肺结节病:一篇综合综述:从过去到现在。

IF 7.9 1区 医学 Q1 IMMUNOLOGY
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2023-10-19 DOI:10.1016/j.jaut.2023.103107
John A Belperio, Michael C Fishbein, Fereidoun Abtin, Jessica Channick, Shailesh A Balasubramanian, Joseph P Lynch Iii
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引用次数: 0

摘要

结节病是一种无菌性非坏死性肉芽肿性疾病,病因不明,可累及多个器官,多发于肺和胸淋巴结。据估计,在全球范围内,每100000人中有2-160人受到影响,5年内死亡率约为7%。对于结节病患者来说,60%的病例的死因是由结节病引起的,其中高达80%的病例是由晚期心肺功能衰竭(肺动脉高压和呼吸道微生物感染)引起的。除日本外,所有种族的结节病死亡病例中,70%以上是由心脏结节病造成的。肺结节病的结节病分期与临床结果相关。在大约30%-80%的病例中,I期和II期有放射学缓解。第三阶段的分辨率只有10%-40%,而第四阶段的分辨率没有变化。高达40%的肺结节病患者进展为IV期,伴有肺实质纤维增生、支气管扩张伴肺门回缩和纤维囊性疾病。这些患者发展为毛细血管前肺动脉高压的风险最高,其中70%的患者可能发生这种情况。毛细血管前肺动脉高压的结节病患者可以对靶向肺动脉高压药物产生反应。IV期纤维细胞结节病伴明显的肺生理损伤,HRCT上纤维化>20%或毛细血管前肺动脉高压,死亡率最高,5年时可>40%。有症状(咳嗽和呼吸困难)并伴有实质浸润和肺功能测试异常(PFT)的患者的一线治疗是口服糖皮质激素,如泼尼松,典型的起始剂量为每天20-40 mg,持续2周至2个月。如果症状、肺活量测定、PFT和射线照片有所改善,泼尼松可以在6-18个月内逐渐减少。可能需要长期使用泼尼松来稳定病情。需要长期使用泼尼松≥10mg/天的患者或因糖皮质激素而产生不良反应的患者可接受二线和三线治疗。二线和三线治疗包括免疫抑制剂(如甲氨蝶呤和硫唑嘌呤)和抗肿瘤坏死因子(TNF)药物;分别地目前正在探索晚期纤维囊性肺病的有效治疗方法。尽管有不同的治疗方法,但复发率从13%到75%不等,这取决于肉瘤的分期、涉及的器官数量、社会经济地位和地理位置。结论:结节病5年随访的死亡率约为7%。不幸的是,10%-40%的结节病患者发展为进行性肺病,结节病导致的死亡中60%以上是由于进行性心肺疾病。口服糖皮质激素是一线治疗,而甲氨蝶呤和硫唑嘌呤被认为是二线治疗,抗TNF药物是第三线治疗,单独使用或作为糖皮质激素保留药物治疗有症状的肺外或肺结节病,胸部X线片上有浸润和PFT异常。根据研究人群的不同,复发率在13%到75%之间。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pulmonary sarcoidosis: A comprehensive review: Past to present.

Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2-160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis. Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%-80% of cases. Stage III only has a 10%-40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, >20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be >40% at 5-years. First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20-40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6-18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography. CONCLUSION: The mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%-40% of patients with sarcoidosis develop progressive pulmonary disease, and >60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for symptomatic extrapulmonary or pulmonary sarcoidosis with infiltrates on chest radiographs and abnormal PFT. Relapse rates have ranged from 13% to 75% depending on the population studied.

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来源期刊
Journal of autoimmunity
Journal of autoimmunity 医学-免疫学
CiteScore
27.90
自引率
1.60%
发文量
117
审稿时长
17 days
期刊介绍: The Journal of Autoimmunity serves as the primary publication for research on various facets of autoimmunity. These include topics such as the mechanism of self-recognition, regulation of autoimmune responses, experimental autoimmune diseases, diagnostic tests for autoantibodies, as well as the epidemiology, pathophysiology, and treatment of autoimmune diseases. While the journal covers a wide range of subjects, it emphasizes papers exploring the genetic, molecular biology, and cellular aspects of the field. The Journal of Translational Autoimmunity, on the other hand, is a subsidiary journal of the Journal of Autoimmunity. It focuses specifically on translating scientific discoveries in autoimmunity into clinical applications and practical solutions. By highlighting research that bridges the gap between basic science and clinical practice, the Journal of Translational Autoimmunity aims to advance the understanding and treatment of autoimmune diseases.
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