The efficacy of cyclosporine A as salvage therapy for severe active ulcerative colitis refractory to glucocorticoid / 中华内科杂志

Objective To clarify the efficacy and safety of cyclosporine A CsA) as salvage therapy in patients with severe active ulcerative colitis (UC) and refractory to steroids.Methods A total of 24 severe active UC patients refractory to steroids and hospitalized from 2006 to 2012,were retrospectively enrolled.Data including demographic features,clinical manifestations,laboratory tests and medications were collected.Results CsA was effective in 15 (62.5％) patients,who did not receive colectomy during 12-week administration.This regimen was tolerable in most patients.Twelve (50.0％) patients reported 16 adverse events,but only one patient withdrew CsA due to intolerance.The rates of adverse events in initial intravenous CsA including 4 mg · kg-1 · d-1,3 mg · kg-1 · d-1 and 2 mg· kg-1 · d-1 were 2/2,9/17 and 1/5 respectively.Responders had higher white blood cell count compared with non-responders (P =0.045).Conclusions CsA could be an effective alternative regimen to colectomy in severe active UC patients who are refractory to steroids.

Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis

BACKGROUND AND PURPOSE: Hypertrophic pachymeningitis (HP) is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are tapered. Here we report three HP cases that were successfully treated with rituximab (RTX). METHODS: From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m²). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI. RESULTS: All three patients were recurrence-free of neurologic symptoms and exhibited prominent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients. CONCLUSIONS: We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.

Manejo de la colitis ulcerosa grave: experiencia a partir de un hospital ministerial / Management of severe ulcerative colitis: experience from a national health service hospital

Introduction: Ulcerative colitis (UC) may present, or evolve as severe crisis. Although guidelines have defined its management and timing in decision-making, this has not been implemented at national level. Objective: To describe the clinical characteristics and management of patients with severe UC in our hospital. Patients and Methods: Retrospective review of medical records of 116 patients with inflammatory bowel disease during the period 1975-2012. Of 97 patients with UC, 21 patients with at least one severe crisis were identified. Results: The median age among patients with UC crisis was 34 years (17-43). Ten patients debuted with severe crisis. Eleven were in maintenance treatment, all with 5-aminosalicylates (5- ASA), eight with systemic steroids and five with immunomodulators. During the crisis, colonoscopy was performed to 13 patients, 11 with extensive involvement and two left colitis. All patients received steroids and 5-ASA, 14 antibiotics, and seven immunomodulators. No patient received medical rescue therapy (cyclosporine/biological therapy). Nine patients required surgery, two due to toxic megacolon and seven due to intravenous-steroid refractory episode. Five patients received parenteral nutrition and six were hospitalized in the critical patients unit. The range of stay in the hospital was 9-51 days. Conclusions: In this study, a significant percentage of patients with CU presented a severe crisis without having a standardized management. It is necessary to establish a consensus for the multidisciplinary management of severe UC. No patient had access to cyclosporine/biological therapy, more than 40 percent of the cases required surgery. These therapeutic strategies should be incorporated into the therapeutic arsenal in hospitals.

La colitis ulcerosa (CU) puede debutar, o evolucionar, como crisis grave. Aunque guías han definido su manejo y temporalidad en la toma de decisiones,esto no ha sido masificado a nivel nacional. Objetivo: Describir las características clínicas y manejo de pacientes con CU grave en nuestro centro. Pacientes y Métodos: Revisión retrospectiva de fichas clínicas de 116 pacientes con enfermedad inflamatoria intestinal, durante el período 1975-2012. De 97 pacientes con CU se identifican 21 pacientes con al menos una crisis grave. Resultados: La mediana de edad en la crisis fue 34 años (17-43). Diez pacientes debutaron con crisis grave. Once estaban en tratamiento de mantención, todos con 5-aminosalicilatos (5-ASA), ocho con esteroides sistémicos y cinco con inmunomoduladores. Durante la crisis, se realizó colonoscopia a 13 pacientes, 11 con compromiso extenso y dos con colitis izquierda. Todos recibieron esteroides y 5-ASA, 14 antibióticos y siete inmunomoduladores. Ningún paciente recibió terapia médica de rescate (ciclosporina/terapia biológica). Nueve pacientes requirieron cirugía, dos por megacolon tóxico y siete por cortico-refractariedad. Cinco pacientes recibieron nutrición parenteral y seis fueron hospitalizados en una Unidad de Pacientes Críticos. El rango de estadía hospitalaria fue de 9-51 días. Conclusiones: En este estudio un porcentaje importante de los pacientes con CU presentó una crisis grave, sin tener un manejo estandarizado. Es necesario establecer un consenso para el manejo multidisciplinario de la CU grave. Ningún paciente tuvo acceso a ciclosporina/ terapia biológica, requiriendo cirugía más de 40 por ciento de los casos. Estas estrategias terapéuticas deben ser incorporadas al arsenal terapéutico hospitalario.

