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2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease

2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease
  Categorical assessment or numeric weight
Step 1. Entry criteria
Characteristic* clinical or radiologic involvement of a typical organ (eg, pancreas, salivary glands, bile ducts, orbits, kidney, lung, aorta, retroperitoneum, pachymeninges, or thyroid gland [Riedel's thyroiditis]) or pathologic evidence of an inflammatory process accompanied by a lymphoplasmacytic infiltrate of uncertain etiology in one of these same organs Yes or No
Step 2. Exclusion criteria: domains and itemsΔ
Clinical
  • Fever
  • No objective response to glucocorticoids

Serologic

  • Leukopenia and thrombocytopenia with no explanation
  • Peripheral eosinophilia
  • Positive antineutrophil cytoplasmic antibody (specifically against proteinase 3 or myeloperoxidase)
  • 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 evaluated
  • Markers consistent with inflammatory myofibroblastic tumor
  • Prominent neutrophilic inflammation
  • Necrotizing vasculitis
  • Prominent necrosis
  • Primarily granulomatous inflammation
  • Pathologic features of macrophage/histiocytic disorder

Known diagnosis of the following:

  • Multicentric Castleman disease
  • Crohn disease or ulcerative colitis (if only pancreatobiliary disease is present)
  • Hashimoto's thyroiditis (if only the thyroid is affected)
 Yes or No◊
If case meets entry criteria and does not meet any exclusion criteria, proceed to step 3.
Step 3. Inclusion criteria: domains and items§
Histopathology
  • Uninformative biopsy
 0
  • Dense lymphocytic infiltrate
 +4
  • Dense lymphocytic infiltrate and obliterative phlebitis
 +6
  • Dense lymphocytic infiltrate and storiform fibrosis with or without obliterative phlebitis
 +13
Immunostaining¥ 0 to 16, as follows:
  • Assigned weight is 0 if the IgG4+:IgG+ ratio is 0 to 40% or indeterminate and the number of IgG4+ cells/HPF is 0 to 9.‡
  • Assigned weight is 7 if 1) the IgG4+:IgG+ ratio is ≥41% and the number of IgG4+ cells/HPF is 0 to 9 or indeterminate; or 2) the IgG4+:IgG+ ratio is 0 to 40% or indeterminate and the number of IgG4+ cells/HPF is ≥10 or indeterminate.
  • Assigned weight is 14 if 1) the IgG4+:IgG+ ratio is 41 to 70% and the number of IgG4+ cells/HPF is ≥10; or 2) the IgG4+:IgG+ ratio is ≥71% and the number of IgG4+ cells/HPF is 10 to 50.
  • Assigned weight is 16 if the IgG4+:IgG+ ratio is ≥71% and the number of IgG4+ cells/HPF is ≥51.
Serum IgG4 concentration
  • Normal or not checked
 0
  • >Normal but <2 times upper limit of normal
 +4
  • 2 to 5 times upper limit of normal
 +6
  • >5 times upper limit of normal
 +11
Bilateral lacrimal, parotid, sublingual, and submandibular glands
  • No set of glands involved
 0
  • 1 set of glands involved
 +6
  • 2 or more sets of glands involved
 +14
Chest
  • Not checked or neither of the items listed is present
 0
  • Peribronchovascular and septal thickening
 +4
  • Paravertebral band-like soft tissue in the thorax
 +10
Pancreas and biliary tree
  • Not checked or none of the items listed is present
 0
  • Diffuse pancreas enlargement (loss of lobulations)
 +8
  • Diffuse pancreas enlargement and capsule-like rim with decreased enhancement
 +11
  • Pancreas (either of above) and biliary tree involvement
 +19
Kidney
  • Not checked or none of the items listed is present
 0
  • Hypocomplementemia
 +6
  • Renal pelvis thickening/soft tissue
 +8
  • Bilateral kidney cortex low-density areas
 +10
Retroperitoneum
  • Not checked or neither of the items listed is present
 0
  • Diffuse thickening of the abdominal aortic wall
 +4
  • Circumferential or anterolateral soft tissue around the infrarenal aorta or iliac arteries
 +8
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.

DNA: deoxyribonucleic acid; IgG4: immunoglobulin G4; RNP: ribonucleoprotein.

* Refers to enlargement or tumor-like mass in an affected organ except in 1) the bile ducts, where narrowing tends to occur, 2) the aorta, where wall thickening or aneurysmal dilatation is typical, and 3) the lungs, where thickening of the bronchovascular bundles is common.

¶ If entry criteria are not fulfilled, the patient cannot be further considered for classification as having IgG4-related disease (IgG4-RD).

Δ Assessment for the presence of exclusion criteria should be individualized depending on a patient's clinical scenario.

◊ If exclusion criteria are met, the patient cannot be further considered for classification as having IgG4-RD.

§ Only the highest-weighted item in each domain is scored.

¥ Biopsies from lymph nodes, mucosal surfaces of the gastrointestinal tract, and skin are not acceptable for use in weighting the immunostaining domain.

‡ "Indeterminate" refers to a situation in which the pathologist is unable to clearly quantify the number of positively staining cells within an infiltrate yet can still ascertain that the number of cells is at least 10/high-power field (HPF). For a number of reasons, most often pertaining to the quality of the immunostain, pathologists are sometimes unable to count the number of IgG4+ plasma cells with precision yet even so, can be confident in grouping cases into the appropriate immunostaining result category.
From: Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Arthritis Rheumatol 2020; 72:7. https://acrjournals.onlinelibrary.wiley.com/doi/10.1002/art.41120. Copyright © 2019 American College of Rheumatology. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).
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