From: IgG4-related prostatitis manifesting as urinary obstruction in a 28-year-old male
Step 1: Must be clinical or radiological involvement of: | ||
Pancreas Salivary glands Bile ducts Orbits | Kidney Lung Aorta | Retroperitoneum Pachymeninges Thyroid Gland |
OR pathological evidence of an inflammatory process accompanied by a lymphoplasmacytic infiltrate in one of these same organs | ||
Step 2: The following exclusion criteria must be applied: | ||
Clinical Fever No response to glucocorticoids Serological Leukopenia and thrombocytopenia Peripheral eosinophilia Positive ANCA antibodies Positive SSA/Ro or SSB/La antibody Positive dsDNA, RNP or Sm antibody Other disease-specific autoantibody Cryoglobulinemia | Radiological Findings suspicious for malignancy or infection not fully investigated Rapid radiological progression Long bone abnormalities Splenomegaly Known diagnosis of: Multicentric Castleman’s disease Crohn’s disease or ulcerative colitis Hashimoto thyroiditis (if only thyroid affected) | Pathological Cellular infiltrates suggesting malignancy not fully investigated Markers consistent with inflammatory myofibroblastic tumor Prominent neutrophilic inflammation Necrotizing vasculitis Prominent necrosis Primary granulomatous inflammation Features of macrophage disorder |
Step 3: Inclusion criteria; total points must equal or exceed 20 to be classified as IgG4-RD | ||
Histopathology Uninformative biopsy: 0 Dense lymphocytic infiltrate: + 4 Dense lymphocytic infiltrate and obliterative phlebitis: + 6 Dense lymphocytic infiltrate and storiform fibrosis: + 13 Chest None apply: 0 Peribronchovascular and septal thickening: + 4 Paravertebral band-like soft tissue in thorax: + 10 Pancreas and Biliary Tree None apply: 0 Diffuse pancreatic enlargement (loss of lobulations): + 8 Diffuse pancreatic enlargement + capsule-like rim with decreased enhancement: + 11 Pancreatic and biliary tree involvement: + 19 | Immunostaining IgG4/IgG ratio 0–40% and number of IgG4 cells/hpf is 0–9: 0 IgG4/IgG ratio ≥ 41% and number of IgG4 cells/hpf is 0–9 OR ratio is 0–40% and cells/hpf ≥ 10: + 7 IgG4/IgG ratio 41–70% and number of IgG4 cells/hpf is ≥ 10 OR ratio is ≥ 71% and cells/hpf 10–50: + 14 IgG4/IgG ratio ≥ 71% and number of IgG4 cells/hpf is ≥ 51: + 16 Serum IgG4 concentration Normal or not detected: 0 > Normal to < 2 × ULN: + 4 2–5 × ULN: + 6 ≥ 5 × ULN: + 11 | Bilateral lacrimal, parotid, sublingual, and submandibular glands None involved: 0 1 involved: + 6 2 + involved: + 14 Retroperitoneum None apply: 0 Diffuse thickening of the abdominal aortic wall: + 4 Circumferential or anterolateral soft tissue around the infrarenal aorta or iliac arteries: + 8 Kidney None apply: 0 Hypocomplementemia: + 6 Renal pelvis thickening/soft tissue: + 8 Bilateral renal cortex low-density areas: + 10 |