Abstract 99. Jahrestagung der DOG, 29. 9. - 2. 10. 01 im ICC, Berlin

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Ocular manifestations in Wegener granulomatosis - a prospective study

1Nölle B., 1Schwarzer A. L., 2Reinhold-Keller E., 2Gross W. L.

1Klinik für Ophthalmologie, Hegewischstr. 2, 24105 Kiel; 2Rheumaklinik Bad Bramstedt und Poliklinik für klinische Rheumatologie der MUL Lübeck, Oskar-Alexander-Str. 26, 24576 Bad Bramstedt

Objective: Wegener granulomatosis (WG) is a frequent primary systemic vasculitis. WG is highly associated with anti-neutrophil cytoplasmatic autoantibodies (ANCA) with specificity for PR-3. Eye manifestations in WG may appear up to 50% in retrospective studies, however, a prospective controlled study is not yet available.
Methods: Between 1.1.1996 and 31.12.1998 124 WG-patients (101 histologically proven) were investigated by ophthalmologists with slit lamp biomicroscopy of anterior and posterior eye segments, analysis of visual acuity, sicca-symptoms, and visual fields using a standardized protocol (mean 9x, min. 1x, max. 30x). ANCA was tested in serum, an interdisciplinary clinical approach was performed including MRI of the orbit.
Results: Some ocular inflammatory manifestions of WG were seen (ocular involvement one side/bilateral): sicca-syndrome (2/79), episcleritis (25/24), scleritis (11/5), keratitis (11/3), cataracta complicata (10/30), orbital granuloma (3/5), visual fields were normal in only 20%, defects were graded into low, medium, and heavy, each caused one third. The various ocular manifestations of WG were not correlated with the presence of ANCA (105 WG ANCA+, 19 WG ANCA-), however, episcleritis was associated with active disease (p<0.05). Initial eye symptoms due to WG were seen in 6.4% of the patients.
Conclusion: In WG vasculitic eye manifestations are more frequent than granuloma formation. In addition, side effects of drug therapy have to be recognized. No ocular subtype with ANCA association was found. Although diagnosis of WG is improved by ANCA testing, and optimization of treatment is available, WG can even today lead to blindness or loss of one eye.




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