|
| (1) Presence of some kidney damage, as manifested by abnormal urinalysis or urine marker(s) or decreased kidney function with either elevated serum IgG or IgE or hypocomplementemia | |
| (2) Abnormal renal radiologic findings: | |
| (a) multiple low-density lesions on enhanced computed tomography; | |
| (b) diffuse kidney enlargement; | |
| (c) hypovascular solitary mass in the kidney; | |
| (d) hypertrophic lesion of the renal pelvic wall without irregularities of the renal pelvic surface. | |
| (3) Elevated serum IgG4 level (>135 mg/dL) | |
| (4) Histological findings in the kidney: | |
| (a) dense lymphoplasmacytic infiltration by >10 IgG4-positive plasma cells/high power field (HPF) and/or IgG4+/IgG+ positive plasma cells > 40%; | |
| (b) characteristic (sclero-) fibrosis surrounding nests of lymphocytes and/or plasma cells; | |
| (5) Histological findings in extrarenal organ(s): | |
| dense lymphoplasmacytic infiltration by >10 IgG4-positive plasma cells/HPF and/or IgG4/IgG-positive plasma cells > 40% | |
| Definite: (1) + (3) + (4) (a), (b) | |
| (2) + (3) + (4) (a), (b) | |
| (2) + (3) + (5) | |
| (1) + (3) + (4) (a) + (5) | |
| Probable: (1) + (4) (a), (b) | |
| (2) + (4) (a), (b) | |
| (2) + (5) | |
| (3) + (4) (a), (b) | |
| Possible: (1) + (3) | |
| (2) + (3) | |
| (1) + (4) (a) | |
| (2) + (4) (a) | |
| Appendix: | |
| (1) Clinically and histologically, the following diseases should be excluded: | |
| Wegener’s granulomatosis, Churg-Strauss syndrome, and extramedullary plasmacytoma. | |
| (2) Radiologically, the following diseases should be excluded: | |
| Malignant lymphoma, urinary tract carcinomas, renal infarction, and pyelonephritis. | |
| (Rarely, Wegener’s granulomatosis, sarcoidosisand metastatic carcinoma) | |
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