A case report of IgG4-related disease: an insidious path to the diagnosis through kidney, heart and brain

摘要: IgG4-related disease, described around the years 2000 as a form of autoimmune pancreatitis, is now increasingly accepted systemic syndrome. The diagnosis based on both comprehensive and organ-specific criteria. For kidney, Mayo clinic classification guidelines Japanese Nephrology Society are used. Ultimately, together with parameters that characterize every organ or apparatus involved, key element confirmation growing levels IgG4 in blood tissues. We describe male patient chronic renal failure associated to hypertension without proteinuria. disease was diagnosed through biopsy. After an initial positive response steroids, he presented tinnitus, histological assessment showed cerebral subsequently cardiac damage, IgG4-related. This case appears unique for type histologically documented neurological parenchymal involvement, at same time, exemplifies subtle pernicious course disease. Frequently, blurred non-specific signs prevail. Here, kidney damage minimal urinary findings, slowly progressive dysfunction other factors can be equivocated differential diagnosis. Neurological involvement represented by tinnitus alone, while alterations were completely asymptomatic. report representative changes literature which may correlated not highlights need, some cases, targeted therapeutic approaches. In addition glucocorticoids, this case, rituximab necessary.