The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease

A validated, multi-domain scoring system to classify IgG4-related disease using entry criteria, exclusion criteria, and weighted inclusion domains.

Characteristic clinical or radiologic involvement of a typical organ, or pathologic evidence of an inflammatory process with a lymphoplasmacytic infiltrate of uncertain etiology in one of these same organs*

Fever*

No objective response to glucocorticoids*

Leukopenia and thrombocytopenia with no explanation*

Peripheral eosinophilia*

Positive antineutrophil cytoplasmic antibody (proteinase 3 or myeloperoxidase)*

Positive SSA/Ro or SSB/La antibody*

Positive double-stranded DNA, RNP, or Sm antibody*

Other disease-specific autoantibody*

Cryoglobulinemia*

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*

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 multicentric Castleman’s disease*

Known diagnosis of Crohn’s disease or ulcerative colitis, if only pancreatobiliary disease is present*

Known diagnosis of Hashimoto thyroiditis, if only the thyroid is affected*

Histopathology*

Uninformative biopsy0

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 points: ratio 0–40% or indeterminate and IgG4+ cells/hpf 0–90

7 points: ratio ≥41% and cells/hpf 0–9 or indeterminate; OR ratio 0–40% or indeterminate and cells/hpf ≥10 or indeterminate+7

14 points: ratio 41–70% and cells/hpf ≥10; OR ratio ≥71% and cells/hpf 10–50+14

16 points: ratio ≥71% and cells/hpf ≥51+16

Serum IgG4 concentration*

Normal or not checked0

> Normal but <2× upper limit of normal+4

2–5× upper limit of normal+6

>5× upper limit of normal+11

Bilateral lacrimal, parotid, sublingual, and submandibular glands*

No set of glands involved0

One set of glands involved+6

Two or more sets of glands involved+14

Chest*

Not checked or neither listed item is present0

Peribronchovascular and septal thickening+4

Paravertebral band-like soft tissue in the thorax+10

Pancreas and biliary tree*

Not checked or none of the listed items is present0

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 listed items is present0

Hypocomplementemia+6

Renal pelvis thickening/soft tissue+8

Bilateral renal cortex low-density areas+10

Retroperitoneum*

Not checked or neither listed item is present0

Diffuse thickening of the abdominal aortic wall+4

Circumferential or anterolateral soft tissue around the infrarenal aorta or iliac arteries+8