Article Text

Download PDFPDF

  1. J. Klotsche1,
  2. C. Sengler1,
  3. F. Dressler2,
  4. D. Foell3,
  5. I. Foeldvari4,
  6. J. P. Haas5,
  7. G. Horneff6,
  8. T. Hospach7,
  9. T. Kallinich8,
  10. I. Liedmann1,
  11. K. Moenkemoeller9,
  12. M. Niewerth1,
  13. F. Weller-Heinemann10,
  14. D. Windschall11,
  15. A. Heiligenhaus12,
  16. K. Minden1,
  17. K. Baquet-Walscheid12
  1. 1German Rheumatism Research Center Berlin, Epidemiology, Berlin, Germany
  2. 2Medizinische Hochschule Hannover, Paediatric Rheumatology, Hannover, Germany
  3. 3University Hospital Münster, Department of Paediatric Rheumatology and Immunology, Münster, Germany
  4. 4Hamburg Centre for Pediatric and Adolescence Rheumatology, Paediatric Rheumatology, Hamburg, Germany
  5. 5German Centre for Child and Adolescent Rheumatology, Paediatric Rheumatology, Garmisch-Partenkirchen, Germany
  6. 6Asklepios Kinderklinik St. Augustin, Centre for General Paediatrics and Neonatology, St. Augustin, Germany
  7. 7Olgahospital Stuttgart, Center for Pediatric Rheumatology, Stuttgart, Germany
  8. 8Charité - Universitätsmedizin Berlin, Department of Paediatric Respiratory Medicine, Immunology and Intensive Care Medicine, Berlin, Germany
  9. 9Kliniken Köln - Kinderkrankenhaus, Paediatric Rheumatology, Köln, Germany
  10. 10Klinikum Bremen-Mitte, Prof-Hess-Kinderklinik, Paediatric Rheumatology, Bremen, Germany
  11. 11St. Josef-Stift Sendenhorst, Clinic for Paediatric and Adolescent Rheumatology, Sendenhort, Germany
  12. 12Franziskus Hospital, Department of Ophthalmology, Münster, Germany


Background Uveitis is an extra-articular manifestation of Juvenile idiopathic arthritis (JIA) with a prevalence of up to 20% developing most frequently in young girls and patients positive for antinuclear antibodies (ANA). Untreated and uncontrolled uveitis may lead to vision-threatening complications and even blindness.

Objectives The main objectives of the analyses were to determine the visual prognosis, uveitis complications and necessity of ocular surgery during the first five years of ocular disease. The likelihood of achieving an inflammation-free phase or even a remission without medication were investigated.

Methods The Inception Cohort of Newly diagnosed patients with JIA (ICON) was initiated in 2010 in order to prospectively follow JIA patients up to 10 years after JIA disease onset. 953 Patients were assessed at enrollment, three-monthly during the first year, and six-monthly afterwards by a standardized physician’s and patient’s case report form including clinical parameters, treatment data and several laboratory parameters such as ESR, CRP or S100A12. Patients who developed uveitis underwent a regular ophthalmological assessment. The treating ophthalmologist three-monthly completed an additional questionnaire, documenting the anterior chamber (AC) cell grade, current uveitis activity (UA) and UA during the previous three months, best corrected visual acuity (BCVA), uveitis-related complications, previous ocular surgery, current topical treatment and clinical course of uveitis and additional parameters. Inactive uveitis was defined by AC cell grade of 0, quiescence of uveitis by inactive uveitis for at least 6 months, and remission by inactive uveitis for at least 6 months without topical steroids or systemic anti-inflammatory medication (steroids or DMARDs).

Results A total of 133 children developed uveitis in the JIA disease course, of which 97 patients were documented via the ophthalmological questionnaire for at least two years resulting in a mean follow-up of 5.8 years (SD 1.8). 76% were female, 86% ANA positive, 70% oligoarthritis, and 22% rheumatoid factor negative polyarthritis and mean age at JIA onset was 3.1 (SD 2.1) and uveitis onset at 4.4 (SD 2.2) years. The mean duration between JIA onset and uveitis onset was 15.7 (SD 15.6) months. At least one ocular complication was reported for 24% of patients at first uveitis documentation and 47% of patients had at least one ocular complication until the five year follow-up. Among those, posterior synechiae (31%) and cataract (27%) were the most frequent, followed by an increased IOP (12%) with or without glaucomatous changes. Ocular surgery was rarely necessary, and visual acuity remained quite good in the majority of patients: After five years, >90% had BCVA of <0.4 LogMAR (Logarithm of the Minimum Angle of Resolution), and 63.5% even of <0.1 LogMAR. About half of the uveitis patients were already treated with DMARDs at uveitis onset. The rate of treatment with biological DMARDs increased from 10% at first uveitis documentation up to 20% at 5-year follow-up. Three in four patients were treated with topical steroids at first assessment, whereas this proportion decreased to 43%. 80 of 97 patients (83%) achieved uveitis quiescence during the first five years of disease, with more than 50% experiencing more than one episode (mean 1.5 episodes (SD 1.0)) during this time period. The mean duration of uveitis quiescence was 23.2 (SD 15.6) months. A total of 39 (40%) patients achieved uveitis remission during follow-up. The likelihood of remission was associated with a lower JIA disease activity (cJADAS10), lower erythrocyte sedimentation rate (ESR) and a higher age at JIA disease onset.

Conclusion The rate of ocular complications is already remarkable at uveitis diagnosis, and increases during uveitis disease course despite anti-inflammatory treatment. However, the visual acuity frequently remains unaffected, and the majority of patients achieve uveitis quiescence and even 40% uveitis remission within 5 years of follow-up.

Acknowledgements The ICON study is funded by a research grant of the Federal ministry of education and research (BMBF, FKZ 01ER0812, FKZ 01ER1504A-C)

Disclosure of Interests Jens Klotsche: None declared, Claudia Sengler: None declared, Frank Dressler: None declared, Dirk Foell: None declared, Ivan Foeldvari: None declared, Johannes-Peter Haas: None declared, Gerd Horneff Speakers bureau: Pfizer, Novartis, Janssen, Chugai, Abbvie, Grant/research support from: Pfizer, Novartis, MSD, Chugai, Roche, Abbvie, Toni Hospach Consultant of: SOBI, Novartis, Tilmann Kallinich: None declared, Ina Liedmann: None declared, Kirsten Moenkemoeller: None declared, Martina Niewerth: None declared, Frank Weller-Heinemann: None declared, Daniel Windschall: None declared, Arnd Heiligenhaus: None declared, Kirsten Minden: None declared, Karoline Baquet-Walscheid: None declared

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.