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Table 2 Proposed diagnostic criteria for IgG4-RD according to ‘Comprehensive diagnostic criteria for IgG4-related disease’ [2, 14, 15]

From: IgG4-related nephritis and interstitial pulmonary disease complicated by invasive pulmonary fungal infection: a case report

Histology (i)The specimen pathology shows the dense lymphoplasmacytic infiltrate, storiform fibrosis or obliterative phlebitis, the infiltration of IgG4-positive cells, and IgG cells more than IgG4 cells ratio of 40%;
Imaging (ii)clinical/radiological examination showing characteristic diffuse or localized swelling or masses in single or multiple organs;
Serology (iii) serum IgG4 concentration > 135 mg/dL;
(iv)Inflammatory markers such as white blood cells count and C-reactive protein concentrations are not elevated, despite the degree of lesions, their spread on imaging analysis and massive cellular infiltration on pathological examination;
Other organ involvement (v)Characteristic findings of IgG4-RD in other organs, including autoimmune pancreatitis, lung involvement, et al.
Treatment (vi)response to steroids.
  1. All 3 criteria (i + ii + iii) are needed for definite diagnosis of IgG4-RD