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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