结缔组织疾病相关的间质性肺疾病。

Interstitial lung disease in the connective tissue diseases.

机构信息

Yale Interstitial Lung Disease Program, Pulmonary & Critical Care Medicine Section, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT 06510, USA.

出版信息

Clin Chest Med. 2012 Mar;33(1):123-49. doi: 10.1016/j.ccm.2012.01.004.

DOI:10.1016/j.ccm.2012.01.004

PMID:22365251

Abstract

The connective tissue diseases (CTDs) are inflammatory, immune-mediated disorders in which interstitial lung disease (ILD) is common and clinically important. Interstitial lung disease may be the first manifestation of a CTD in a previously healthy patient. CTD-associated ILD frequently presents with the gradual onset of cough and dyspnea, although rarely may present with fulminant respiratory failure. Infection and drug reaction should always be ruled out. A diagnosis of idiopathic ILD should never be made without a careful search for subtle evidence of underlying CTD. Treatment of CTD-ILD typically includes corticosteroids and immunosuppressive agents.

摘要

结缔组织病（CTD）是一种炎症性、免疫介导的疾病，其中间质性肺疾病（ILD）很常见且具有重要的临床意义。ILD 可能是之前健康患者出现 CTD 的首发表现。CTD 相关的 ILD 常表现为逐渐出现的咳嗽和呼吸困难，但罕见情况下也可能以暴发性呼吸衰竭为首发表现。应始终排除感染和药物反应。如果没有仔细寻找潜在 CTD 的细微证据，决不能轻易诊断为特发性 ILD。CTD-ILD 的治疗通常包括皮质类固醇和免疫抑制剂。