Resumo

VARGAS-ROCHA, Brian E.; VARGAS-ROCHA, Vladimir E.  e  SEGALES-ROJAS, Patricia. RETROPERITONEAL TUMOR IN THE CLINICAL SPECTRUM OF IGG4 RELATED DISEASE. Rev Cient Cienc Méd [online]. 2020, vol.23, n.1, pp. 87-91. ISSN 2077-3323.

We present the case of a 55-year-old woman, with noclinical pathological history, who presented with abdominal pain, abdominopelvic tomography showing retroperitoneal tumoral in the periphery of the abdominal aorta, which affects the left kidney producing hydronephrosis. Exploratory laparotomy, left radical nephrectomy, retroperitoneal tumorectomy and resection of a fragment of abdominal aorta were performed, with histopathological report, corroborated by immunohistochemistry of retroperitoneal fibrosis and IgG4 aortitis. The spectrum of diseases related to IgG4, are of immunological origin that can involve almost any organ. The epidemiology is not precise, although a greater predilection for the Asian race is observed, being more common in the seventh decade of life, it should be suspected in patients with unexplained pain in one or more organs.The diagnosis lies in the coexistence of several clinical, laboratorial and histopathological parameters, without being any of these pathognomonic. We present a case with unusual presentation of retroperitoneal fibrosis and the importance of the diagnosis lies in the therapeutic decision, since it responds well to immunosuppressive treatment, without requiring surgical management.

Palavras-chave : Autoimmune; retroperitoneal fibrosis; Ormond disease.

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