Nationwide survey for primary sclerosing cholangitis and IgG4‐related sclerosing cholangitis in Japan

Abstract

Background We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4-related sclerosing cholangitis (IgG4-SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4-SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4-SC lacking pancreatic involvement. Methods The design was a questionnaire-based, multicenter retrospective study. The enrolled subjects were patients with PSC and IgG4-SC without pancreatic involvement diagnosed after 2005. Results We enrolled 197 PSC and 43 IgG4-SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4-SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed.IgG4-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 IgG4 were significantly elevated in IgG4-SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4-SC was considerably better than that of PSC. Conclusion We confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.

Ulcerative Colitis with and without Primary Sclerosing Cholangitis: Two Different Diseases?

“…The study from Singapore [26] had enrolled 10 cases with PSC (mean age 50.9 years), confirming the male predominance; however, the outcome was better, with only 1 death during 6.6 years of follow-up. The only epidemiological data available in Asia are data from our group presenting the prevalence of PSC in Japan, as reported in 2014 [29]. According to this work and other previously reported data from the West, incidence and prevalence rates of PSC are summarized in table 1.…”

“…According to this study [29], several characteristic features of Japanese PSC patients, which had been scarcely demonstrated before by epidemiological studies from other countries, were demonstrated. First, there were 2 peaks in age distribution at diagnosis, that is, in the 3rd and 7th decades of life (fig.…”

“…In the study from Singapore [26], IBD was found in 20% of patients with PSC, although only 10 cases were included. In our nationwide study in Japan, however, the prevalence of IBD as a comorbidity was low in Japanese PSC patients [29]. The presence of IBD was restricted to only 34% (68/197) of PSC patients.…”

The Difficulty of the Definition of Igg4 Related Sclerosing Cholangitis – Should Cases with Bile Duct Stricture Limited to Intrapancreatic Area be Included in this Disease?

“…Thus, intrapancreatic biliary stricture as well as wall thickening is rare without pancreatic head lesion [3]. In fact, according to the recent Japanese national survey of IgG4-SC patients without AIP, only 2% (3/43) belonged to Type 1 [6]. Even if intrapancreatic biliary wall thickening is observed and its pathological findings do not contradict those of IgG4 related diseases, it may be difficult to say that its inflammatory origin is bile duct because such biliary lesion is very rare without pancreatic head lesion.…”

Cholangiocarcinoma with respect to IgG4 reaction

“…Both diseases exhibit similar clinicopathological behaviors, that is, bile duct stenosis and biliary obstruction. PSC often develops into cholangiocarcinoma (incidence 7-15%, annual incidence of 0.5-1.5%) [44,45]. The relationship between cholangiocarcinoma and IgG4-SC is unclear, although a few cases of IgG4-SC are associated with cholangiocarcinoma or its precursor lesion [2,46].…”