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| (1) Presence of some kidney damage, as manifested by abnormal urinalysis or urine marker(s) or decreased kidney function with either elevated serum IgG level, hypocomplementemia, or elevated serum IgE level |
| (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 renal pelvic wall without irregularity of the renal pelvic surface |
| (3) Elevated serum IgG4 level (IgG4 ≥ 135 mg/dl) |
| (4) Histologic findings in the kidney |
| (a) Dense lymphoplasmacytic infiltration with infiltrating IgG4-positive plasma cells > 10/high-power field (HPF) and/or IgG4/IgG-positive plasma cells > 40% |
| (b) Characteristics fibrosis surrounding nests of lymphocytes and/or plasma cells |
| (5) Histologic findings in extrarenal organ(s): |
| Dense lymphoplasmacytic infiltration with infiltrating IgG4-positive plasma cells > 10/HPF and/or IgG4/IgG-positive plasma cells > 40% in extrarenal organ(s) |
| 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, sarcoidosis, and metastatic carcinoma) |
| (3) Cases with suspected disease according to the diagnostic algorithm are classified into probable or possible IgG4-RKD according to these criteria |
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