Large Inflammatory Pericardial Effusion and Macrocytic Anemia in a Vogt-Koyanagi-Harada Patient

Elizabeth Onyekachi Ugoani,

Abstract

Vogt-Koyanagi-Harada (VKH) disease is a multisystemic autoimmune disease which targets pigmented tissues, especially the eyes, in a genetically susceptible individual (HLA-DR4 carrier). It is characterized by a bilateral, visionthreatening uveitis that evolves in 4 phases and by extraocular manifestations in other tissues containing melanin. We report an association of complete VKH disease, vitamin B-deficient macrocytic anemia and a large, circumferential pericardial effusion in an 82-year old woman. Although there is no known report, to the best of our knowledge, of cardiac manifestations in VKH disease, we argue that the inflammatory pericardial effusion we found is a cardiac manifestation of VKH disease since this disease is directed against melanin cells which can be found in the heart, and because we ruled out other common causes of pericardial effusion such as tuberculosis and neoplasm. Since genetic susceptibility to autoimmune diseases is a fertile environment for the development of auto-immune diseases, which frequently coexist, we suggest that our patient presents pernicious anemia associated with VKH disease. In the light of the above observations, we suggest a systematic screening of VKH patients (Electrocardiogram, transthoracic echocardiography) for possible, potentially lethal cardiac manifestations before beginning VKH therapy.