Nationwide survey for primary sclerosing cholangitis and <scp>IgG</scp>4‐related sclerosing cholangitis in <scp>J</scp>apan

Abstract

Abstract Background We previously conducted nationwide surveys for primary sclerosing cholangitis ( PSC ) in J apan, and demonstrated several characteristic features of J apanese PSC patients, yet patients with IgG 4‐related sclerosing cholangitis ( IgG 4‐ SC ) might be misdiagnosed as PSC . Since the clinical diagnostic criteria of IgG 4‐ SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG 4‐ SC lacking pancreatic involvement. Methods The design was a questionnaire‐based, multi‐center retrospective study. The enrolled subjects were patients with PSC and IgG 4‐ SC without pancreatic involvement diagnosed after 2005. Results We enrolled 197 PSC and 43 IgG 4‐ SC patients without pancreatic lesions. The male dominance was significantly evident in IgG 4‐ SC ( P = 0.006). In patients with PSC , two peaks in age distribution were clearly observed. IgG 4‐ SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC , while serum IgG and IgG 4 were significantly elevated in IgG 4‐ SC . Inflammatory bowel disease ( IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG 4‐ SC was considerably better than that of PSC . Conclusion We confirmed several interesting clinical details of PSC in J apanese patients: two peaks in the age distribution and lower prevalence of IBD .