The rapid increase of ulcerative colitis (UC) in incidence over the past decade is consistently observed, particularly in Asian countries such as China, Korea, Hong Kong, and Japan. For that changing epidemiology of UC, current clinical features of complicated disease including colorectal cancer (CRC) and primary sclerosing cholangitis (PSC) have not yet been clarified in East Asia when compared with Western cohorts. Therefore, we conducted the study aimed to reveal the incident and clinical course of the complication in Japanese patients with UC. We retrospectively retrieved the medical records of patients with UC and those with PSC who were diagnosed in Chiba University Hospital. The comorbidity rate and clinical course of both CRC and extraintestinal manifestation were assessed in the UC cohort. Between June 1991 and August 2017, 1242 were diagnosed with UC and 69 patients were diagnosed with PSC. In the present cohort, 37 patients had PSC-UC; the cumulative risks of PSC in patients with UC and of UC in patients with PSC were 3.0% and 53.6%, respectively. The median observation periods in the PSC and UC cohorts were 6.1 and 7.7 years, respectively. CRC was identified in 23 (1.9%) patients with UC. Most CRC were curative and CRC-related death was seen in only one (0.08%) patient. In the PSC-UC cohort, CRC and cholangiocarcinoma were identified in 2 (5.41%) and 4 (10.8%) patients, respectively. From the initial diagnosis of UC, overall survival was significantly lesser in patients with PSC-UC than in those with UC (log-rank; p <0.001; 10-year survival rate: 80.4% vs. 99.4%). In regards to the burden of diseases during 26 years, patients who occurred UC ≤25 years old (young-onsets patients) tended to increase in patients with UC and those with PSC-UC. In our cohort, the comorbidity rate of PSC-UC was higher than that obtained in previous reports, and the complication of PSC indicated poor prognosis. PSC will be changing to one of the major critical complication in patients with UC in East Asia, particularly in Japan.

IgG4-related disease (IgG4-RD) is a recently established immune-mediated systemic disorder characterized by tissue infiltration of IgG4-positive plasma cells and high serum levels of IgG4. The clinical manifestations of IgG4-RD involve a variety of organs and glands, including salivary, lacrimal and thyroid glands, and pancreas, kidney, lung and skin. Recently, Tokura et al. classified the skin manifestations of IgG4-RD into seven subtypes. We report a patient with a rare clinical manifestation of hypergammaglobulinaemic purpura as a skin lesion of IgG4-RD. A 54-year-old Japanese man presented to the Division of Gastroenterology of our clinic with a 1-month history of anorexia and weight loss (5.3 kg), and a 2week history of jaundice. Blood tests revealed a high serum level of conjugated bilirubin (7.4 mg/dL, normal range < 0.5 mg/dL), high serum IgG4 level of 1140 mg/dL (normal range 4.8–105 mg/dL), low serum complement levels and high level of antinuclear antibody (ANA) (1 : 1280; normal range < 1 : 40). Biopsy of the Vater papilla revealed lymphocyte and plasma cell infiltration of the mucosa, and additional immunohistochemistry revealed 22 IgG4-positive cells per high-power field (HPF) and 55.4% (31/56) IgG4+/ IgG+ plasma cells infiltrating the tissue (Fig. 1a,b). The diagnosis was IgG4-RD according to the Japanese comprehensive diagnostic criteria for IgG4-RD, and treatment was scheduled. Prior to treatment commencement, the patient was referred to our department for a consultation because of the appearance of multiple asymptomatic palpable purpuric papules on his legs (Fig. 2a). Tests for antineutrophilic cytoplasmic antibodies were negative. We suspected the palpable purpura as a manifestation of IgG4-RD, and took a skin biopsy. Histological examination reveled swelling of vessel walls in the papillary dermis, with nuclear dust that represented leucocytoclastic vasculitis (Fig. 2b). Direct immunofluorescence identified deposition of IgG, IgM and IgA in the walls of the small vessels of the upper

Summary The short‐term prognosis of patients with severe acute exacerbation of chronic hepatitis B (CHB) leading to acute liver failure is extremely poor. We have reported the efficacy of corticosteroid in combination with nucleoside analogue in the early stages, but virological efficacy has not been documented. Our aim was to elucidate the virological efficacy of this approach. Thirteen patients defined as severe acute exacerbation of CHB by our uniform criteria were prospectively examined for virological responses to treatment. Nucleoside analogue and sufficient dose of corticosteroids were introduced as soon as possible after the diagnosis of severe disease. Of the 13 patients, 7 (54%) survived, 5 (38%) died and 1 (8%) received liver transplantation. The decline of HBV DNA was significant between the first 2 weeks ( P = 0.02) and 4 weeks ( P < 0.01). Mean reduction in HBV DNA during the first 2 weeks was 1.7 ± 0.9 log copies per mL in overall patients, 2.1 ± 0.8 in survived patients and 1.2 ± 0.9 in dead/transplanted patients. The decline of HBV DNA was significant between the first 2 weeks ( P = 0.03) and 4 weeks ( P = 0.02) in survived patients, but not in dead/transplanted patients. Our study shows that corticosteroid treatment in combination with nucleotide analogue has sufficient virological effect against severe acute exacerbation of CHB, and a rapid decline of HBV DNA is conspicuous in survived patients.