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Mohamed El Hamdaoui, Jean-Luc Charuel, Remi Guillevin, Lucile Musset, Valérie Touitou, Phuc Le Hoang, Coralie Bloch-Queyrat, Bahram Bodaghi; Atypical ANCA-Associated Inflammatory Ophthalmic Diseases : A New Clinical Entity?. Invest. Ophthalmol. Vis. Sci. 2012;53(14):4264.
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The search for antineutrophil cytoplasmic antibodies (ANCA) is classical in the etiological investigation of uveitis. These antibodies guide the search for a systemic vasculitis when their antigen specificity is of anti-proteinase 3 type or anti-myeloperoxidase type. However, the atypical ANCA have not obvious antigenic specificity and their association with one or more disease remains uncertain in term of specificity. These antibodies can be either P or C by indirect immunofluorescence.We distinguish the atypical p and c-ANCA (with respectively perinuclear and cytoplasmic aspect), and the "atypical perinuclear aspects" associated with the specificity of the nuclear membrane of neutrophils.The aim of this study is to describe the clinical and paraclinical characteristics of inflammatory ophthalmologic diseases associated with atypical ANCA.
Retrospective and descriptive study of patients managed for an inflammatory ophthalmic disease between 2003 and 2011 by uveitis and internal medicine units at Pitié-Salpétrière Hospital (Paris, France). Only patients with atypical ANCA were included.
25 patients were included. 92% of patients show signs of intermediate uveitis (isolated or associated with signs of anterior uveitis). The association between intermediate uveitis and positive serology for atypical ANCA is statistically significant (p=0.004, Chi-square test).68% of patients presented with clinical and/or angiographic signs of peripheric retinal vascularitis. The other inflammatory diseases associated with atypical ANCA were: scleritis and optic neuritis.Macular edema and retinal ischemia were present in 52 % and 12% of cases, respectively. Half of the patients had a final visual acuity of 20/40.A CNS MRI was preformed in 15 cases. White-matter lesions compatible with multiple sclerosis were identified in 5 cases and CNS vasculitis was noted in 2 cases.
The prognosis and clinical features are homogeneous in atypical ANCA seropositive patient. However in most cases, severe intermediate uveitis is the main ocular manifestation. The association between antibodies and demyelinating diseases or/and CNS vascularitis is a major finding. A prospective study needs to be performed before any further conclusions.
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