IgG4 retroperitoneal fibrosis


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  • Clinical

    • 60% of retroperitoneal fibrosis is due to IgG4
    • responds to steroids

  • Criteria

    • >30 IgG4 positive plasma cells per hpf
    • IgG4/IgG ratio >40%
    • typically in a setting of storiform fibrosis, obliterative phlebitis and tissue eosinophilia

  • Sites

    • retroperitoneal fibrosis
    • IgG4 sclerosing cholangitis
    • autoimmune pancreatitis
    • sialoadenitis

  • Stains

    • IgG, IgG4
    • CD138, CD3, CD20
    • ALK to exclude inflammatory myofibroblastic tumor
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