Mañá Juan, Rubio-Rivas Manuel, Villalba Nadia, Marcoval Joaquim, Iriarte Adriana, Molina-Molina María, Llatjos Roger, García Olga, Martínez-Yélamos Sergio, Vicens-Zygmunt Vanessa, Gámez Cristina, Pujol Ramón, Corbella Xavier
Department of Internal Medicine Department of Dermatology Department of Pulmonary Department of Pathology Department of Ophthalmology Department of Neurology Department of PET Unit-Institut de Diagnòstic per la Imatge University of Barcelona Faculty of Medicine and Health Sciences, Universitat Internacional de Catalunya Bellvitge University Hospital, Bellvitge Biomedical Research Institute-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain.
Medicine (Baltimore). 2017 Jul;96(29):e7595. doi: 10.1097/MD.0000000000007595.
Cohort studies of large series of patients with sarcoidosis over a long period of time are scarce. The aim of this study is to report a 40-year clinical experience of a large series of patients at Bellvitge University Hospital, a tertiary university hospital in Barcelona, Spain. Diagnosis of sarcoidosis required histological confirmation except in certain specific situations. All patients underwent a prospective study protocol. Clinical assessment and follow-up of patients were performed by a multidisciplinary team.From 1976 to 2015, 640 patients were diagnosed with sarcoidosis, 438 of them (68.4%) were female (sex ratio F/M 2:1). The mean age at diagnosis was 43.3 ± 13.8 years (range, 14-86 years), and 613 patients (95.8%) were Caucasian. At diagnosis, 584 patients (91.2%) showed intrathoracic involvement at chest radiograph, and most of the patients had normal pulmonary function. Erythema nodosum (39.8%) and specific cutaneous lesions (20.8%) were the most frequent extrapulmonary manifestations, but there was a wide range of organ involvement. A total of 492 patients (76.8%) had positive histology. Follow-up was carried out in 587 patients (91.7%), over a mean of 112.4 ± 98.3 months (range, 6.4-475 months). Corticosteroid treatment was administered in 255 patients (43.4%), and steroid-sparing agents in 49 patients (7.7%). Outcomes were as follows: 111 patients (18.9%) showed active disease at the time of closing this study, 250 (42.6%) presented spontaneous remission, 61 (10.4%) had remission under treatment, and 165 (28.1%) evolved to chronic sarcoidosis; among them, 115 (19.6%) with mild disease and 50 (8.5%) with moderate to severe organ damage. A multivariate analysis showed that at diagnosis, age more than 40 years, the presence of pulmonary involvement on chest radiograph, splenic involvement, and the need of treatment, was associated with chronic sarcoidosis, whereas Löfgren syndrome and mediastinal lymphadenopathy on chest radiograph were indicators of good outcome.Sarcoidosis is a multisystem disease with protean clinical-radiographic manifestations. Although almost half of patients follow a spontaneous resolution or under treatment, a significant number of them may have several degrees of organ damage. This study emphasizes the value of a multidisciplinary approach and long-term follow-up by specialized teams in sarcoidosis.
长期以来，针对大量结节病患者的队列研究较为匮乏。本研究旨在报告西班牙巴塞罗那的一家三级大学医院——贝尔维奇大学医院对大量患者长达40年的临床经验。除某些特定情况外，结节病的诊断需要组织学证实。所有患者均接受前瞻性研究方案。患者的临床评估和随访由多学科团队进行。
1976年至2015年期间，640例患者被诊断为结节病，其中438例（68.4%）为女性（男女比例为2:1）。诊断时的平均年龄为43.3±13.8岁（范围为14 - 86岁），613例患者（95.8%）为白种人。诊断时，584例患者（91.2%）胸部X线片显示胸腔内受累，且大多数患者肺功能正常。结节性红斑（39.8%）和特异性皮肤病变（20.8%）是最常见的肺外表现，但器官受累范围广泛。共有492例患者（76.8%）组织学检查呈阳性。587例患者（91.7%）进行了随访，平均随访时间为112.4±98.3个月（范围为6.4 - 475个月）。255例患者（43.4%）接受了皮质类固醇治疗，49例患者（7.7%）接受了免疫抑制剂治疗。结果如下：在本研究结束时，111例患者（18.9%）疾病仍处于活动期，250例（42.6%）出现自发缓解，61例（10.4%）在治疗后缓解，165例（28.1%）发展为慢性结节病；其中，115例（19.6%）病情较轻，50例（8.5%）有中度至重度器官损害。多因素分析显示，诊断时年龄超过40岁、胸部X线片显示肺部受累、脾脏受累以及需要治疗与慢性结节病相关，而Löfgren综合征和胸部X线片显示纵隔淋巴结肿大是预后良好的指标。
结节病是一种具有多种临床 - 影像学表现的多系统疾病。尽管几乎一半的患者可自发缓解或经治疗后缓解，但仍有相当数量的患者可能存在不同程度的器官损害。本研究强调了多学科方法以及由专业团队进行长期随访在结节病治疗中的价值。
