Ectopic ACTH syndrome in a patient with Crohn's disease.

We present the case of a 62-year-old man with Crohn's disease who consulted for abdominal pain and lower limbs edema. The patient developed Cushing's syndrome due to ectopic secretion of ACTH. Diagnostic imaging tests showed multiple metastatic liver lesions and asymmetric thickening of the ileum, that was suspected as the primary tumor. This tumor produced destabilizing gastrointestinal bleeding and an urgent surgical resection was performed. The histopathological study of the resection specimen confirmed a grade 3 neuroendocrine tumor.

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.

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.

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.

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.

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.

A Rare Case of Drug-induced Erectile Dysfunction with Adalimumab and Certolizumab Solved after Switch to Ustekinumab in a Crohn's Disease Patient.

BACKGROUND: Crohn Disease (CD) is an intestinal inflammatory condition characterized by a complex pathogenesis, with elevated levels of inflammatory cytokines. Adalimumab and certolizumab are two biologic drugs inhibiting TNF-α. OBJECTIVE: We report the first case of a probable relationship, according to Naranjo causality assessment score, between two consecutive treatments with TNF-α inhibitors and induced erectile dysfunction (ED), that disappeared after switching to another biologic drug (ustekinumab). CASE PRESENTATION: This case report describes a possible and important association of two TNF-α inhibitors (certolizumab and adalimumab) and ED in a male patient with CD, with resolution after switching to Ustekinumab (anti-interleukin 12 and 23 biologic drug). A 65 years old man experienced erectile dysfunction during treatment with an anti-TNF. The adverse effect disappeared after discontinuation of the drug. All necessary urologic exams were carried out. Adalimumab was replaced by certolizumab and sexual disfunction symptoms appeared again, improving typically at the end of treatment periods and getting worse with each new dose. RESULTS: Switching to ustekinumab lead to a resolution of the erectile dysfunction. CONCLUSION: We describe for the first time a sexual dysfunction possibly due to two similar anti TNF drugs and its resolution after the switch to another similar but different drug, highlighting the potential difference between biologic drugs.

[An unusual cause of obstructive jaundice: giant gastroduodenal artery aneurysm]. / Una causa infrecuente de ictericia obstructiva: aneurisma de la arteria gastroduodenal de grandes dimensiones.

Visceral artery aneurysms are rare vascular lesions. Hepatic aneurysms are the second most common type of visceral aneurysm after those of the splenic artery. These aneurysms often have a nonspecific clinical presentation and are difficult to diagnose before rupture. Because the natural course of hepatic aneurysms leads to rupture, with a high rate of morbidity and mortality, their early diagnosis is essential for surgical correction. We report a case of obstructive jaundice caused by a 7-cm gastroduodenal artery aneurysm in which the diagnosis was suggested by abdominal computed tomography and magnetic resonance cholangiography and was confirmed by angiography. The patient was surgically treated. In the following 48 h he presented ischemic-based acute hepatic failure and underwent left hepatic lobe resection. The patient finally died from a pulmonary thromboembolism.

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)

Prognosis of Patients with Ulcerative Colitis in Sustained Remission After Thiopurines Withdrawal.

BACKGROUND: The ideal length of treatment with thiopurines in patients with ulcerative colitis (UC) in sustained remission remains unknown. It is widely accepted that the drug withdrawal is associated with a worse outcome. The aim of this study was to analyze the outcome after this withdrawal and to identify predictors of relapse. METHODS: A multicenter and retrospective study was designed. A total of 102 patients with UC who discontinued thiopurines in a situation of sustained remission were included. All the patients were followed up until last revision or until relapse (understood as the occurrence of signs and symptoms of UC that required a rescue treatment). RESULTS: After thiopurines withdrawal, overall relapse was recorded in 32.35% of the patients: 18.88% in the first year, 36.48% in the third, and 43.04% in the fifth year after withdrawal. On multivariate analysis, predictors of relapse were the time from diagnosis of UC until the starting of thiopurines (hazard ratio [HR], 1.01; 95% confidence interval [CI], 1.01-1.02; P = 0.039), the number of relapses before the withdrawal (HR, 1.3; 95% CI, 1.01-1.66; P = 0.029), pancolitis (HR, 5.01; 95% CI, 1.95-26.43; P = 0.028), the duration of treatment with thiopurines (HR, 0.15; 95% CI, 0.03-0.66; P = 0.013) and the situation of biological remission at withdrawal (HR, 0.004; 95% CI, 0.0001-0.14; P = 0.002). CONCLUSIONS: The withdrawal of thiopurines in patients with UC, although in sustained remission, is related to a high relapse rate. Clinical variables such as the extent of the disease, the duration of treatment or time from diagnosis to the start of thiopurines should be considered before stopping these drugs.

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

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

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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

No disponible