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. 2012:2012:358371.
doi: 10.1155/2012/358371. Epub 2012 Jul 31.

Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009

Kazushige Uchida  1 , Atsushi Masamune, Tooru Shimosegawa, Kazuichi Okazaki
Affiliations

Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009

Kazushige Uchida et al. Int J Rheumatol. 2012.

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.

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Figures

Figure 1
Figure 1
Sex and age of onset of IgG4-related disease. The male : female ratio was 1 : 0.77, and the average of age of disease onset was 58.8 years.
Figure 2
Figure 2
Mikulicz' disease without autoimmune pancreatitis. The number of patients with Mikulicz's disease without autoimmune pancreatitis, who visited hospitals in Japan in 2009, was approximately 4304 (95% confidence interval; range 3360–5048).
Figure 3
Figure 3
IgG4-related retroperitoneal fibrosis without autoimmune pancreatitis. The number of patients with IgG4-related retroperitoneal fibrosis without autoimmune pancreatitis, who visited hospitals in Japan in 2009 was approximately 272 (95% confidence interval; range 246–303).
Figure 4
Figure 4
IgG4-related renal disease without autoimmune pancreatitis. The number of patients with IgG4-related renal disease without autoimmune pancreatitis, who visited hospitals in Japan in 2009, was approximately 57 (95% confidence interval; range 47–66).
Figure 5
Figure 5
IgG4-related pulmonary disease without autoimmune pancreatitis. The number of patients with IgG4-related pulmonary disease without autoimmune pancreatitis, who visited hospitals in Japan in 2009, was approximately 354 (95% confidence interval; range 283–424).
Figure 6
Figure 6
IgG4-related lymphadenopathy without autoimmune pancreatitis. The number of patients with IgG4-related lymphadenopathy without autoimmune pancreatitis, who visited hospitals in Japan in 2009, was approximately 203 (95% confidence interval; range 187–240).
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