From Kansai Med U-Japan: IgG4-Related Disease in Japan

http://www.ncbi.nlm.nih.gov/pubmed/22899936

Int J Rheumatol. 2012;2012:358371. Epub 2012 Jul 31.

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

Uchida K, Masamune A, Shimosegawa T, Okazaki K.

Source

Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, 2-3-1 Shinmachi, Hirakata, Osaka 573-1197, Japan.

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-relatedpulmonary 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.