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L.Y.C. Chen et al.
Hematologists should include IgG4-RD in the differential diagnosis of these abnormalities.
• Other common manifestations of IgG4-RD include autoimmune pancreatitis, obstructive jaundice, orbital pseudotumor, lacrimal and salivary gland swelling, retroperitoneal fibrosis, and tubulointerstitial nephritis.
• Serum protein electrophoresis and IgG subclass evalu- ation should be performed in patients with suspected IgG4-RD. Serum IgG4 levels are elevated in approximate- ly 70% of cases. Mildly increased serum IgG4 (1.5-5 g/L) is a non-specific finding, but a markedly elevated level (>5 g/L) is 90% specific for IgG4-RD.
• A firm diagnosis requires histological confirmation based on the International Consensus Criteria which
include a dense lymphoplasmacytic infiltrate, storiform fibrosis and obliterative phlebitis. There must be an increased number of IgG4+ plasma cells with an IgG4:IgG plasma cell ratio >40%.
• Early recognition and diagnosis are essential because patients typically respond well to steroids or rituximab in the early stages of the disease, but fibrotic disease and late com- plications such as chronic pancreatitis are often irreversible.
Acknowledgments
The authors thank the clinicians and pathologists of the IgG4 West working group in Vancouver for their invaluable collabora- tion, and Dr. Erica Peterson for providing critical comments on the draft of this manuscript.
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