概述
肺纤维化
肺纤维化
肺纤维化可以使肺部气囊（肺泡）周围和之间的组织形成瘢痕并增厚，如右图所示。正常的肺脏和肺泡如上左图所示。
肺纤维化是一种肺部疾病，发生于受损和出现疤痕的肺组织。这种增厚、僵硬的组织使肺更加难以正常运作。随着肺纤维化的恶化，您会逐渐感觉气短。
与肺纤维化相关的瘢痕形成可由多种因素引起。但在大多数情况下，医生无法查明问题的根源。如果无法发现原因，就称为特发性肺纤维化。
肺纤维化引起的肺损伤无法修复，但药物和治疗有时可帮助缓解症状和提高生活质量。对一些人来说，可能适合肺移植。
妙佑医疗国际的肺纤维化治疗
症状
肺纤维化的体征和症状可能包括：
- 气短（呼吸困难）
- 干咳
- 疲劳
- 不明原因的体重减轻
- 肌肉和关节疼痛
- 手指或脚趾尖变宽和变圆（杵状指）
肺纤维化的病程以及症状的严重程度因人而异。有的人病得很快，病情严重。有的人则有中度症状，在数月或数年时间里缓慢恶化。
有些人的症状可能会在几天到几周内迅速恶化（急性加重），如严重气短，可能会持续几天到几周。对于急性加重患者，可能会使用机械呼吸机。医生还可能开具抗生素、皮质类固醇药物或其他药物来治疗急性加重。
When symptoms suddenly get worse
In people with pulmonary fibrosis, especially idiopathic pulmonary fibrosis, shortness of breath can suddenly get worse over a few weeks or days. This is called an acute exacerbation. It can be life-threatening. The cause of an acute exacerbation may be another condition or an illness, such as a lung infection. But usually the cause is not known.
When to see a doctor
If you have symptoms of pulmonary fibrosis, contact your doctor or other healthcare professional as soon as possible. If your symptoms get worse, especially if they get worse fast, contact your healthcare team right away.
病因
肺纤维化可在肺部肺泡周围和之间的组织形成疤痕并使之增厚。这会让氧气更难进入血液。造成这种损伤的因素多种多样，包括长期接触某些毒素、某些医疗状况、放射疗法和某些药物。
职业因素与环境因素
长期接触大量毒素和污染物会对您的肺造成损伤。这些因素包括：
- 矽尘
- 石棉样纤维
- 硬质合金粉尘
- 煤尘
- 粮食粉尘
- 鸟粪和动物粪便
放疗
一些接受放疗的肺癌或乳腺癌患者在最初治疗几个月甚至几年后会出现肺损伤的迹象。损伤的严重程度取决于：
- 肺部的辐射暴露量
- 接受的总辐射量
- 是否还使用化疗
- 基础肺病
药物
许多药物都会损伤肺部，特别是以下药物：
- 化疗药物。用于杀死癌细胞的药物，如甲氨蝶呤（Trexall、Otrexup 等）和环磷酰胺，也会损伤肺组织。
- 心脏药物。某些用于治疗心律不齐的药物，如胺碘酮（Cordarone、 Nexterone、Pacerone）可能会损伤肺组织。
- 某些抗生素。某些抗生素，如呋喃妥因（Macrobid、Macrodantin 等）或乙胺丁醇可引起肺损伤。
- 抗炎药。某些抗炎药，如利妥昔单抗（Rituxan）或柳氮磺吡啶（Azulfidine、Azulfidine）可引起肺损伤。
医疗状况
肺损伤也可由多种疾病引起，包括:
- 皮肌炎
- 多发性肌炎
- 混合性结缔组织病
- 全身性红斑狼疮
- 类风湿关节炎
- 结节病
- 硬皮病
- 感染性肺炎
许多物质和疾病都可以导致肺纤维化。即便如此，在很多情况下无法找到具体原因。不明原因的肺纤维化叫做特发性肺纤维化。
研究人员对引发特发性肺纤维化的原因有几种理论，包括病毒和接触烟草烟雾。此外，一些形式的特发性肺纤维化在家族中遗传，遗传可能在特发性肺纤维化中发挥作用。
许多特发性肺纤维化患者也可能患有胃食管反流病（GERD）— 一种胃酸流回食管的疾病。目前已有研究在评估 GERD 是否可能是导致特发性肺纤维化的风险因素，或者 GERD 是否可能导致病情进展更快。但还需要开展更多的研究来确定特发性肺纤维化与 GERD 之间的联系。
Your work and surroundings
The type of work you do and where you work or live could be the cause or part of the cause for pulmonary fibrosis. Having continuous or repeated contact with toxins or pollutants — substances that harm the quality of water, air or land — can damage your lungs, especially if you do not wear protective gear. Examples include:
- Silica dust.
