ABSTRACT
BACKGROUND: Multiple evanescent white dot syndrome (MEWDS) is an acquired chorioretinal disorder of unknown etiology. We investigated the possibility that MEWDS might be related to a specific HLA subtyping. METHODS/RESULTS: Blood was obtained from nine patients affected by MEWDS. HLA-B51 was found in four of these nine patients with MEWDS. CONCLUSION: There was a 3.7-fold increased frequency of HLA-B51 in patients affected by MEWDS (relative risk 5.86). MEWDS might then be related to the presence of a specific HLA subtype, HLA-B51. However, due to the small sample size, our results need to be confirmed by further testing.
Subject(s)
HLA Antigens/classification , Retinal Diseases/immunology , Vision Disorders/immunology , HLA-B Antigens/analysis , HLA-B51 Antigen , Humans , Reference Values , Retina/pathology , Retinal Diseases/pathology , SyndromeABSTRACT
PURPOSE: Multiple evanescent white dot syndrome (MEWDS) is a benign acquired chorioretinal disorder occurring mostly in young adults. Its pathophysiology is unknown. To describe the results of indocyanine green angiography (ICGA) in MEWDS. PATIENTS AND METHODS: Four patients with MEWDS were investigated by ICGA. RESULTS: In all cases, ICGA revealed numerous choroidal hypofluorescent lesions that largely outnumbered the lesions visible with either fundoscopy or fluorescein angiography. Three cases showed a blind spot enlargement on perimetry associated with the presence of a large peripapillary hypofluorescent zone on ICGA. Three cases showed macular granularity on fundoscopy correlating with a significant subfoveal hypfluorescent lesion on ICGA. Evolution was always favorable with disappearance of the hypofluorescent choroidal lesions. CONCLUSIONS: Our results confirm that MEWDS is primarily a choroidal disorder. The blind spot enlargement and the macular granularity, frequently detected in MEWDS, result from larger peripapillary and subfoveal choroidal lesions.
Subject(s)
Choroid Diseases/diagnosis , Contrast Media , Fluorescein Angiography , Indocyanine Green , Retinal Diseases/diagnosis , Adult , Female , Follow-Up Studies , Humans , Male , Syndrome , Visual Fields/physiologyABSTRACT
BACKGROUND: Multiple evanescent white dot syndrome (MEWDS) is a benign acquired isolated chorioretinal disorder. Symptoms include photopsia, visual blur and scotomas. Ocular examination reveals multiple white dots at the level of the deep retina. A parainfectious disorder was suggested but the exact mechanism of MEWDS is still unknown. Postulating that MEWDS might be an antigen driven inflammatory reaction, we analyzed HLA subtypes in patients with MEWDS. PATIENTS AND METHODS: Sixteen patients were diagnosed with MEWDS in Lausanne from 1985 to 1994. Blood was withdrawn in 9/16 patients. HLA-A, -B and -DR were sought. RESULTS: HLA-B51 was detected in 4/9 patients (44.4%). Other HLA subtypes were detected sporadically. CONCLUSIONS: The frequency of HLA-B51 haplotype was found to be 3.7 times more elevated than in a normal control caucasian group. This suggests the possibility that MEWDS might be a genetically determined disorder as it is the case for other ocular diseases like Birdshot chorioretinopathy (HLA-A29), Harada's disease (HLA-DRMT3), acute anterior uveitis (HLA-B27) or Behçet's disease (HLA-B51). We have no explanation for the presence of HLA-B51 in both Behçet's disease and MEWDS. The association of HLA-B51 and MEWDS needs confirmation by further testing.