Comprehensive Diagnostic Criteria for IgG4-Related Disease
Although IgG4-related disease (IgG4-RD) is a novel and important disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells, its clinical diagnostic criteria have not been fully e
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Comprehensive Diagnostic Criteria for IgG4-Related Disease Hisanori Umehara and Kazuichi Okazaki
6.1
Introductory Remarks
IgG4-related disease (IgG4-RD) is a new disease concept first recognized in the twenty-first century [1, 2]. The condition is characterized by elevated serum IgG4 values and marked IgG4-positive plasma cell infiltration of affected lesions [1, 2]. The initial clue to its detection was contributed by Hamano et al. [3], who reported elevated serum IgG4 concentrations in patients with sclerosing pancreatitis. Following this observation, many additional reports on this disease, pertaining to clinical, pathology, radiological, and therapeutic aspects, have been supplied by investigators from Japan and beyond. IgG4-RD now attracts worldwide attention, and the formulation of a carefully considered disease concept and diagnostic criteria are both awaited eagerly. Under the auspices of the Japanese Ministry of Health, Labor and Welfare, two research groups focusing on IgG4-RD have been organized. One is a group for research on the new disease entity of IgG4-related multiorgan lymphoproliferative syndrome (IgG4+MOLPS) (group leader: Hisanori Umehara, Department of Hematology & Immunology, Kanazawa Medical University, 66 members). The other is charged with establishing diagnostic and therapeutic approaches for IgG4-related systemic sclerosing diseases (group leader: Kazuichi Okazaki, Third Depatment of Internal Medicine, Kansai Medical University; 55 members). The two authors contributed equally to this work. H. Umehara (*) Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Kahoku, Uchinada, Ishikawa 920-0293, Japan e-mail: [email protected] K. Okazaki Division of Gastroenterology and Hepatology, The third Department of Internal Medicine, Kansai Medical University, 10-15 Fumizono-cho, Moriguchi City, Osaka 570-8506, Japan e-mail: [email protected]
Because IgG4-RD is a disease process that afflicts multiple organs, both groups are comprised of a versatile membership with representation of specialists from various clinical fields, pathologists, and basic investigators. These two cooperative groups have accomplished much in the field to date, including the achievement of a broad consensus on the disease name (IgG4-RD) and publication of a comprehensive set of diagnostic criteria. In this chapter, we outline these comprehensive diagnostic criteria for IgG4-RD [4].
6.2
Comprehensive Diagnostic Criteria for IgG4-RD
The establishment of diagnostic criteria for IgG4-RD was complicated by the fact that IgG4-RD is a truly multifaceted disorder that encompasses many conditions once considered to be separate but now recognized to overlap broadly. These conditions include autoimmune pancreatitis [5–8], Mikulicz’s disease [9, 10], Riedel’s thyroiditis [11], Küttner’s tumor [11, 12], retroperitoneal fibrosis [13, 14], inflammatory pseudotumor [15], interstitial nephritis [16, 17], interstitial pneumonia [18, 19], and others [1] (Fig. 6.1). In addition, accurate pat
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