间质性肺疾病并发气胸、纵隔气肿的临床特点分析

doi:10.3877/cma.j.issn.1674-6902.2024.01.011

目的

分析间质性肺疾病(interstitial lung disease, ILD)并发气胸、纵隔气肿患者的临床特点。

方法

选择2011年11月至2022年8月我院收治的ILD并发气胸、纵隔气肿患者36例为对象，对ILD临床特点及转归进行分析。

结果

36例患者中，气胸20例，纵隔气肿16例，两类患者中以特发性肺纤维化(idiopathic fibrosis of the lung, IPF)占比例最高，分别为8例(40.00%)、4例(25.00%)，其他ILD类型包括：特发性胸膜肺弹力纤维增生症(idiopathic pleuroparenchymal fibroelastosis, iPPFE)、特发性非特异性间质性肺炎(idiopathic non-specific interstitial pneumonia, iNSIP)、自身免疫特征的间质性肺炎(interstitial pneumonia with autoimmune features, IPAF)、皮肌炎(dermatomyositis interstitial lung disease, DM-ILD)、类风湿性(rheumatoid interstitial lung disease, RA-ILD)、显微镜下多血管炎(microscopic polyvasculitis interstitial lung disease, MPA-ILD)、肺泡蛋白质沉积症(pulmonary alveolar proteinosis, PAP)、化疗药相关、隐源性机化性肺炎(cryptogenic organizing pneumonia, COP)。影像学类型以普通型(usual interstitial pneumonia, UIP)及NSIP为主要表现。此类人群存在咳嗽症状，可同时合并肺气肿、肺大泡、肺源性心脏病、胸腔积液、感染等。部分患者同时存在ILD加重或发生ILD急性加重期(acute exacerbation of ILD, AE-ILD)。对比转归为好转的患者，死亡患者中，出现ILD加重，发生AE-ILD，住院期间应用呼吸机治疗，发病前应用糖皮质激素治疗，入院时氧合指数相对较低的患者比例较高(P<0.05)，其中发生AE-ILD的气胸及纵隔气肿患者死亡。

结论

气胸及纵隔气肿是ILD的并发症之一，影响ILD患者的预后，应积极预防其发生，当ILD患者出现呼吸困难加重、胸痛、氧合下降等情况时，需积极完善相关检查，警惕气胸及纵隔气肿的发生。

关键词: 间质性肺疾病, 气胸, 纵隔气肿, 临床特点

Objective

To analyze the clinical characteristics of patients with interstitial lung disease (ILD) complicated with pneumothorax and mediastinal emphysema.

Methods

The clinical data of 36 ILD patients with pneumothorax and mediastinal emphysema diagnosed and treated in the respiratory department of our hospital from November, 2011 to August, 2022 were retrospectively analyzed, the clinical characteristics of this population were described, and the outcome was analyzed.

Results

There were 20 patients with pneumothorax and 16 patients with mediastinal emphysema. IPF accounted for the highest proportion of the two types of patients, 8 cases (40.00%) and 4 cases (25.00%), respectively. Other ILD types include: idiopathic pleuroparenchymal fibroelastosis(iPPFE), idiopathic non-specific interstitial pneumonia(iNSIP), interstitial pneumonia with autoimmune features(IPAF), dermatomyositis interstitial lung disease(DM-ILD), rheumatoid interstitial lung disease(RA-ILD), microscopic polyvasculitis interstitial lung disease(MPA-ILD), pulmonary alveolar proteinosis(PAP), chemotherapy related interstitial lung disease, cryptogenic organizing pneumonia(COP). usual interstitial pneumonia(UIP) and NSIP were the main imaging types. The patients had cough symptoms. They were complicated with emphysema, pulmonary bullae, pulmonary heart disease, pleural effusion, infection, etc. Some patients also had ILD exacerbation or acute exacerbation of ILD(AE-ILD). Among the patients who died, the proportion of patients with ILD exacerbation, AE-ILD, ventilator treatment during hospitalization and glucocorticoid treatment before onset was higher (P<0.05), and the proportion of patients with pneumothorax and mediastinal emphysema who developed AE-ILD was higher (P<0.05).

