Kawano et al reported criteria for IgG4-related kidney disease. The authors are from multiple institutions in Japan.
Criteria for IgG4-related kidney disease:
(1) one or both of the following
(1a) evidence of renal damage by urinalysis, urine marker, etc
(1b) decreased renal function with (elevated serum IgG OR elevated serum IgG OR decreased serum complement)
(2) abnormal imaging studies of the kidney
(2a) multiple low-density lesions on enhanced CT
(2b) diffuse renal enlargement
(2c) hypovascular solitary mass in the kidney
(2d) hypertrophic lesion of the renal pelvic wall without irregularities of the renal pelvic surface
(3) elevated serum IgG4 concentration (> 135 mg/dL)
(4) histologic findings n the kidney
(4a) dense lymphoplasmacytic infiltrate with > 10 IgG4 positive plasma cells per high power field (hpf) OR IgG4 positive plasma cells >40% of plasma cells
(4b) IgG4-like sclerofibrosis surrounding nests of lymphocytes and/or plasma cells
(5) histologic findings in extra-renal organs typical for IgG4 (see 4)
Other conditions in the differential diagnosis (Wegener's granulomatosis, Churg-Strauss syndrome, plasmacytoma, etc) should be excluded.
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Findings
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Interpretation
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1, 3, 4a, 4b
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definite
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2. 3. 4a. 4b
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definite
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2, 3, 5
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definite
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1, 3, 4a, 5
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definite
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1, 4a. 4b
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probable
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2, 4a, 4b
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probable
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2, 5
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probable
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3, 4a, 4b
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possible
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1, 3
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possible
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2, 3
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possible
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1, 4a
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possible
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2, 4a
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possible
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