Abstract
The number of patients with autoimmune pancreatitis who visited hospitals in Japan in 2007 was approximately 2709 (95% confidence interval; range 2540–3040). Because IgG4-related disease is a new clinical entity, there are no data with regard to its prevalence. To estimate the number of patients with IgG4-related disease in Japan, we randomly selected hospitals using stratification and asked them how many patients they had with IgG4-related disease in 2009. The number of patients with Mikulicz's disease, IgG4-related retroperitoneal fibrosis, IgG4-related renal disease, IgG4-related pulmonary disease, and IgG4-related lymphadenopathy who visited hospitals in Japan in 2009 was approximately 4304 (95% confidence interval; range 3360–5048), 272 (95% confidence interval; range 264–306), 57 (95% confidence interval; range 47–66), 354 (95% confidence interval; range 283–424), and 203 (95% confidence interval; range 187–240), respectively. The total number of patients with IgG4-related disease without autoimmune pancreatitis in Japan was approximately 5190 (95% confidence interval; range 4141–6084). The male : female ratio was 1 : 0.77, and the average of age of disease onset was 58.8 years. The total number of patients with IgG4-related disease in Japan in 2009, including autoimmune pancreatitis, was approximately 8000.
Highlights
IgG4-related disease (IgG4-RD) has recently been proposed as a new disease entity, and a number of case reports and studies evaluating the clinical characteristics of IgG4RD have appeared in the literature
Type 1 autoimmune pancreatitis (AIP) is commonly complicated with other organ involvement (OOI) [4, 5]
We conducted a national survey for IgG4-RD, based on a national survey for AIP in 2009
Summary
IgG4-related disease (IgG4-RD) has recently been proposed as a new disease entity, and a number of case reports and studies evaluating the clinical characteristics of IgG4RD have appeared in the literature. In 1995, Yoshida et al proposed autoimmune pancreatitis (AIP) [1]. Type 1 AIP is characterized by IgG4. Type 2 AIP is characterized by neutrophil infiltration. Type 1 AIP is commonly complicated with other organ involvement (OOI) [4, 5]. Kamisawa et al proposed IgG4-related sclerosing disease [6]. This concept is based on sclerosing fibrosis. Systemic IgG4-related plasmacytic syndrome (SIPS) and IgG4-positive multiorgan lymphoproliferative syndrome (IgG4-MOLPS) were proposed based on lymphoproliferation [7, 8]. We conducted a national survey for IgG4-RD, based on a national survey for AIP in 2009
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