- Asbestos fibers.
- Hard metal dusts.
- Wood, coal and grain dusts.
- Mold.
- Bird and animal droppings.
Radiation treatments
Some people who receive radiation therapy to the chest, such as for lung or breast cancer, show signs of lung damage months or sometimes years after treatment. How severe the damage is may depend on:
- How much of the lung was exposed to radiation.
- The total amount of radiation given.
- Whether chemotherapy also was used.
- Whether there is underlying lung disease.
Medicines
Many medicines can damage the lungs. Some examples include:
- Chemotherapy. Medicines designed to kill cancer cells, such as methotrexate (Trexall, Otrexup, others), bleomycin and cyclophosphamide (Cytoxan), can damage lung tissue.
- Heart medicines. Some medicines used to treat irregular heartbeats, such as amiodarone (Nexterone, Pacerone), may harm lung tissue.
- Some antibiotics. Antibiotics such as nitrofurantoin (Macrobid, Macrodantin) or ethambutol (Myambutol) can cause lung damage.
- Anti-inflammatory medicines. Certain anti-inflammatory medicines such as rituximab (Rituxan) or sulfasalazine (Azulfidine) can cause lung damage.
Medical conditions
Lung damage can also result from a number of conditions, including:
- Dermatomyositis, an inflammatory disease marked by muscle weakness and a skin rash.
- Lupus, a disease that occurs when the body's immune system attacks its own tissues and organs.
- Mixed connective tissue disease, which has a mix of symptoms of different disorders, such as lupus, scleroderma and polymyositis.
- Pneumonia, an infection that inflames the air sacs in one or both lungs.
- Polymyositis, an inflammatory disease that causes muscle weakness on both sides of the body.
- Rheumatoid arthritis, an inflammatory disease that affects joints and other body systems.
- Sarcoidosis, an inflammatory disease that most often affects the lungs and lymph nodes.
- Scleroderma, a group of rare diseases that involve hardening and tightening of the skin as well as problems inside the body.
Idiopathic pulmonary fibrosis
Many substances and conditions can lead to pulmonary fibrosis. Even so, in many people, the cause is never found. But risk factors such as smoking or exposure to air pollution could be related to the condition, even if the cause cannot be confirmed. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.
Many people with idiopathic pulmonary fibrosis also may have gastroesophageal reflux disease, also called GERD. This condition occurs when acid from the stomach flows back into the esophagus. GERD may be a risk factor for idiopathic pulmonary fibrosis or cause the condition to worsen faster. But more studies are needed.
风险因素
让您更容易患肺纤维化的因素包括：
- 年龄。虽然肺纤维化在童年和婴儿时期就可诊断出，但是该病影响中年和老年人的可能性要高得多。
- 性别。与女性相比，特发性肺纤维化更容易影响男性。
- 抽烟。烟民和戒烟者患上肺纤维化的几率比从不抽烟者高得多。肺纤维化可能出现在肺气肿患者身上。
- 某些职业。如果您从事采矿、农场经营或建筑行业的工作，或者您接触已知会损伤肺部的污染物，则您患上肺纤维化的风险升高。
- 癌症治疗。对胸部进行放射治疗或使用某些化疗药物会增加肺纤维化的风险。
- 遗传因素。一些类型的肺纤维化可在家族中遗传，因此可能需要考虑基因因素。
并发症
肺纤维化的并发症可能包括：