Quick response to rituximab in a patient with Ro52-Jo1 complex-associated antisynthetase syndrome debuting with rapidly progressive interstitial lung disease and diffuse alveolar haemorrhage / Rápida respuesta a rituximab en un paciente con síndrome antisintetasa asociado a anti-Ro52-Jo1 que debuta con enfermedad intersticial rápidamente progresiva y hemorragia alveolar difusa

Antisynthetase syndrome (AAS) is an inflammatory myopathy that may debut with interstitial lung disease (ILD). A few studies show cases in which patients with high concentrations of anti-Ro52 in patients with ILD might achieve a better response if treated with rituximab than other patients with ILD and Ro-52 whose levels are lower. We present a patient with lung involvement as the first and unique manifestation of AAS with anti-Jo and anti-Ro52-positive antibodies. The ILD was rapid and progressive and led him into the intensive care unit in a matter of days, despite several immunosuppressive treatments. As soon as the patient received this treatment, he experienced an improvement. We therefore suggest the prompt determination of anti-Ro52 antibody concentrations in this subgroup of patients, which would offer a therapeutic approach based first on rituximab over other immunosuppressive treatments (AU)

El síndrome antisintetasa (SA) es una miopatía inflamatoria que puede debutar en forma de enfermedad pulmonar intersticial (EPI). Algunos estudios muestran casos de pacientes con EPI secundaria a miopatía inflamatoria y positividad de anti-Ro52 a altas concentraciones que presentan mejor respuesta a rituximab que pacientes similares con positividad de anti-Ro52 a bajas concentraciones. Presentamos un paciente con enfermedad intersticial como primera y única manifestación de SA y con positividad para anti-Ro52 a concentraciones altas y anti-Jo1. La EPI fue rápida y progresiva, conduciéndole a ingreso en la unidad de cuidados intensivos e intubación orotraqueal a pesar de varios tratamientos inmunosupresores. Solo experimentó mejoría cuando se inició rituximab. Por ello, proponemos medir los títulos del anticuerpo contra Ro-52 en todos los pacientes con EPI secundaria a SA y positividad de anti-Ro52, y si se evidencian valores altos decidir rituximab como tratamiento de primera línea antes que otras terapias inmunosupresoras (AU)

[Clinical and functional characteristics of patients prior to lung transplantation: report of experience at the Clínica Puerta de Hierro]. / Características clínicas y funcionales antes del trasplante pulmonar. Experiencia en la Clínica Puerta de Hierro.

OBJECTIVE: The time at which lung transplantation is indicated is determined by clinical and functional criteria that vary according to the particular disease. The aim of our study was to present the criteria according to which patients were placed on waiting lists for lung transplantation in our hospital. PATIENTS AND METHODS: We analyzed retrospectively the clinical characteristics, lung function, heart function, and 6-minute walk test results of patients who had received a lung transplant in our hospital from January 2002 through September 2005. RESULTS: During the study period 100 lung transplants were performed. The mean age of the patients was 45 years (range, 15-67 years) and 57% were men. The diseases that most often led to a lung transplant were chronic obstructive pulmonary disease (COPD) (35%), pulmonary fibrosis (29%), and bronchiectasis (21%). Lung function values differed by disease: mean (SD) forced expiratory volume in 1 second (FEV1) was 20% (11%) and forced vital capacity (FVC) was 37% (15%) in patients with COPD; FEV1 was 41% (15%) and FVC, 40% (17%) in patients with pulmonary fibrosis; and FEV1 was 23% (7%) and FVC, 37% (10%) in patients with bronchiectasis. CONCLUSIONS: The patients who received lung transplants in our hospital were in advanced phases of their disease and met the inclusion criteria accepted by the various medical associations when they were placed on the waiting list.

