“…The third step was chosen to confirm an elevated serum IgG4 level, and the following step consisted of two complementary components: radiologic and histopathologic examinations. If renal pathology was not available, a careful differential diagnosis to rule out malignant lymphoma, urinary tract carcinomas, renal infarction, pyelonephritis, Wegener's granulomatosis [17,18], sarcoidosis [19] and metastatic carcinoma was necessary, and non-renal histological finding with infiltrating IgG4-positive plasma cells [10/high power field (HPF) or IgG4/IgG [40% was necessary to support the radiologic findings. As the pathologic examination part, the following characteristic renal pathological findings of IgG4-RKD were listed: (a) marked lymphoplasmacytic infiltration, accompanied by [10 infiltrating IgG4-positive plasma cells/HPF and/or a ratio of IgG4/IgG-positive plasma cells [40%, (b) characteristic fibrosis surrounding several infiltrating cells, (c) other useful findings for the differential diagnosis [positive findings: lesions extending into the renal capsule, eosinophil infiltration, well-defined regional lesion distribution, marked fibrosis, negative findings: (necrotizing) angiitis, granulomatous lesion, neutrophil infiltration, advanced tubulitis].…”