Conclusion

Pneumothorax and mediastinal emphysema are both of the complications of ILD. They can affect the prognosis of patients with ILD, and should be actively prevented. When patients with ILD have increased dyspnea, chest pain, decreased oxygenation and other conditions, it is necessary to actively improve the relevant examination, and be alert to the occurrence of pneumothorax and mediastinal emphysema.

Key words: Interstitial lung disease, Pneumothorax, Mediastinal emphysema, Clinical features

图1 男，80岁，IPAF，2018年6月诊断为ILD，应用糖皮质激素治疗，2018年7月23日胸部CT提示双肺网格影、磨玻璃密度影，可见牵张性支气管扩张(B)。2018年7月27日呼吸困难加重，胸部CT提示纵隔气肿形成，双肺容积较2018年7月23日缩小，左肺为著(A)

图2 病例1，男，74岁，RA-ILD(UIP)，诊断ILD后3年出现纵隔气肿(A)；病例2，女，75岁，MPA-ILD(UIP)，诊断ILD后1年出现纵隔气肿(B)；病例3，女，61岁，iPPFE，诊断ILD后1年出现气胸、纵隔气肿(C)。病例4，男，53岁，DM-ILD(NSIP)，抗MDA5抗体阳性，诊断ILD后1个月出现纵隔气肿，应用大剂量激素冲击、环磷酰胺、IVIG治疗后死亡(D)

表1 气胸及纵隔气肿患者转归情况分析[n(%)]

临床资料	气胸(n＝20)			纵隔气肿(n＝16)
临床资料	好转	死亡	P值	好转	死亡	P值
性别
男	8(72.73)	3(27.27)	1.000	6(85.71)	1(14.29)	1.000
女	7(77.78)	2(22.22)		8(88.89)	1(11.11)
气胸
单侧	10(66.67)	5(33.33)	0.266	0(0.00)	0(0.00)	0.000
双侧	5(100.00)	0(0.00)		0(0.00)	0(0.00)
肺压缩体积						0.000
<30%	12(85.71)	2(14.29)	0.131	4(100.00)	0(0.00)
>30%	3(50.00)	3(50.00)		－	－
小叶中心肺气肿^a	10(100.00)	0(0.00)	0.033	7(100.00)	0(0.00)	0.475
间质性肺气肿	12(80.00)	3(20.00)	0.560	13(92.86)	1(7.14)	0.242
ILD加重^a	5(50.00)	5(50.00)	0.033	11(84.62)	2(15.38)	1.000
皮下气肿	2(50.00)	2(50.00)	0.249	4(100.00)	0(0.00)	1.000
吸烟史	6(75.00)	2(25.00)	1.000	4(80.00)	1(20.00)	1.000
细菌感染	12(70.59)	5(29.41)	0.539	7(77.78)	2(22.22)	0.475
真菌感染	4(50.00)	4(50.00)	0.539	4(100.00)	0(0.00)	1.000
肺心病	4(57.14)	3(42.86)	0.290	5(83.33)	1(16.67)	1.000
应用呼吸机治疗^a	0(0.00)	4(100.00)	0.001	0(0.00)	0(0.00)	0.000
既往应用激素治疗^a	5(50.00)	5(50.00)	0.033	8(80.00)	2(20.00)	0.500
存在AE-ILD^a	0(0.00)	5(100.00)	0.000	1(33.33)	2(66.67)	0.025
诊断ILD到发生气胸/纵隔气肿时间(年)	2.74±2.43	6.42±6.56	0.075	2.86±2.31	1.04±1.36	0.303
氧合指数(mmHg)^a	313.60±93.47	170.60±42.77	0.004	341.38±83.59	186.00±33.94	0.025

表1 气胸及纵隔气肿患者转归情况分析[n(%)]

