肺纤维化是一种肺部结疤（称为纤维化）导致呼吸困难的疾病。这是因为疤痕导致肺部组织变厚变硬，难以将氧气吸收到血液中。特发性肺纤维化 (IPF) 是最常见的诊断类型。

What Is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease where there is scarring of the lungs—called fibrosis—which makes it difficult to breathe. This is because the scarring causes the tissues in the lungs to get thick and stiff and makes it hard to absorb oxygen into the bloodstream. Idiopathic pulmonary fibrosis (IPF) is the most commonly diagnosed type. 肺纤维化是一种肺部结疤（称为纤维化）导致呼吸困难的疾病。这是因为疤痕导致肺部组织变厚变硬，难以将氧气吸收到血液中。特发性肺纤维化 (IPF) 是最常见的诊断类型。

Key Points

• Pulmonary fibrosis is an interstitial lung disease. Interstitial lung diseases are a group of conditions that cause inflammation and scarring around the tiny air sacs (alveoli) in the lungs.

• Inhaling hazardous chemicals can be one cause of pulmonary fibrosis. PF can also be caused by certain diseases, medication and genetics. Most often the cause is unknown. This is called idiopathic pulmonary fibrosis (IPF).

• 肺纤维化是一种间质性肺病。间质性肺病是一组引起肺泡周围炎症和瘢痕形成的病症。
• 吸入危险化学品可能是肺纤维化的原因之一。 PF 也可能由某些疾病、药物和遗传引起。大多数情况下，原因不明。这称为特发性肺纤维化 (IPF)。

Living with Pulmonary fibrosis

• Oxygen therapy 氧疗
• Pulmonary rehabilitation 肺康复
• Clinical trials 临床试验
• Lung transplant 肺移植

Although there is no cure for IPF, there are approved medications to slow the progression. Currently, two drugs are FDA-approved for treatment of idiopathic pulmonary fibrosis (IPF), which is the most common form of PF. These include nintedanib (Ofev®) and pirfenidone (Esbriet®). These medications are called anti-fibrotic agents, meaning that they have shown in clinical trials to slow down the rate of fibrosis or scarring in the lungs. These drugs are approved for patients with mild, moderate and severe IPF. While neither medication is recommended over the other, one may work better for you. Your doctor will work with you to prescribe treatment tailored to your needs. While taking these medications, you will be monitored closely for any serious side effects. Monitoring will include routine blood work to make sure your liver remains healthy. 虽然 IPF 无法治愈，但有批准的药物可以减缓其进展。 目前，FDA 批准了两种药物用于治疗特发性肺纤维化 (IPF)，这是最常见的 PF 形式。这些包括尼达尼布 (Ofev®) 和吡非尼酮 (Esbriet®)。这些药物被称为抗纤维化药物，这意味着它们在临床试验中显示可以减缓肺部纤维化或疤痕形成的速度。这些药物被批准用于轻度、中度和重度 IPF 患者。虽然两种药物都不推荐使用另一种，但一种可能更适合您。您的医生将与您一起制定适合您需求的治疗方案。在服用这些药物时，您将受到密切监测，了解是否有任何严重的副作用。监测将包括常规血液检查，以确保您的肝脏保持健康。

Reference

Pulmonary Fibrosis Medications

Vocabulary

• idiopathic 特发性
• Interstitial 间质性