Impact of Epstein-Barr virus infection on inflammatory bowel disease (IBD) clinical outcomes.

OBJECTIVE: to evaluate the role of Epstein-Barr virus (EBV) on the intestinal mucosa in the evolution of inflammatory bowel disease (IBD). The risk factors for EBV infection and the frequency of EBV-associated lymphoproliferative disorders in IBD patients were also investigated. METHODS: intestinal biopsies of IBD patients with available EBV status determined by Epstein-Barr-encoding RNA (EBER) in situ hybridization were identified in the Pathology Database of our center. Clinical information, including phenotypic characteristics of IBD, previous treatments, diagnosis of lymphoma and patient outcome were reviewed in all cases. RESULTS: fifty-six patients with IBD (28 Crohn's disease, 27 ulcerative colitis and one unclassified colitis) were included. EBV in intestinal mucosa was positive in 26 patients (46 %) and was associated to a lymphoproliferative syndrome in one case. EBV positivity was associated with severe histological activity (52 % vs 17.2 %; p 0.007), the presence of a lymphoplasmacytic infiltrate (50 % vs 33.3 %; p 0.03) and active steroid treatment (61.5 % vs 33.3 %; p 0.03). Multivariate analyses only found an association between EBV and lymphoplasmacytosis (p 0.001). Escalation in previous treatment was significantly more frequent in the EBER+ group (53.8 % vs 26.7 %; p 0.038). No cases developed lymphoma during follow-up. CONCLUSIONS: EBV on the intestinal mucosa is associated with a poor outcome of IBD and the need for escalation of therapy. Lymphoplasmacytic infiltrate is associated with EBV infection. EBER+ patients used steroids more frequently compared with EBER- patients. No EBER+ patients developed lymphoma during follow-up.

Perianal Crohn's disease: clinical implications, prognosis and use of resources.

OBJECTIVE: to investigate the prevalence of perianal disease, the associated phenotypical factors, its influence on prognosis and its impact on the use of health resources for patients with Crohn's disease. METHODS: a unicentric retrospective observational study was performed with 430 patients with Crohn's disease tracked through a monographical consultation of intestinal inflammatory disease. Demographic and phenotypical data of Crohn's disease, pharmacological and surgical treatments, complementary tests carried out and hospital admissions were analyzed. A comparative study between those patients without perianal disease and those with perianal disease was performed, both in simple form and complex form. RESULTS: the prevalence of perianal disease was 40.2 %, and fistulas and abscesses were the most frequent manifestations. These appearances were associated with an affected rectum and the existence of extra-intestinal manifestations. The patients with perianal disease most frequently required immuno-suppressant and biological treatment, but no further abdominal surgery. Amongst the patients with perianal disease, the need for biologics was more frequent for luminal disease (42.8 % vs 30.7 %). Furthermore, more explorations were needed, aimed at the study of perianal disease and recto-colonoscopies, although more magnetic resonance (MR)/computed tomography (CT) enterographies were not required. CONCLUSIONS: perianal disease has a high prevalence among patients with Crohn's disease, especially when the rectum is affected. It is associated with a worse prognosis and more frequently requires biological treatments due to perianal and luminal evolution, especially in cases of complex perianal disease. This condition calls for more hospital admissions and complementary tests.

Megacolon in inflammatory bowel disease: response to infliximab.

Megacolon is a serious complication of inflammatory bowel disease that often requires a colectomy. Infliximab is a therapeutic alternative when conventional treatment fails, before resorting to surgery. Its use is currently based on the publication of isolated cases. We present a series of 12 patients with megacolon treated with infliximab, five with signs of systemic toxicity. Seventy-five percent of the patients avoided a colectomy during their acute episode after early infliximab treatment, 2.45 days after the megacolon diagnosis. There was a greater risk of surgery among patients with ulcerative colitis and toxicity criteria. Two more patients required follow-up surgery despite long-term infliximab treatment. No patient suffered significant treatment-related adverse effects or significant post-surgery complications.

