology, Rheumatology Unit; Department of Pediatrics/College of MedicineUniversity of FloridaGainesvilleUSA
  4. 4.CAVIVER- NGO ClinicFortalezaBrazil
  5. 5.Ophthalmology UnitGeneral Hospital of FortalezaFortalezaBrazil
  6. 6.Autoimmune Laboratory- School of Medical ScienceUniversity of CampinasCampinasBrazil
  7. 7.Rheumatology Unit-Department of Medicine- School of Medical ScienceUniversity of CampinasCampinasBrazil

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