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Article TitleThe ACG Monograph on GI Diseases and Endoscopy in Pregnancy and Postpartum Period

We assessed the real-world effectiveness and safety of vedolizumab (VDZ) in moderate-severe Crohn's disease (CD). Retrospective cohort study of seven medical centers, from May 2014 to December 2015. Adults with moderate-severe CD treated with VDZ, with follow-up after initiation of therapy, were included. Using the multivariable Cox proportional hazard analyses, we identified independent predictors of clinical remission or mucosal healing with VDZ. Rates of serious infection (requiring antibiotics, resulting in discontinuation of VDZ, hospitalization or death) and serious adverse events (discontinuation of VDZ, hospitalization or death) were described quantitatively. We included 212 patients with moderate-severe CD (median age 34 years; 40% male; 90% tumor necrosis factor (TNF)-antagonist exposed) with a median follow-up (IQR) of 39 weeks (25-53). Twelve-month cumulative rates of clinical remission, mucosal healing, and deep remission (clinical remission+mucosal healing) were 35%, 63%, and 26%, respectively. Individuals with prior TNF-antagonist exposure (hazard ratio (HR) 0.40; 95% confidence interval (CI): 0.20-0.81), smoking history (HR 0.47; 95% CI: 0.25-0.89), active perianal disease (HR 0.49; 95% CI: 0.27-0.88), and severe disease activity (HR 0.54; 95% CI: 0.31-0.95) were less likely to achieve clinical remission. Those with prior TNF-antagonist exposure (HR 0.29; 95% CI: 0.12-0.73), and severe disease activity (HR 0.54; 95% CI: 0.31-0.95) were less likely to achieve mucosal healing. During 160 patient years of follow-up (PYF) and 1,433 VDZ infusions, 5 patients developed infusion reactions (3.5 per 1,000 infusions), 21 developed serious infections (13 per 100 PYF), and 17 developed serious adverse events (10 per 100 PYF). A minority of adverse events required discontinuation of therapy (6 per 100 PYF). VDZ is a safe and effective treatment option for moderate-severe CD in routine practice. Clinical remission and deep remission (clinical remission and mucosal healing) can be achieved in 1/3 of individuals, and a minority of individuals require discontinuation of therapy due to adverse events.

INTRODUCTION:Systemic mastocytosis (SM) is a rare disease characterized by infiltration of mast cells in extracutaneous organs. Although gastrointestinal (GI) manifestations such as nausea, vomiting, abdominal pain are observed in up to 60-80% of patients, colon lesions are rarely identified. Here we describe an unusual case of SM that manifested as a colon mass on routine screening colonoscopy.CASE DESCRIPTION/METHODS:A 71-year-old, asymptomatic, female with a long history of urticaria pigmentosa was referred to a gastroenterology clinic for evaluation of an eight by twelve centimeter, polypoid mass involving the cecum and ileocecal valve, found on screening colonoscopy. Subsequently, a CT scan noted mucosal hyperenhancement with slight vascular engorgement and colonic wall thickening involving the cecum and proximal ascending colon. Pathology from the initial biopsies revealed dense (>100 per high powered field) infiltration of mast cells that stained positive for CD117 and CD25 cell surface proteins. The initial, large, polypoid, cecal mass was not present on repeat colonoscopy with upper endoscopy. Instead, there was patchy nodular- appearing mucosa in the right colon. Follow-up biopsies of the upper and lower GI tract revealed dense infiltration of mast cells in the duodenum, ileum, ascending colon and transverse colon as well as elevated serum tryptase level (38 ug/L), all findings consistent with GI involvement of SM. Histamine-blocking therapy with ranitidine and cetirizine was commenced.DISCUSSION:Systemic mastocytosis results from a sporadic somatic mutation in the KIT gene that encodes a protein implicated in cell growth, division & survival that results in overproduction of mast cells that accumulate in organs. There are four subtypes of SM that range in severity from slow and indolent to rapidly progressive towards organ failure. It is important to recognize and categorize systemic involvement of mastocytosis to aid in prognosis and treatment. Frequently, GI symptoms are mild but can include more serious effects of mast cell infiltration including peptic ulcer disease, malabsorption and non-cirrhotic portal hypertension. Specifically important to gastroenterologists is the recognition of GI involvement of mastocytosis disorders and understanding of appropriate evaluation, including upper and lower endoscopy with random biopsies to identify mast cell infiltration. Although GI symptoms are frequent and result in endoscopic evaluation, there are only rare reports of colon masses.