临床资料	气胸(n＝20)			纵隔气肿(n＝16)
临床资料	好转	死亡	P值	好转	死亡	P值
性别
男	8(72.73)	3(27.27)	1.000	6(85.71)	1(14.29)	1.000
女	7(77.78)	2(22.22)		8(88.89)	1(11.11)
气胸
单侧	10(66.67)	5(33.33)	0.266	0(0.00)	0(0.00)	0.000
双侧	5(100.00)	0(0.00)		0(0.00)	0(0.00)
肺压缩体积						0.000
<30%	12(85.71)	2(14.29)	0.131	4(100.00)	0(0.00)
>30%	3(50.00)	3(50.00)		－	－
小叶中心肺气肿^a	10(100.00)	0(0.00)	0.033	7(100.00)	0(0.00)	0.475
间质性肺气肿	12(80.00)	3(20.00)	0.560	13(92.86)	1(7.14)	0.242
ILD加重^a	5(50.00)	5(50.00)	0.033	11(84.62)	2(15.38)	1.000
皮下气肿	2(50.00)	2(50.00)	0.249	4(100.00)	0(0.00)	1.000
吸烟史	6(75.00)	2(25.00)	1.000	4(80.00)	1(20.00)	1.000
细菌感染	12(70.59)	5(29.41)	0.539	7(77.78)	2(22.22)	0.475
真菌感染	4(50.00)	4(50.00)	0.539	4(100.00)	0(0.00)	1.000
肺心病	4(57.14)	3(42.86)	0.290	5(83.33)	1(16.67)	1.000
应用呼吸机治疗^a	0(0.00)	4(100.00)	0.001	0(0.00)	0(0.00)	0.000
既往应用激素治疗^a	5(50.00)	5(50.00)	0.033	8(80.00)	2(20.00)	0.500
存在AE-ILD^a	0(0.00)	5(100.00)	0.000	1(33.33)	2(66.67)	0.025
诊断ILD到发生气胸/纵隔气肿时间(年)	2.74±2.43	6.42±6.56	0.075	2.86±2.31	1.04±1.36	0.303
氧合指数(mmHg)^a	313.60±93.47	170.60±42.77	0.004	341.38±83.59	186.00±33.94	0.025

