April 2014
Volume 55, Issue 13
ARVO Annual Meeting Abstract  |   April 2014
The value of rescreening in primary retinal vasculitis.
Author Affiliations & Notes
  • Mirjam EJ Van Velthoven
    Ophthalmology, Rotterdam Eye Hospital, Rotterdam, Netherlands
  • Seerp Baarsma
    Ophthalmology, Rotterdam Eye Hospital, Rotterdam, Netherlands
  • Petrus M Van Hagen
    Department of Clinical Immunology, Erasmus Medical Center, Rotterdam, Netherlands
  • Jan A van Laar
    Department of Clinical Immunology, Erasmus Medical Center, Rotterdam, Netherlands
  • Tom Missotten
    Ophthalmology, Rotterdam Eye Hospital, Rotterdam, Netherlands
  • Footnotes
    Commercial Relationships Mirjam Van Velthoven, None; Seerp Baarsma, None; Petrus Van Hagen, None; Jan van Laar, None; Tom Missotten, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science April 2014, Vol.55, 5793. doi:
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      Mirjam EJ Van Velthoven, Seerp Baarsma, Petrus M Van Hagen, Jan A van Laar, Tom Missotten; The value of rescreening in primary retinal vasculitis.. Invest. Ophthalmol. Vis. Sci. 2014;55(13):5793.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose: Primary retinal vasculitis (PRV), defined as a retinal vasculitis without systemic disease associations or infection, is an ophthalmic disease entity since 1880 (reported by Eales). As some of the underlying systemic vasculitis entities can be life threatening, it is questionable if PRV can be accepted as a diagnosis, or continued reinvestigation is warranted.

Methods: Retrospective case series of 179 uveitis patients with a follow-up of > 1 year, visiting a tertiary uveitis clinic during 2010, in whom vasculitis was the dominant feature of their uveitis. Files were analyzed for date and diagnosis of (non)infectious or systemic diseases associated with retinal vasculitis.

Results: After initial routine screening, 72 patients had been labelled PRV. By 2010, mean follow-up was 7.3 years (range 1-27). Within 5 year of follow-up, 54% of the patients were diagnosed with a specific disease known to be associated with retinal vasculitis (such as Behçet’s disease and sarcoidosis). In patients with longer follow-up, an additional 23% of patients were re-diagnosed with a specific label by year 10, another 14% of patients obtained a specific diagnosis after more than 10 years of follow-up. At least 91% of patients with PRV were re-labelled during their follow-up.

Conclusions: Most or perhaps all patients initially labelled as PRV will get an systemic (non)infectious inflammatory diagnosis, known to be linked with retinal vasculitis. Of course, patients could have multiple autoimmune diseases and can get treated with new agents during their follow-up. Nevertheless, apart from intensive initial screening, repeated rescreening seems to be indicated in patients with retinal vasculitis, to rule out severe systemic diseases. The term retinal vasculitis "of unknown origin" is certainly not as eloquent but in our opinion more transparent than primary retinal vasculitis.

Keywords: 746 uveitis-clinical/animal model • 461 clinical (human) or epidemiologic studies: natural history • 745 uvea  

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