Description

The American College of Rheumatology (ACR) and the European Union Against Rheumatism (EULAR) published classification criteria for IgG4-related disease. These included both inclusion and exclusion criteria.


Patient selection: suspected IgG4-related disease

 

Exclusion criteria:

(1) recurrent fever (> 38°C)

(2) no objective response to glucocorticoids

(3) leukopenia and thrombocytopenia with no explanation

(4) peripheral eosinophilia (absolute eosinophil count > 3,000 per µL)

(5) positive ANCA antibody (c-ANCA, p-ANCA)

(6) positive SSA/Ro or SSB/La antibody

(7) positive double-stranded DNA, RNP or Smith antibody

(8) other disease-specific autoantibody

(9) cryoglobulinemia

(10) radiological findings suspicious for malignancy or infection, including rapid radiological progression

(11) long bone abnormalities consistent with Erdheim-Chester disease

(12) splenomegaly > 14 cm

(13) cellular infiltrate consistent with malignancy

(14) markers consistent with inflammatory myofibroblastic tumor

(15) prominent neutrophilic inflammation

(16) necrotizing vasculitis

(17) prominent necrosis

(18) primarily granulomatous inflammation

(19) evidence of a macrophage/histiocytic disorder

(20) multicentric Castleman's disease

(21) Crohn's disease or ulcerative (if only pancreatobiliary disease is present)

(22) Hashimoto's thyroiditis (if only the thyroid is affected)


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