什么是间质性肺病?

Interstitial lung disease refers to a group of about 100 chronic lung disorders characterized by inflammation and scarring that make it hard for the lungs to get enough oxygen. 这种疤痕被称为肺纤维化.

The symptoms and course of these diseases may vary from person to person. The common link between the many forms of the disease is that they all begin with inflammation.

• 毛细支气管炎: 小气道(细支气管)发炎.
• 牙槽炎: inflammation of the air sacs where oxygen and carbon dioxide exchange in the blood takes places (alveoli).
• 血管炎: inflammation that involves the small blood vessels (capillaries).

Fibrosis leads to permanent loss of your lung tissue’s ability to carry oxygen. 气囊, as well as the lung tissue around the air sacs and the lung capillaries, 会在疤痕组织形成时被破坏吗.

这种疾病的病程可缓慢，也可迅速. People who have it may notice variation in symptoms, from very mild to moderate to very severe. The condition may stay the same for a long time or it may change quickly. 这种疾病的病程难以预测. If it progresses, the lung tissue thickens and becomes stiff, making breathing more difficult.

什么导致间质性肺病?

肺间质性疾病的病因尚不清楚. 主要影响因素包括:

• 吸烟
• 某些药物或药物
• Exposure to substances at work or in the environment such as organic or inorganic dusts
• Certain connective tissue or collagen diseases and sarcoidosis
• 家族病史
• 放射治疗

肺间质性疾病的症状是什么?

Each person may experience interstitial lung disease differently, 但最常见的症状包括:

• 呼吸急促呼吸急促，尤指活动时
• 干咳，干咳，无痰
• 极度疲劳和虚弱
• 食欲不振
• 不明原因的体重减轻
• 胸部不适
• 呼吸困难，可能又快又浅
• 肺部出血

The symptoms of interstitial lung diseases may look like other lung conditions or medical problems. 与您的医疗保健提供者进行诊断.

如何诊断间质性肺疾病?

除了完整的病史和体格检查, the healthcare provider may also request pulmonary function tests. These tests help to measure the lungs’ ability to move air into and out of the lungs. 它们可能包括:

肺量测定法

肺活量计是一种用来检查肺功能的仪器. 肺活量测定法是最简单、最常见的检测方法之一. 它可以用来:

• 确定肺部接收、保持和移动空气的能力
• 寻找肺部疾病
• 看看治疗效果如何
• 确定肺部疾病的严重程度
• Find out whether the lung disease is restrictive (decreased airflow) or obstructive (disruption of airflow)

峰值流量监测

This device is used to measure the how fast you can blow air out of the lungs. Disease-related changes can cause the large airways in the lungs to slowly narrow. 这将减缓空气离开肺部的速度. This measurement is very important in evaluating how well or how poorly the disease is being controlled.

胸部x光检查

This test takes pictures of internal tissues, bones, and organs.

血液测试

Arterial blood gas may be done to check the amount of carbon dioxide and oxygen in the blood. Other blood tests may be used to look for possible infections.

CT扫描

This test uses a combination of X-rays and computer technology to produce horizontal, 或轴向, 身体的图像(通常称为切片). CT扫描比普通的x光扫描更详细.

支气管镜检查

This is direct exam of the main airways of the lungs (bronchi) using a flexible tube called a bronchoscope. 支气管镜检查有助于评估和诊断肺部疾病, 检查堵塞, 取出组织或液体样本, 并帮助清除异物. 支气管镜检查 may include a biopsy or bronchoalveolar lavage.

支气管肺泡灌洗

Removing cells from the lower respiratory tract to help identify inflammation and exclude certain causes.

肺活检

Removing a small piece of tissue from the lung so it can be examined under a microscope.

如何治疗间质性肺病?

由于病因很多，治疗方法也各不相同. 有些肺间质性疾病无法治愈. 治疗的目的是防止更多的肺疤痕, 症状管理, 帮助你保持活力和健康. 治疗不能修复已经发生的肺疤痕.

治疗方法包括:

• 肺移植
• 口腔医学, including corticosteroids to reduce inflammation and cyclophosphamide (Cytoxan) to suppress the immune system
• 氧气治疗，从便携式容器
• 肺康复

Check with your healthcare provider about getting flu and pneumococcal shots. Getting a flu shot every year can help prevent both the flu and pneumonia. 除了, 肺炎球菌会引起一些小问题, 比如耳部感染, but can also develop into life-threatening illnesses of the lungs (pneumonia), 脑和脊髓的覆盖(脑膜炎), 血液(菌血症). 任何人都可能感染肺炎球菌病, 但是2岁以下的儿童, 65岁及以上的成年人, 有某些健康问题的人, 而吸烟者的风险最高.  

肺间质性疾病要点

• Interstitial lung disease is the name for a group of 100 lung disorders that inflame or scar the lungs.
• 事故原因尚不清楚. Major contributing factors are smoking and inhaling environmental or occupational pollutants.
• The most common symptoms are shortness of breath, especially with activity, and a dry, hacking cough.
• Tests that help measure the lungs’ ability to exchange oxygen and carbon dioxide are used to diagnose the condition. 血液测试 and imaging tests may also be used to see how severe the problem is and monitor it over time.
• The goal of treatment for people with the disease is to prevent more scarring and manage symptoms.

下一个步骤

Tips to help you get the most from a visit to your healthcare provider:

• 知道你访问的原因和你想要发生什么.
• 在拜访之前，写下你想要回答的问题.
• Bring someone with you to help you ask questions and remember what your healthcare provider tells you.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your healthcare provider gives you.
• Know why a new medicine or treatment is prescribed, and how it will help you. 还要知道副作用是什么.
• 询问你的病情是否可以用其他方法治疗.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your healthcare provider if you have questions.