Crohn's disease and cystic fibrosis: there is still a lot to learn.

The relationship between cystic fibrosis (CF) and the risk of developing inflammatory bowel disease (IBD) is not clear. CFTR mutations can influence dysbiosis and increased intestinal permeability, which are two key elements in the pathophysiology of IBD. These patients have increased intestinal inflammation, as demonstrated by increase pro-inflammatory gene expression in the bowel, specific fecal markers (fecal calprotectin), gross lesions (capsule endoscopy) and histological lesions on examination of surgical specimens.

IgG4-related sclerosing mesenteritis, a rare condition that causes abdominal pain.

The identification of IgG4-related disease as a distinct immune-mediated condition encompassing disorders that were traditionally seen as idiopathic has been a revolution in the diagnostic and therapeutic algorithm in several medical fields. This condition usually involves multiple organs (isolated organ involvement is uncommon except in the pancreas) with characteristic histopathological findings. We report a case that was assessed due to abdominal pain and subsequently diagnosed with IgG4-related sclerosing mesenteritis. A comprehensive work-up of the case ruled out other conditions and a diagnosis of IgG4-related sclerosing mesenteritis was made according to radiographic and histopathological criteria.

Topical mesalazine as a cause of Stevens-Johnson syndrome.

Mesalazine is a drug routinely used in ulcerative colitis and usually has few side effects. There have been reports of uncommon cases of severe mucocutaneous damage, such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), induced by salicylates. It is important to diagnose these promptly due to the high morbidity and mortality rates. We describe the case of a 46-year-old female with ulcerative proctitis, who developed SJS following topical mesalazine use. The lesions responded well to intravenous corticosteroids after discontinuation of the drug.

Downhill varices: an uncommon cause of upper gastrointestinal bleeding.

BACKGROUND: Upper gastrointestinal bleeding (UGIB) is a common condition in gastroenterology, but "Downhill Varices" (DHV) or varices of the upper oesophagus are an uncommon cause of UGIB, with different aetiology from lower third oesophageal varices and different therapeutic implications. CASE REPORT: A 28-year-old male patient, with a history of chronic kidney failure secondary undergoing haemodialysis and superior vena cava syndrome (SCVS) due to multiple catheter replacements, was admitted to the Emergency Department with haematemesis secondary to a varicose vein rupture in the proximal third of oesophagus, treated initially with ethanolamine. Subsequent diagnostic studies showed the collateral circulation secondary to the SCVS. No further endoscopic or endovascular therapy could be performed and the patient will finally undergo a surgical bypass. DISCUSSION: DHVs are a very uncommon condition and endoscopic band ligation emerges as the appropriate therapeutic approach for the bleeding event. The definitive therapy continues to be that for the cause of the SVCS.

Treatment with adalimumab in a patient with regenerative nodular hyperplasia secondary to azathioprine.

INTRODUCTION: regenerative nodular hyperplasia (RNH) is a rare liver disease with an etiology that is not well understood. Among the etiological factors are purine-analogue drugs such asazathioprine. CASE REPORT: we present a case of a 47-year-old patient diagnosed with Crohn´s disease in treatment with azathioprine due to corticosteroid dependency who developed RNH with clinical and laboratory signs of portal hypertension one year after starting treatment. After discontinuation of azathioprine, the patient started treatment and, given the poor disease progression, started treatment with adalimumab. This was continued with an excellent response and without deleterious effects on the liver. DISCUSSION: the relevance of this case is twofold: First, this is a rare and early side effect of azathioprine treatment and this is an irreversible disease with potentially serious complications. Second, because treatment was carried out with biological drugs (adalimumab) despite the patient having advance liver disease with portal hypertension without any evidence of it worsening, nor signs of deleterious effects or complications, given that there is scarce or no experience with adalimumab treatment in this type of situation.