Journal
INTERNATIONAL JOURNAL OF RHEUMATOLOGY
Volume 2012, Issue -, Pages -Publisher
HINDAWI LTD
DOI: 10.1155/2012/580814
Keywords
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Categories
Funding
- Ministry of Health, Labor, and Welfare (MHLW) of Japan
- Japanese Ministry of Education, Culture, Sports, Science and Technology [13557160, 15024236, 15390313, 13877075, 17591060]
- Uehara Memorial Foundation
- Vehicle Racing Commemorative Foundation
- Kanazawa Medical University Research Foundation [C2009-4, S2004-16, S2007-5]
- Kanazawa Medical University [K2011-7]
- High-Tech Research Center of Kanazawa Medical University [H2011-11]
- Grants-in-Aid for Scientific Research [24791010] Funding Source: KAKEN
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IgG4-related disease is a new disease classification established in Japan in the 21st century. Patients with IgG4-related disease display hyper-IgG4-gammaglobulinemia, massive infiltration of IgG4+ plasma cells into tissue, and good response to glucocorticoids. Since IgG4 overexpression is also observed in other disorders, it is necessary to diagnose IgG4-related disease carefully and correctly. We therefore sought to determine cutoff values for serum IgG4 and IgG4/IgG and for IgG4+/IgG+ plasma cells in tissue diagnostic of IgG4-related disease. Patients and Methods. We retrospectively analyzed serum IgG4 concentrations and IgG4/IgG ratio and IgG4+/IgG+ plasma cell ratio in tissues of 132 patients with IgG4-related disease and 48 patients with other disorders. Result. Serum IgG4 > 135 mg/dl demonstrated a sensitivity of 97.0% and a specificity of 79.6% in diagnosing IgG4-related disease, and serum IgG4/IgG ratios > 8% had a sensitivity and specificity of 95.5% and 87.5%, respectively. IgG4+ cell/IgG+ cell ratio in tissues > 40% had a sensitivity and specificity of 94.4% and 85.7%, respectively. However, the number of IgG4+ cells was reduced in severely fibrotic parts of tissues. Conclusion. Although a recent unanimous consensus of all relevant researchers in Japan recently established the diagnostic criteria for IgG4-related disease, findings such as ours indicate that further discussion is needed.
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