A continuous spectrum of neutrophilic dermatoses in Crohn's disease.

UNLABELLED: The inflammatory bowel disease is accompanied by cutaneous manifestations in approximately 10% of the cases. Neutrophilic dermatoses are located on the dermis and/or epidermis and are characterised on histological examination by the presence of an infiltrate that consists largely of neutrophils. The prototype of neutrophilic dematoses is Sweetacute;s syndrome; which is rarely associated with Crohns disease. CASE REPORT: A 63 year old woman was admitted to hospital with pyrexia, abdominal pain, episcleritis and skin lesions. She presented erythematous lesions on trunk, legs and arms, with tendency towards formation of plaques, nodules and vesicular pustular lesions. Both the colonoscopy and colonic biopsies confirmed the diagnosis of colonic Crohns disease. Cutaneous biopsies re-vealed an infiltrate consisting mainly of neutrophils. These biopsies, together with clinical details led to the diagnosis of Sweetacute;s syndrome. A methylprednisolone treatment rapidly improved the skin lesions and clinical symptoms. The different clinical forms of neutrophilic dermatosis are an extra intestinal manifestation of Crohns disease, and are some-times found concurrently in the same patient, which would indicate a common pathogenesis with different clinical presentations (spectrum of neutrophilic dermatoses).

Prevalence of mutations of the NOD2/CARD15 gene and relation to phenotype in Spanish patients with Crohn disease.

BACKGROUND: We assessed the prevalence of R702W, G908R, and L1007fs coding mutations in the NOD2/CARD15 gene and the genotype-phenotype relation in Spanish patients with Crohn disease. METHODS: A cohort of 204 unrelated patients with Crohn disease and 140 healthy controls were studied. The phenotype was established before commencement of genotyping. Genotyping of the R702W, G908R, and L1007fs gene polymorphisms of NOD2/CARD15 was performed by two independent laboratories using different techniques. In the case of discordant results, specific sequencing of DNA strands was performed. RESULTS: At least one mutation was present in 32.8% of patients compared to 10.7% in controls (OR = 4.08, 95% CI 2.21 to 7.50). In patients with Crohn disease, the frequency of R702W, G908R, and L1007fs carriers was 13.7%, 8.3%, and 14.2%, respectively. Compound heterozygotes and homozygotes occurred in 3.4% and 2.9% of patients and in none of the controls. The correlation of genotype-Vienna classification showed a significant association with ileal disease (RR = 1.61, 95% CI 1.21-2.15, P = 0.001) and an inverse association with colonic localization (RR = 0.29, 95% CI 0.11-0.80, P = 0.007). There was a significant association between G908R carriership and previous appendectomy, surgical interventions, and stricturing behavior. A gene-dosage effect on phenotypic characteristics was not observed. CONCLUSIONS: In a Spanish population from Madrid, mutations of the NOD2/CARD15 gene were a marker of susceptibility to Crohn disease and were associated with ileal disease. Carriers of the G908R mutation showed a stricturing disease behavior, history of appendectomy, and surgical interventions over the course of the disease.

Infliximab treatment and prognostic factors for response in patients with Crohn's disease.

OBJECTIVE: The aim of this study is to report our experience with infliximab and analyse prognostic factors for response in Crohn's disease (CD). MATERIAL AND METHODS: Consecutive patients were prospectively enrolled in the study when referred for infliximab infusion. Data collected included indication for infusion, patient epidemiological characteristics, Vienna classification, previous surgery, previous medications and extra-intestinal manifestations. Adverse events and clinical response were tabulated separately for patients with luminal or fistulous Crohn's disease. RESULTS: 28 patients were treated (7 with inflammatory and 21 with fistulizing disease). Patients received a total of 116 infusions of infliximab: 57.1% (4 of 7) of patients with luminal disease had complete response within a median of 17.5 days (range 15-28 days), and 62% (13 of 21) of patients with fistulizing disease had complete response within a median of 9 days (range 6-51 days). All patients (5) without relapse received concomitant treatment with immune modifiers. The group of patients with previous resection or perianal fistula repair had complete response more frequently p = 0.03 (OR = 30; IC 95% = 1.47-119.8). CONCLUSIONS: Infliximab is safe and beneficial in clinical practice for Crohn's disease. The re-treatment regimen of infliximab is effective in maintaining clinical response. Immunosuppressant therapy may have a role in the duration of maintained clinical remission in patients with fistulizing disease. In patients with perianal fistulizing disease infliximab treatment is more effective when previous resection or fistula repair is present.

Hidatidosis intraportal como causa de hipertensión portal / Intraportal hydatidosis as manifestation of portal hypertension

No disponible

Tratamiento con infliximab y factores predictivos de respuesta en la enfermedad de Crohn / In fliximab treatment and prognostic factors for response in patients with Crohn's disease

Objetivo: describir nuestra experiencia con el infliximab y analizar la existencia de factores predictivos de respuesta en pacientes con enfermedad de Crohn (EC).Material y métodos: estudio longitudinal prospectivo donde se incluyeron consecutivamente a los pacientes tratados con infliximab. Se clasificaron según la indicación del tratamiento, características epidemiológicas, clasificación de Viena, antecedentes de cirugía, tratamientos concomitantes y manifestaciones extraintestinales. Se recogieron los efectos secundarios derivados del tratamiento y los resultados obtenidos durante el seguimiento clínico Resultados: se incluyeron 28 pacientes (7 con enfermedad intraluminal y 21 con enfermedad fistulosa) y se realizaron en total 116 infusiones de infliximab. En el grupo de enfermedad intraluminal respondieron 4 (57,1 por ciento), con una mediana de 17,5 días (15-28). En los enfermos con indicación por enfermedad fistulosa respondieron completamente 13 (62 por ciento) con una mediana de tiempo de 9 días (6-51). Los 5 pacientes en los que no se produjo la reapertura de la enfermedad fistulosa durante el seguimiento estaban concomitantemente con inmunosupresores. No encontramos factores predictivos de respuesta excepto en el grupo de enfermos con enfermedad perianal y antecedentes de cirugías prevías que presentaron con más frecuencia respuesta completa p=0,03 (OR=30; IC 95 por ciento= 1,47-119,8).Conclusiones: el tratamiento con infliximab benefició a un alto porcentaje de pacientes. Es un tratamiento seguro y eficaz. Tiene un importante papel en el mantenimiento de la respuesta en la EC. Los inmusupresores podrían tener un papel en la duración del mantenimiento de la respuesta en la enfermedad fistulizante. En la enfermedad fistulizante perianal se obtienen mejores resultados en la inducción, cuando se ha realizado cirugía previamente (AU)