IgG4-related diseases (IgG4-RD) are rare disorders with many organs characteristically involved. Typically, infiltration of organs can lead to pancreatitis, retroperitoneal and mediastinal fibrosis, sialadenitis and orbital inflammation) [1-4]. Multiple organ involvement with typical combinations is the rule, but affection of single organs has been described [5]. In peripheral blood, IgG4 levels are usually elevated and eosinophilia can appear. Comprehensive diagnostic criteria include characteristic clinical presentation (organ involvement), elevated serum levels of IgG4, and histopathological examination [6].

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