Background: A new variant of endemic pemphigus foliaceus in El Bagre (El Bagre-EPF), Colombia, South America, shares features with Senear-Usher syndrome and occurs in an endemic fashion. Patients affected by El Bagre-EPF have heterogeneous antigenic reactivity not only to the skin but to other organs, including the heart. Here we test for autoantibodies to the areae compositaeof the heart (structure consisting of typical desmosomal amalgamated fascia adherensmolecules)and evaluate any possible clinical correlation.
Methods: A case-control study comparing 45 patients and 45 controls from the endemic area, matched by demographics including age, gender, weight, work activities, and comorbidities, was performed. Direct and indirect immunofluorescence, immunohistochemistry, confocal microscopic studies, and echocardiogram studies were completed.
Results: The main clinical abnormally seen in the El Bagre-EPF patients was left ventricular hypertrophy in 15/45 patients, compared with no such findings in the control population (P < 0.1). Seventy percent of El Bagre-EPF patients and none of the controls displayed polyclonal autoreactivity using different immunoglobulins and complement to the areae compositae of the heart using different methods and antibodies (P < 0.1).
Conclusions: Patients affected by El Bagre-EPF demonstrated autoantibodies to the areae compositae of the heart. This finding was associated with left ventricular hypertrophic cardiomyopathy.The areae compositaemay play a role incell junction tension and the El Bagre-EPF patients’ autoantibodies possibly disrupting these junctions and thereby contributing to the left ventricular hypertrophy.
La fibrilación auricular es la arritmia más prevalente en la práctica clínica, y se asocia con una morbimortalidad significativa, la cual, en parte, se explica por el riesgo de fenómenos embólicos. En la actualidad, el uso de anticoagulantes es el estándar de manejo en aquellos pacientes con riesgo embólico significativo (dado por un puntaje ≥ 2 en la escala CHA 2 DS 2 Vasc). Sin embargo, algunos pacientes tienen contraindicaciones que impiden recibir este tipo de tratamiento a largo plazo, en cuyo caso se considera el cierre percutáneo de la orejuela como medida para la disminución del riesgo de embolia. Al requerir una punción transeptal, la presencia de dispositivos de cierre de defectos del septo interauricular dificulta el procedimiento de manera significativa. Se presenta el caso de una paciente con contraindicación absoluta para recibir anticoagulantes debido a sangrado gastrointestinal y antecedente de cierre percutáneo de comunicación interauricular sometida a cierre percutáneo de orejuela como alternativa terapéutica a la anticoagulación.
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