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)
