Abstract
IgG4-related disease has recently been recognized as a systemic condition that is characterized by increased serum IgG4 levels and responsiveness to steroid therapy. Patients with IgG4-RD show organ enlargement or nodular lesions with abundant lymphocyte and IgG4-positive plasma cell infiltration and fibrosis, and they frequently present both clinically and radiologically with findings suggestive of malignancy, which may result in unnecessary resection [1–3]. According to the comprehensive clinical diagnostic criteria for IgG4-RD [4], the diagnosis of IgG4-RD is made in the presence of a characteristic diffuse/localized swelling or mass in one or more organs with increased serum IgG4 levels or histological findings of abundant IgG4-positive plasma cell and lymphocyte infiltration associated with fibrosis.
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