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[结缔组织病的肺部表现]

[Pulmonary manifestations of connective tissue diseases].

作者信息

Rehbock B

机构信息

Praxis für Diagnostische Radiologie - Spezialgebiet Lunge, Bismarckstr. 45-47, 10627, Berlin, Deutschland,

出版信息

Radiologe. 2015 Mar;55(3):241-54; quiz 255. doi: 10.1007/s00117-014-2789-1.

DOI:10.1007/s00117-014-2789-1
PMID:25693496
Abstract

Systemic autoimmune diseases are responsible for about 25% of all deaths due to interstitial lung disease; therefore, an early identification of patients with pulmonary manifestation changes the management. Detection, differential diagnostic classification and staging of the pneumological pattern of findings are largely based on high-resolution computed tomography (HR-CT). The main differential diagnostic challenges are interstitial manifestations which present with radiological-histopathological phenotypes of interstitial pneumonia. The most common form of interstitial pulmonary reaction form of connective tissue diseases is the nonspecific interstitial pneumonia (NSIP) pattern. In rheumatoid arthritis, a usual interstitial pneumonia (UIP) pattern is dominant. Uncharacteristic reactions of airways and pleura can be the leading symptom or present as accompanying findings. A serious complication is pulmonary hypertension. Drug-induced lung lesions can present with similar HR-CT morphology as connective tissue diseases and can only be differentiated in the temporal and clinical context.

摘要

系统性自身免疫性疾病约占间质性肺疾病所致所有死亡的25%;因此,早期识别有肺部表现的患者可改变治疗方案。对检查结果的肺部模式进行检测、鉴别诊断分类和分期主要基于高分辨率计算机断层扫描(HR-CT)。主要的鉴别诊断挑战是表现为间质性肺炎放射学-组织病理学表型的间质性表现。结缔组织病最常见的间质性肺反应形式是非特异性间质性肺炎(NSIP)模式。在类风湿关节炎中,通常的间质性肺炎(UIP)模式占主导。气道和胸膜的非典型反应可能是主要症状或作为伴随发现出现。严重的并发症是肺动脉高压。药物性肺损伤可呈现与结缔组织病相似的HR-CT形态,且只能在时间和临床背景中进行鉴别。

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