Enfermedad de Crohn perianal: implicaciones clínicas, pronósticas y consumo de recursos / Perianal Crohn's disease: clinical implications, prognosis and use of resources

Objetivo: conocer la prevalencia de la enfermedad perianal, los factores fenotípicos asociados, su influencia sobre el pronóstico y el impacto en el uso de recursos sanitarios en los pacientes con enfermedad de Crohn.Métodos: estudio observacional retrospectivo unicéntrico en el que incluimos 430 pacientes con enfermedad de Crohn en seguimiento en una consulta monográfica de enfermedad inflamatoria intestinal. Analizamos datos demográficos y fenotípicos de la enfermedad de Crohn, tratamientos farmacológicos y quirúrgicos, pruebas complementarias realizadas e ingresos hospitalarios, realizando estudio comparativo entre los pacientes sin enfermedad perianal y con enfermedad perianal, así como entre las formas simples y complejas.Resultados: la prevalencia de la enfermedad perianal fue del 40,2 % y sus manifestaciones más frecuentes fueron fístulas y abscesos. Su presencia se asoció a la afectación rectal y la existencia de manifestaciones extraintestinales. Los pacientes con enfermedad perianal precisaron con más frecuencia tratamiento inmunosupresor y biológico e ingresos, pero no más cirugía abdominal. Entre los pacientes con enfermedad perianal también fue más frecuente la necesidad de biológicos por la enfermedad luminal (42,8 % vs. 30,7 %). Además, condicionó un mayor consumo de exploraciones dirigidas al estudio de la enfermedad perianal y recto colonoscopias, pero no de entero-resonancia magnética (entero-RM)/entero-tomografía axial computarizada (entero-TAC).Conclusiones: la enfermedad perianal tiene una alta prevalencia en los pacientes con enfermedad de Crohn, sobre todo cuando existe afectación rectal. Se asocia a un peor pronóstico y requiere con más frecuencia tratamientos biológicos tanto por la evolución perianal como luminal, especialmente en la enfermedad perianal compleja. Esto condiciona más necesidad de ingresos hospitalarios y realización de exploraciones complementarias. (AU)

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 en la enfermedad inflamatoria intestinal: respuesta a infliximab / No disponible

El megacolon es una complicación grave de la enfermedad inflamatoria intestinal que con frecuencia requiere colectomía. Infliximab sería una alternativa terapéutica cuando fracasa el tratamiento convencional, antes de la cirugía. En la actualidad, su uso se basa en la publicación de casos aislados. Presentamos nuestra serie de 12 pacientes con megacolon (cinco con signos de toxicidad sistémica) tratados con infliximab. El 75% de los pacientes evitaron la colectomía durante el episodio agudo tras la instauración precoz del tratamiento con infliximab (2,45 días desde el diagnóstico del megacolon), apreciando un mayor riesgo de cirugía entre los pacientes con colitis ulcerosa y criterios de toxicidad. Pese a mantener infliximab a largo plazo, dos pacientes más requirieron cirugía en el seguimiento. Ningún paciente sufrió efectos adversos relevantes en relación con el tratamiento ni complicaciones posquirúrgicas significativas

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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

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Mesalazina administrada de forma tópica como causa de síndrome de Stevens-Johnson / Topical mesalazine as a cause of Stevens-Johnson syndrome

La mesalazina es un fármaco usado habitualmente en la colitis ulcerosa que suele cursar con pocos efectos secundarios. Se han descrito casos infrecuentes de lesiones mucocutáneas graves como el síndrome de Stevens-Johnson (SSJ) y la necrólisis epidérmica tóxica (NET) secundarias a salicilatos. Es importante su diagnóstico precoz por su alta morbimortalidad. Presentamos el caso de una mujer de 46 años con proctitis ulcerosa que desarrolló un SSJ tras la administración tópica de mesalazina. La evolución de las lesiones fue favorable tras la suspensión del fármaco e inicio de corticoides intravenosos

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

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.

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.

Mesenteritis esclerosante relacionada con IgG4, una entidad rara causante de dolor abdominal / IgG4-related sclerosing mesenteritis, a rare condition that causes abdominal pain

La distinción de la enfermedad relacionada con IgG4 como una entidad inmunomediada que engloba patologías consideradas clásicamente como idiopáticas ha supuesto una revolución en el algoritmo diagnóstico y terapéutico en diferentes especialidades médicas. Esta entidad suele implicar a diversos órganos (la afectación de un órgano aislado, a excepción del páncreas, es infrecuente), lo cual determina unos hallazgos histopatológicos característicos. Presentamos el caso de un paciente valorado por dolor abdominal que, tras estudio exhaustivo y habiendo descartado otras etiologías, fue diagnosticado de mesenteritis esclerosante IgG4 cumpliendo criterios radiológicos e histopatológicos (AU)

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 (AU)

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.

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.

'Downhill varices': una causa poco frecuente de hemorragia digestiva alta / Downhill varices: an uncommon cause of upper gastrointestinal bleeding

Introducción: la hemorragia digestiva alta (HDA) es una entidad común en gastroenterología, pero las ‘Downhill varices’ (DHV) son una causa infrecuente de HDA, con diferente etiología de las varices esofágicas de tercio inferior y con diferentes implicaciones terapéuticas. Caso clínico: varón de 28 años de edad, con antecedentes de insuficiencia renal crónica sometido a hemodiálisis y síndrome de vena cava superior(SVCS) debido a múltiples sustituciones catéter, acude a urgencias por hematemesis secundaria a ruptura de una variz en tercio proximal de esófago, que inicialmente se trata con etanolamina. El estudio diagnóstico posterior demostró la circulación colateral secundaria al SVCS. No se pudo realizar terapia endoscópica o endovascular y el paciente finalmente se someterá a un bypass quirúrgico. Discusión: las DHV son una entidad infrecuente y la ligadura endoscópica con bandas se erige como el abordaje terapéutico apropiado para el evento hemorrágico. La terapia definitiva sigue siendo la de la causa del SVCS (AU)

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 (AU)

Tratamiento con inmunosupresores en paciente con enfermedad de Crohn e inmunodeficiencia común variable / Treatment with immunosupressants in a patient with Crohn's disease and common variable immunodeficiency

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Listeria monocytogenes y su relación con terapia no biológica en la enfermedad inflamatoria intestinal / Listeria monocytogenes and its relationship with non-biological therapy in inflammatory bowel disease

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Correlación entre la localización del depósito amiloide y las manifestaciones endoscópicas y clínicas en amiloidosis digestiva sintomática / Correlation between location of amyloid deposits and endoscopic and clinical manifestations in symptomatic gastrointestinal amyloidosis

No disponible