特发性肺纤维化（IPF）是一种罕见的肺部疾病，可导致肺部的组织变得僵硬。这使您更难接管空气并自然呼吸。
目前尚不清楚为什么IPF会发展。这个单词特发性actually means unknown.
Each case of IPF is different, so it’s important to speak with your doctor regarding the specifics about disease progression.
IPF isn’t the only condition that can make breathing more difficult.Get informed about the differences between IPF and chronic obstructive pulmonary disease (COPD)。
诊断时医生可能会考虑一些潜在原因肺纤维化。这些可以包括：
- 环境暴露，例如污染和毒素
- 药物
- radiation therapy
- 感染
- other diseases
However, in the majority of pulmonary fibrosis cases, doctors aren’t able to identify a cause. If they’re unable to find a cause, the disease will be labeled idiopathic.
Genetic factors may play some role in the development of pulmonary fibrosis and IPF, but it’s rare for these conditions to occur in more than one member of the same family.发现other risk factors for IPF here。
IPF会导致肺部疤痕和僵硬。随着时间的流逝，疤痕会变得严重，僵硬将使呼吸更加困难。
Common symptoms include:
- shortness of breath
- 慢性咳嗽
- chest discomfort
- weakness
- fatigue
- weight loss
最终，您的肺可能无法吸收足够的氧气来为您的身体提供所需的含量。这最终会导致呼吸衰竭，heart failure，，，，and other health concerns.
However, IPF progresses slowly, and treatments are available.了解有关IPF警告信号的更多信息。
IPF doesn’t have formal stages, but there are unique periods in the progression of the disease.
首次被诊断出时，您可能不需要氧气帮助。不久之后，您可能会发现在活动期间需要氧气，因为您在步行，园艺或清洁时很难呼吸。
一个s scarring in your lungs becomes severe, you’ll likely need oxygen at all times. That includes when you’re active, at rest, and even sleeping.
In the later stages of IPF, high-flow oxygen machines can be used to develop increased levels of oxygen. When you’re away from home, portable machines can provide continuous oxygen.
IPF逐渐进展。有些人还会经历疾病的耀斑，或者呼吸困难的时期。在这些耀斑期间发生的肺部损伤是不可逆转的，您不太可能恢复爆炸前的任何肺功能。
但是，采取某些行动可以帮助减慢疾病的进展。Check out this list of questions to ask your doctor about IPF progression。
Since the signs and symptoms of IPF develop slowly over time, it’s difficult for doctors to diagnose IPF immediately. Scarring caused by IPF also looks similar to scarring caused by其他肺部疾病。
几项测试可用于确认IPF并排除其他可能的原因。
Tests used to diagnose IPF include:
- 胸部X射线
- high-resolution computer tomography (HRCT) scan
- 运动测试
- lung function tests
- 脉搏血氧仪衡量你的血氧水平
- blood gas test，可以测量您的血液水平和二氧化碳的血液水平
- 肺活检
一个ccording to the Pulmonary Fibrosis Foundation,50,000 new casesof IPF are diagnosed each year in the United States.
Treatment options are available to help you manage and reduce your symptoms.
医疗的主要目标是减少肺部炎症，保护肺组织并减缓肺功能的丧失。这将使您呼吸更轻松。
最常见的治疗选择包括氧疗法and medications. Oxygen therapy helps with breathing, and药物help to control inflammation and reduce lung tissue scarring。
食品药品监督管理局（FDA）批准了两种IPF药物，即Nintedanib（Ofev）和Pirfenidone（Esbriet）。
一个lung transplant也可能需要。因为这是一个重大操作，所以肺移植通常被视为最终治疗步骤。目前，这是IPF的唯一方法。
早期治疗对于被诊断出患有这种肺部状况的人至关重要。Learn more about how treatment can help you manage the symptoms of IPF。
管理IPF的重要部分是学习采用健康的生活方式实践，以减轻症状并改善您的前景和生活质量。这些生活方式的改变包括：
- stopping smoking, if you currently do
- maintaining a moderate weight, which may require weight loss
- staying on top of all vaccines, medications, and vitamins or supplements
- using an oxygen monitor to keep your oxygen saturation (also known as blood oxygen levels) in optimal range
You may also consider joining a pulmonary rehabilitation support group. These groups, which may be arranged by your doctor’s office or a local hospital, connect you with healthcare professionals and possibly other people who are living with IPF.
在一起,你可以学习呼吸和调节exercises as well as techniques for managing stress, anxiety, and the many emotions this diagnosis may cause.
Learning tools for stress management might help prevent worsening symptoms. Support groups can also ease anxiety and feelings of loneliness as you face questions or uncertainty when the disease progresses.
IPF是一种进行性疾病，这意味着它会随着时间的流逝而变得更糟。虽然您可以采取措施管理症状，但您不能完全阻止疤痕和肺部损伤。
For some people, the disease may progress very quickly. For others, it may take many years before breathing problems are so difficult that they require you to use oxygen.
When lung function becomes severely limited, it can trigger serious complications. These include:
- heart failure
- pneumonia
- 肺动脉高压
- pulmonary embolism，，，，or a blood clot in your lungs
IPF引起的硬化肺组织可能导致呼吸衰竭，心力衰竭或其他威胁生命的状况。
感染，心力衰竭或肺栓塞后，现有症状也会突然变得更糟。
移植手术可以帮助改善您的前景。其他治疗方法和生活方式的改变也可以帮助减缓疾病的进展。
Medlineplus说IPF通常会影响50至70岁的人之间的人们。因为它会影响人们以后的生活，所以诊断后的平均预期寿命为3至5年。
如果您拥有IPF，那么您自己的预期寿命将取决于几个因素。这些包括：
- 你的年龄
- your overall health
- 疾病进展的速度
- the intensity of symptoms
Researchers are working to raise money for clinical trials that may ultimately lead to a lifesaving discovery. Until a nonsurgical cure for IPF is found, having a lung transplant can help extend your life.Find out more about life expectancy and IPF.