Purpose:
Multifocal Choroiditis and Panuveitis syndrome (MFCPU) is frequently associated to profound visual impairment due to multifaceted clinical complications. The condition is rare and litterature on its etiology/pathogenesis is sparse and still unclear. Our study aims to investigate the clinical characteristics, evolution and immunogenetic correlations of MFCPU.
Methods:
Retrospective cohort study with control group. Sixty patients (120 eyes) with MFCPU were evaluated from January 2002 through January 2010 at a single center academic hospital. Demographics and clinical data were collected. Every patient was typed for HLA class I and class II antigens using the standard microlymphotoxicity test and polymerase chain reaction with sequence-specific primers.
Results:
Among affected patients, the mean duration of symptoms was 54 months and mean visual acuity was 20/500 at initial presentation. A majority of women was included (sex ratio 5:1). Choroidal neovascularisation, chronic cystoid macular edema and epiretinal fibrosis were the leading causes of visual loss at presentation or throughout the evolution of the disease. Immunogenetically significant increased frequencies of HLA A31 and HLA DR4 were found. HLA DR3 and HLA DR7 were increased but did not reach statistical significance. None of the represented allele were correlated to worse evolution neither to better response to immunosuppressive or immunomodulative treatments.
Conclusions:
This study supports the hypothesis of immunogenetic correlation derived from other well described HLA-type related uveitis syndromes. MFCPU is still open clinically and immunogenetically for debate. Further large multicenter studies with DNA typing could help clarify the origin and treatment of MFCPU.
Keywords: retina • retinochoroiditis • inflammation