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OBJECTIVE: To report two new cases of IgG4-related retroperitoneal fibrosis, a recently described pathology.
METHODS: We analyze two cases diagnosed in our center and performed a literature review.
RESULT: IgG4 related disease is a recently described entity that includes previously not related pathologies. The clinical manifestations are highly variable and its presentation is usually subacute. The treatment of choice is glucocorticoids. In our first case the outcome was favorable with corticosteroids and azathioprine. However, the second case required surgery on 2 occasions with radical nephrectomy. The diagnosis of the latter was made nine years after the onset of symptoms when the biopsy was reviewed; at that moment immunosuppressive therapy was not started.
CONCLUSIONS: It is very important to know and diagnose this disease because of the good response to treatment that prevents complications.
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