1	高　莉，杨文芬. 不同湿化温度对间质性肺疾病患者行无创通气的影响[J/CD]. 中华肺部疾病杂志(电子版), 2018, 11(6): 703-707.
2	Ibtissam EO, Sara HC, Hamza EH, et al. Pulmonary air leak syndrome in rheumatoid arthritis patient[J]. SAGE Open Med Case Rep, 2022, 10: 10：2050313X221125361.
3	Palmucci S, Galioto F, Fazio G, et al. Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay[J]. Insights Imaging, 2022, 13(1): 108.
4	Jeng MJ, Lee YS, Tsao PC, et al. Neonatal air leak syndrome and the role of high-frequency ventilation in its prevention[J]. J Chin Med Assoc, 2012, 75(11): 551-559.
5	Fujiwara T. Pneumomediastinum in pulmonary fibrosis. Detection by computed tomography[J]. Chest, 1993, 104(1): 44-46.
6	Watanabe H, Ishikawa H, Inui T, et al. The Macklin effect in tension pneumomediastinum in a patient with interstitial pneumonia[J]. Respirol Case Rep, 2022, 10(11): e01045.
7	Nishimoto K, Fujisawa T, Yoshimura K, et al. Pneumothorax in connective tissue disease-associated interstitial lung disease[J]. PLoS One, 2020, 15(17): e0235624.
8	Gonçalves TA, Parente DB, Barreto MM. Pneumomediastinum and pneumorrhachis as complications of dermatomyositis[J]. J Bras Pneumol, 2021, 47(6): e20210352.
9	Luo M, Wang JL. Compound heterozygous mutation of RTEL1 in interstitial lung disease complicated with pneumothorax and emphysema: A case report and literature review[J]. Respirol Case Rep, 2022, 10(10): e01032.
10	Sahoo D, Kar N, Devi S, et al. A case of paraquat poisoning presenting with spontaneous pneumothorax and pneumomediastinum[J]. Cureus, 2020, 12(12): e11943.
11	Porcel José M. Secondary spontaneous pneumothorax in idiopathic pulmonary fibrosis: Grim news[J]. Respirology, 2018, 23(5): 448-449.
12	Lococo F, Boracchia L, Rapicetta C, et al. Cervical subcutaneous emphysema and pneumomediastinum as an unusual complication of idiopathic pulmonary fibrosis[J]. ANZ J Surg, 2018, 88(10): E747-E748.
13	Colombi D, Ehlers-Tenenbaum S, Palmowski K, et al. Spontaneous pneumomediastinum as a potential predictor of mortality in patients with idiopathic pulmonary fibrosis[J]. Respiration, 2016, 92(1): 25-33.
14	Iyoda A, Azuma Y, Sakai T , et al. Necessity of multi-step surgical treatment for patients with interstitial lung disease and a pneumothorax[J]. Ann Thorac Cardiovasc Surg, 2022, 28(5): 329-333.
15	Nishimoto K, Fujisawa T, Yoshimura K, et al. The prognostic significance of pneumothorax in patients with idiopathic pulmonary fibrosis[J]. Respirology, 2018, 23(5): 519-525.
16	Iwasawa T, Ogura T, Takahashi H, et al. Pneumothorax and idiopathic pulmonary fibrosis[J]. Jpn J Radiol, 2010, 28(9): 672-679.
17	Ryo Y, Osamu N, Kyuya G, et al. Pneumothorax in patients with idiopathic pulmonary fbrosis: a real-world experience[J]. BMC Pulm Med, 2021, 21(1): 5.
18	Kang J, Seo WJu, Lee EY, et al. Pleuroparenchymal fibroelastosis in rheumatoid arthritis-associated interstitial lung disease[J]. Respir Res, 2022, 23(1): 143.
19	Tavakolian K, Udongwo N, Douedi S, et al. Idiopathic pleuroparenchymal fibroelastosis[J]. J Med Cases, 2022, 13(5): 235-239.
20	Sarɩ A, Önder Ö，Armagan B, et al. Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease [J]. Turk J Med Sci, 2022, 52(1): 83-88.
21	Kono M, Nakamura Y, Enomoto Y, et al. Pneumothorax in patients with idiopathic pleuroparenchymal fibroelastosis：Incidence, clinical features, and risk factors[J]. Respiration, 2021, 100(1): 19-26.
22	Lucas RM, Gago L, Sepriano A, et al. Spontaneous pneumomediastinum，a rare manifestation of clinically amyopathic dermatomyositis[J]. ARP Rheumatol, 2022, 1: 251-254.
23	Yamaguchi K, Yamaguchi A, Itai M, et al. Clinical features of patients with anti-melanoma differentiation-associated gene-5 antibody-positive dermatomyositis complicated by spontaneous pneumomediastinum[J]. Clin Rheumatol, 2019, 38(12): 3443-3450.
24	O′Callaghan M, Rooney L, Khan J, et al. A man with malaise, myalgia, and rapidly progressive interstitial lung disease[J]. Chest, 2022, 162(3): e111-e116.
25	Imad Z, Abdalla YA, Hamza SB, et al. Triple-negative sjogren′s syndrome and recurrent pneumothorax: An uncommon presentation of autoimmune disease[J]. Cureus, 2022, 14(7): e26636.
26	Nakamata A, Tsuchiya N, Miyara T, et al. Computed tomography findings, temporal course, and clinical relevance of subpleural pulmonary interstitial emphysema in patients with pneumomediastinum[J]. Acta Radiologica Open, 2021, 10(7): 1-9.
27	Tachibana Y, Taniguchi H, Kondoh Y, et al. Pulmonary interstitial emphysema is a risk factor for poor prognosis and a cause of air leaks[J]. Respir Investig, 2019, 57(5): 444-450.
28	Okamoto S, Tsuboi H, Noma H, et al. Predictive factors for pneumomediastinum during management of connective tissue disease-related interstitial lung disease: A retrospective study[J]. Intern Med, 2021, 60(18): 2887-2897.
29	Lococo F, Boracchia L, Rapicetta C, et al. Cervical subcutaneous emphysema and pneumomediastinum as an unusual complication of idiopathic pulmonary fibrosis[J]. ANZ J Surg, 2018, 88(10): E747-E748.
30	虞松平，周　丽，陈　勇，等. 皮肌炎并发纵隔气肿危险因素分析[J]. 浙江医学，2012, 34(7): 532-534.