Características clínicas y funcionales antes del trasplante pulmonar. Experiencia en la Clínica Puerta de Hierro / Clinical and functional characteristics of patients prior to lung transplantation: report of experience at the Clínica Puerta de Hierro

OBJETIVO: El momento para indicar un trasplante pulmonar está definido por criterios clínicos y funcionales diferentes para cada enfermedad. El objetivo de este estudio es presentar cuáles fueron los criterios por los que en nuestro hospital se incluyó a los pacientes en lista de espera de trasplante pulmonar. PACIENTES Y MÉTODOS: Se ha realizado un análisis retrospectivo de las características clínicas, la función respiratoria, la prueba de la marcha de 6 minutos y el estudio cardiológico de los pacientes que recibieron un trasplante pulmonar entre enero de 2002 y septiembre de 2005. RESULTADOS: En el período estudiado se realizaron 100 trasplantes pulmonares. La edad media de los pacientes era de 45 años (rango: 15-67) y el 57% eran varones. Las enfermedades que con mayor frecuencia motivaron el trasplante pulmonar fueron la enfermedad pulmonar obstructiva crónica (EPOC, 35%), la fibrosis pulmonar (29%) y las bronquiectasias (BQ) (21%). La media ± desviación estándar de la función pulmonar osciló entre el 20 ± 11% del volumen espiratorio forzado en el primer segundo (FEV1) y el 37 ± 15% de la capacidad vital forzada (FVC) en la EPOC; del 41 ± 15% del FEV1 y el 40 ± 17% de la FVC en la fibrosis pulmonar, y del 23 ± 7% del FEV1 y el 37 ± 10% de la FVC en las BQ. CONCLUSIONES: Los pacientes que recibieron un trasplante pulmonar en nuestro centro se encontraban en fases muy evolucionadas de su enfermedad y cumplían los criterios de inclusión admitidos por las diferentes sociedades médicas cuando se les incluyó en lista de espera

OBJECTIVE: The time at which lung transplantation is indicated is determined by clinical and functional criteria that vary according to the particular disease. The aim of our study was to present the criteria according to which patients were placed on waiting lists for lung transplantation in our hospital. PATIENTS AND METHODS: We analyzed retrospectively the clinical characteristics, lung function, heart function, and 6-minute walk test results of patients who had received a lung transplant in our hospital from January 2002 through September 2005. RESULTS: During the study period 100 lung transplants were performed. The mean age of the patients was 45 years (range, 15-67 years) and 57% were men. The diseases that most often led to a lung transplant were chronic obstructive pulmonary disease (COPD) (35%), pulmonary fibrosis (29%), and bronchiectasis (21%). Lung function values differed by disease: mean (SD) forced expiratory volume in 1 second (FEV1) was 20% (11%) and forced vital capacity (FVC) was 37% (15%) in patients with COPD; FEV1 was 41% (15%) and FVC, 40% (17%) in patients with pulmonary fibrosis; and FEV1 was 23% (7%) and FVC, 37% (10%) in patients with bronchiectasis. CONCLUSIONS: The patients who received lung transplants in our hospital were in advanced phases of their disease and met the inclusion criteria accepted by the various medical associations when they were placed on the waiting list

Results of clinical lung transplant from uncontrolled non-heart-beating donors.

BACKGROUND: The scarcity of grafts for lung transplant and the growing number of candidates expecting an organ has led to an increase of deaths in patients waiting for lung transplantation. Non-heart-beating donors (NHBD) represent a promising source of grafts for those who are involved in clinical lung transplantation. We present the results of our series of 17 out-of-hospital NHBD lung transplantations performed since 2002. METHODS: We have collected data from 17 donors and recipients involved in NHBD lung transplants since 2002, as well as data referring to the type of procedure and peri-operative events. We describe the incidence of post-operative complications with special attention to primary graft disfunction (PGD), bronchial healing, bronchiolitis obliterans syndrome (BOS), and survival. We used Kaplan-Meier method to obtain the survival curve. RESULTS: G2-G3 PGD was reported in 9 patients (53%), with a complete restoration of the partial pressure of arterial oxygen/fraction of inspired oxygen ratio in 170 hours for G2 and 168 hours for G3. There were no deaths directly related to PGD. Acute rejection was detected in 7 patients (41%), 4 of which exceeded grade 1. The incidence of BOS after transplantation was 1 (7%) of 14 patients during the first year, 2 (11%) of 9 in the second year, and 2 (50%) of 4 in the third year. Hospital mortality rate was 17%. The survival rates were 82% at 3 months, 69%, at 1 year, and 58% at 3 years. CONCLUSIONS: Mid-term results confirm the adequacy of uncontrolled NHBD as a promising complementary source of lung donors for clinical transplant.