特发性 肺 纤维化

迹象 & 症状

Symptoms of IPF often appear gradually and include:

• 呼吸急促呼吸急促，尤指在体育活动期间或之后
• Chronic, dry hacking cough
• Crackle sound in the lungs heard through a stethoscope
• Rounding of the fingernails, a condition called clubbing

IPF的症状可能与其他导致肺瘢痕形成的疾病相似, so diagnosing IPF often involves ruling out other conditions. 与你的十大赌博平台排行榜多次访问可能需要最终确定你的诊断和治疗方法.

诊断

诊断特发性肺纤维化需要肺科十大赌博平台排行榜的意见, 放射科十大赌博平台排行榜和, 在很多情况下, 病理学家在评估间质性肺疾病患者方面经验丰富. 为了做出准确的诊断，在这些不同的专家之间进行面对面的讨论往往是必要的.

To determine if you have IPF, 你的十大赌博平台排行榜会首先对你进行全面的病史和体格检查. 病史将包括讨论其他可能与肺瘢痕有关的医疗问题, such as connective tissue diseases, and reviewing any medications you're taking. 体检将包括用听诊器听你的胸部，检查是否有爆裂声，并仔细检查你的皮肤和关节.

In addition to a thorough medical history and physical exam, your doctor may conduct the following 测试s:

• 肺 function 测试 (PFT). 这 测试 包括一系列测量气流的呼吸练习, 肺中的空气量和肺从空气中提取氧气的能力. 这 allows your doctor to assess the function of your lungs.
• High resolution computed tomography (HRCT). 这 is a special type of CT扫描 这为你的十大赌博平台排行榜提供了你肺部的高分辨率图像. Images are taken in several different ways, including lying on your back (supine), 胸部平躺(俯卧)，同时呼气(动态呼气). 这些图像对于确定您是否患有IPF非常有价值. Having an HRCT is very similar to having a regular CT扫描; they both take only a few minutes.
• 血液测试. We may order blood 测试s, also called serologies, to look for evidence of connective tissue diseases such as 类风湿性关节炎 or 硬皮病. 这些结缔组织疾病的一些患者在更典型的体征和症状出现之前就出现了肺部问题.
• Six-minute walk 测试. 这个测试评估你在六分钟内可以行走的距离和血氧饱和度, measured by finger or ear probe, you achieve while walking.
• 支气管镜检查. 在这个 测试, the doctor passes a flexible fiberoptic scope, about the diameter of a pencil, into the lungs to take fluid and tissue samples. 这 测试 doesn't require an overnight stay in the hospital. 目前尚不清楚支气管镜检查是否有助于诊断IPF, but it may be performed in certain circumstances.
• Surgical lung biopsy. Some patients need this 测试 to definitively diagnose IPF. A cardiothoracic surgeon performs the surgical lung biopsy, 通常通过一英寸长的切口使用小工具和相机. 病人 will need to stay in the hospital for a few days.

治疗

两种抗纤维化药物-尼达尼布和吡非尼酮-于2014年秋季被批准用于特发性肺纤维化. While these medications are not a cure, 随着时间的推移，它们都被证明可以减缓肺功能的衰退.

正在进行的其他治疗IPF药物的研究已经显示出初步的希望，但需要更多的研究.

肺康复是一个有组织的锻炼和教育计划，旨在提高耐力, 强度, and exercise capacity, as well as to decrease fatigue and shortness of breath, for people with chronic lung disease, including people with IPF.

另外, 治疗与IPF相关的任何其他医学问题是很重要的, 如胃食管反流病(GERD)和肺动脉高压.

最后，肺移植可能是一些IPF患者的有效治疗选择.