[1]	张艳兰, 徐琳, 王彩英, 杨洪玲, 庞琳. 56例先天性梅毒新生儿的临床特征及预后[J/OL]. 中华实验和临床感染病杂志(电子版), 2024, 18(03): 163-169.
[2]	孙剑玥, 黄瑾, 沈逸冕, 周超群, 程记伟. 重症脑卒中后呼吸机相关肺炎临床及病原学特点[J/OL]. 中华实验和临床感染病杂志(电子版), 2024, 18(01): 11-18.
[3]	卢杨, 周梦缘, 许益敏, 卢志远, 魏志豪, 肖灿. 下颌第三磨牙拔除术引起皮下气肿合并纵隔气肿1例[J/OL]. 中华口腔医学研究杂志(电子版), 2024, 18(03): 185-188.
[4]	邵世锋, 肖钦, 沈方龙, 张迅, 郝志鹏, 伍正彬, 谢晓娟, 王耀丽. 老年胸主动脉钝性伤的重症救治分析[J/OL]. 中华肺部疾病杂志(电子版), 2024, 17(05): 762-767.
[5]	胡菊英, 李银华, 洪兰, 王宏勇, 丁先军, 李承美, 谭心海. 儿童感染大叶性肺炎与支气管肺炎临床特征分析[J/OL]. 中华肺部疾病杂志(电子版), 2024, 17(05): 813-816.
[6]	翁祖平, 曾新敏, 李光明, 黄敏, 杨华. 支气管脂肪瘤一例并文献复习[J/OL]. 中华肺部疾病杂志(电子版), 2024, 17(04): 667-669.
[7]	孙晓容, 刘佳铭, 刘禹. 以单侧肺起病的继发性重症间质性肺病的综合管理一例[J/OL]. 中华肺部疾病杂志(电子版), 2024, 17(03): 499-501.
[8]	马壮, 张晶. 人博卡病毒合并急性呼吸道感染的临床特点及病原菌检出[J/OL]. 中华肺部疾病杂志(电子版), 2023, 16(06): 841-844.
[9]	陈憩, 顾于蓓. 不同亚型上消化道克罗恩病的临床特点和预后差异研究[J/OL]. 中华消化病与影像杂志(电子版), 2024, 14(02): 121-127.
[10]	栗慧慧, 林香春. 早发结直肠癌的临床特征及危险因素分析[J/OL]. 中华临床医师杂志(电子版), 2024, 18(02): 195-200.
[11]	金炜. 骨髓增生异常/骨髓增殖性肿瘤伴环形铁粒幼细胞和血小板增多的临床特点及治疗进展[J/OL]. 中华临床医师杂志(电子版), 2024, 18(01): 101-104.
[12]	李文娟, 杨帆, 张安琪, 尹晓晴, 李媛媛, 郑方芳. 夜遗尿患儿隐性脊椎裂影像表现与临床症状及疗效的相关性[J/OL]. 中华介入放射学电子杂志, 2024, 12(04): 356-361.
[13]	吴瑾文, 王利昭, 周丹, 任红. 肺部经皮穿刺活检术后气胸发生的风险因素及其风险应对方案分析[J/OL]. 中华介入放射学电子杂志, 2024, 12(02): 150-154.
[14]	陆志斌, 王黎, 何思东, 周存荣, 潘春峰. 肺淋巴管肌瘤病伴气胸胸腔镜手术治疗1例及文献复习[J/OL]. 中华胸部外科电子杂志, 2024, 11(01): 67-70.
[15]	傅林海, 赵俊俊, 丁剑毅, 杨露萍, 王彬, 魏德胜, 喻光懋. 亚肺叶切除术后迟发性纵隔气肿机制[J/OL]. 中华胸部外科电子杂志, 2024, 11(01): 62-66.

阅读次数

全文

摘要

选择文件类型/文献管理软件名称

选择包含的内容

Analysis of the clinical characteristics of interstitial lung disease complicated by pneumothorax and hystitial diastinum

模态框（Modal）标题

选择文件类型/文献管理软件名称

选择包含的内容

Analysis of the clinical characteristics of interstitial lung disease complicated by pneumothorax and hystitial diastinum