Abstract

SO.20.02

Systemic immunosuppression in peripheral multifocal chorioretinitis

Bernhard Nölle, Johann Roider
Klinik für Ophthalmologie, Universitätsklinikum Schlewig-Holstein, Campus Kiel, Kiel

Background
Peripheral multifocal chorioretinitis (PMC) is a clinically defined uveitis with suggested autoimmune pathogenesis. Only a few reports give informations on benefits of systemic immunosuppressive therapy, but guidelines for treatment are not available. The effect of systemic immunsuppression was analyzed in a prospective single center observation study.
Patients and methods
Consecutive clinical analysis of 20 PMC-patients, immunophenotyping of vitreous cells (6 patients), analysis of serum autoantibodies to nuclear antigens (ANA), neutrophil cytoplasmic antigens (ANCA), retinal antigens, and HLA-typing were performed. Systemic and local prednisolone was applied in 7 patients (control group), the other received methotrexate (Mtx) or mycofenolate mofetil (MMF) in addition (study group).
Results
Peripheral multifocal chorioretinitis shows a chronic inflammatory vitritis, associated chronic anterior uveitis, a predilection to women (17/20), bilateral involvement (19/20), high age (69.9 years), a long period between symptoms and diagnosis (8 months). Characteristics of vitreous cells were small T-lymphocytes expressing CD2, CD3, CD4 or CD8; about 36% of vitreous cells expressed CD68, small amounts of B-cells and markers of cell activation (IL2-receptor, transferrin receptor) were seen. 11 of 20 patients showed antinuclear antibodies, 3 of them with high titers. None of the patients have ANCA, antiretinal antibodies were seen in 8 patients without a specific pattern. In HLA-typing there was significant reduction of the antigens HLA-B7, DR1 and DR15. Other antigens were seen frequently, but not significantly elevated (HLA-DR 4, -7, -8). Systemic immunosuppression with Mtx or MMF (n=13) shows more improvement than systemic corticosteroids (n=7) with respect to visual acuity and intraocular inflammatory cells. Withdrawal of medication was followed by a disease relapse in two of three patients.
Conclusions
Peripheral multifocal chorioretinitis is another candidate for a human autoimmune uveitis. In this disease we find aspects of a genetic predisposition (HLA-typing results), an elevated frequency of autoantibodies, beneficial effects of systemic immunosuppression and a predilection for women. It seems reasonable to further investigate this rare disorder.

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