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Table 1 The 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) criteria for immunoglobulin G4-related disease (IgG4-RD) (

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
  1. First, clinical or radiological involvement of one of 11 typical organs implicated with IgG4-RD must be established. Second, exclusion criteria consisting of 32 clinical, serological, radiological, and pathological items must be applied. Third, eight weighted inclusion criteria domains are applied- if a threshold score of 20 points is achieved, the case may be classified as IgG4-RD