Enfermedad relacionada con IgG4. Presentación de un caso en Ecuador y revisión de la literatura. / IgG4 related diseases. A case report in Ecuador and review of the literature.
Resumen
Resumen.
La enfermedad relacionada con IgG4 (ER-IgG4) puede involucrar múltiples órganos, donde genera tejido fibroso-inflamatorio y provoca varias manifestaciones clínicas de predominio sistémico que pueden llevar a confusión diagnóstica. En este reporte de caso se presenta un paciente masculino de 57 años de edad, con hábito tabáquico previo, hipertenso y con diagnóstico de diabetes mellitus tipo 2. Su sintomatología inicial consistió en protrusión ocular izquierda, posteriormente se le practicó toracotomía por lesión pleural y colecistectomía como tratamiento para la presunta miomatosis vesicular observada en un TAC abdominal. Los hallazgos histopatológicos evidenciaron respuesta inflamatoria crónica linfocitaria y marcada plasmocitosis policlonal en múltiples órganos, con fibrosis asociada. Se realizaron diversos estudios de laboratorio, imágenes e inmunohistopatológicos que descartaron enfermedad autoinmunitaria como una gammapatía monoclonal sospechada tras electroforesis capilar. Se optó por estudios posteriores de inmunohistoquímica que permitieron dar con el diagnóstico de ER-IgG4.
Abstract.
The IgG4-related disease (IgG4-RD) can involve multiple organs, generating fibrous-inflammatory tissue, which causes several clinical manifestations of systemic predominance that can lead to diagnostic confusion. In this case report, we present a 57-year-old male patient, former smoker, with hypertension and with a diagnosis of type II diabetes mellitus; the patient had a left ocular protrusion as initial symptomatology, a thoracotomy was later carried out due to pleural injury, and cholecystectomy as a treatment for the presumed vesicular myomatosis observed with an abdominal CT. The histopathological findings evidenced a chronic lymphocytic inflammatory response and marked polyclonal plasmacytosis in multiple organs, with associated fibrosis. Several studies were carried out to discard both a possible autoimmune disease and a suspected monoclonal gammopathy after capillary electrophoresis. Immunohistochemical studies of the affected tissues allowed for the diagnosis of ER-IgG4.
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