Differences in the Prognosis according to the Periods of Diagnosis in Ulcerative Colitis / 대한소화기학회지

BACKGROUND/AIMS: Infliximab was approved for the treatment of ulcerative colitis (UC) in 2006 and has recently been used as rescue therapy in steroid-refractory UC. The aim of this study was to investigate the differences of medication use and prognosis in UC patients according to the periods of diagnosis. METHODS: From 1987 to 2012, a total of 1,422 patients with UC were retrospectively reviewed in 12 hospitals. The study population was divided into two groups according to the periods of diagnosis as follows; group A: 1987-2005, group B: 2006-2012. Analyzed variables were compared by using chi-square test and logistic regression analysis. RESULTS: Mean age of the subjects was 42.2 years, and the mean follow-up period was 4.7 years. In univariate analysis, the use of infliximab in group B was significantly higher than group A (4.5% vs. 7.6%, p=0.016), and UC-related hospitalization (45.8% vs. 40.1%, p=0.031) and UC-related surgery (6.4% vs. 3.5%, p=0.010) in group B was significantly lower than that of group A. The use of oral steroid in surgery group was significantly higher than non-surgery group in multivariate analysis (OR 1.85, 95% CI 1.03-3.30, p=0.039). CONCLUSIONS: Infliximab might play an important role for the treatment of steroid-refractory UC. Well-designed prospective trials based on the efficacy and safety of infliximab are required in the future.

Efficacy of Infliximab Rescue Therapy in Hospitalized Patients with Steroid-Refractory Ulcerative Colitis: Single Center Experience

BACKGROUND/AIMS: In hospitalized patients with acute steroid-refractory UC, infliximab has been demonstrated to be one of the medical rescue therapies to avoid colectomy. We report the result of a retrospective observational study to find the efficacy and safety of infliximab as a rescue therapy in our hospital. METHODS: Between January 2007 and January 2010, 9 hospitalized patients with steroid-refractory UC were selected to receive three infusions of infliximab (5 mg/kg), at weeks 0, 2, and 6. Efficacy of treatment was evaluated at 8 weeks after the first infliximab infusion and at the end of follow-up period. Adverse events related to infliximab rescue therapy were also collected. RESULTS: Seven patients (77.8%) had completed 3 infusions of infliximab and achieved clinical response at 8 weeks after the first infliximab infusion. Clinical remission rate and the rate of mucosal healing at 8 weeks were 57.1% (4/7) and 71.4% (5/7), respectively. They were followed up for median time of 24.9 months (19.5-53.6 months). One patient underwent emergency colectomy at weeks 2, due to colon perforation, while another patient had discontinued infliximab treatment at weeks 4, because of Clostridium difficile-associated colitis. Finally, colectomy was avoided in 77.8% (7/9) of cases. There was no mortality. CONCLUSIONS: Rescue therapy with infliximab has sustained clinical benefit in 88.9% of our hospitalized patients with acute steroid-refractory UC. Future prospective and long-term follow-up trials with a large number of patients are needed to confirm the efficacy and safety of the treatment.

A Case of Steroid Refractory Acute GVHD Treated with IL-2 & TNF-alpha Blocker in a Myelodysplastic Syndrome Patient who Underwent Unrelated Allogeneic Stem Cell Transplantation

Hematopoietic stem cell transplantation has evolved as a central treatment modality for the management of various hematologic malignancies. Despite adequate posttransplantation immunosuppressive therapy, acute GVHD remains a major cause of morbidity and mortality, even for the patients who have received HLA identical sibling grafts. Once established, acute GVHD is difficult to treat, and the best primary treatments such as corticosteroid have shown responses of approximately 50%. Once GVHD becomes steroid-refractory, the chances of survival are slim at best, and the possibility of long-term complications from chronic GVHD is almost always the rule. Many agents are currently being evaluated to treat this malady, including ATG, monoclonal antibodies, pentostatin, denileukin diftitox, etc. We reported here on a case of steroid refractory acute GVHD that was treated with IL-2 and TNF-alpha blocker in myelodysplastic syndrome patient who underwent unrelated allogeneic stem cell transplantation.