From: Immunoglobulin G4-related Disease: A New Systemic Disease Emerging in Japan
| Step | Categorical assessment or numeric weight |
|---|---|
| Step 1. Entry criteria | Yes or No |
| Characteristic clinical or radiologic involvement of a typical organ OR pathologic evidence of an inflammatory process accompanied by a lymphoplasmacytic infiltrate of uncertain etiology | |
| Step 2. Exclusion criteria: domains and items | Yes or No |
| Clinical | |
| Fever | |
| No objective response to glucocorticoid | |
| Serologic | |
| Leukopenia and thrombocytopenia with no explanation | |
| Peripheral eosinophilia | |
| Positive antineutrophil cytoplasmatic antibody | |
| Positive SSA/Ro or SSB/La antibody | |
| Positive double-stranded DNA, RNP, or Sm antibody | |
| Other disease-specific autoantibody | |
| Cryoglobulinemia | |
| Radiologic | |
| Known radiologic findings suspicious for malignancy or infection that have not been sufficiently investigated | |
| Rapid radiologic progression | |
| Long bone abnormalities consistent with Erdheim-Chester disease | |
| Splenomegaly | |
| Pathologic | |
| Cellular infiltrates suggesting malignancy that have not been sufficiently investigated | |
| Markers consistent with inflammatory myofibroblastic tumor | |
| Prominent neutrophilic infiltration | |
| Necrotizing vasculitis | |
| Prominent necrosis | |
| Primarily granulomatous infiltration | |
| Pathologic features of macrophage/histiocytic disorder | |
| Known diagnosis of the following: | |
| Multicentric Castleman’s disease | |
| Crohn’s disease or ulcerative colitis (if only pancreatobiliary disease is present) | |
| Hashimoto thyroiditis (if only the thyroid is affected) | |
| Step 3. Inclusion criteria: dominant and items | |
| Histopathology | |
| Dense lymphocytic infiltrate | +4 |
| Dense lymphocytic infiltrate and obliterative phlebitis | +6 |
| Dense lymphocytic infiltrate and storiform fibrosis with or without obliterative phlebitis | +13 |
| Immunostaining | |
| The IgG4+:IgG+ ratio and number of IgG4+ cells/hpf | +0-16 |
| Serum IgG4 concentration | |
| Normal but < 2x upper limit of normal | +4 |
| 2-5x upper limit of normal | +6 |
| >5x upper limit of normal | +11 |
| Bilateral lacrimal, parotid, sublingual, and submandibular glands | |
| One set of glands involved | +6 |
| Two or more sets of glands involved | +14 |
| Chest | |
| Peribronchovascular and septal thickening | +4 |
| Paravertebral band-like soft tissue in the thorax | +10 |
| Pancreas and biliary tree | |
| Diffuse pancreas enlargement (loss of lobulations) | +8 |
| Diffuse pancreas enlargement and capsule-like rim with decreased enhancement | +11 |
| +19 | |
| Pancreas (either of above) and biliary tree involvement | |
| Kidney | +6 |
| Hypocomplementemia | +8 |
| Renal pelvis thickening/soft tissue | +10 |
| Bilateral renal cortex low-density areas | |
| Retroperitoneum | +4 |
| Diffuse thickening of the abdominal aortic wall | +8 |
| Circumferential or anterolateral soft tissue around the infrarenal aorta iliac arteries | |
| Step 4: Total inclusion points | |
| A case meets the classification criteria for IgG4-RD if the entry criteria are met, no exclusion criteria are present, and the total points is ≥20. |
