概述
What is interstitial lung disease
Interstitial lung diseases (ILDs) are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. They are classified as restrictive lung diseases, meaning the lungs have trouble expanding upon exhale. There are several subsets of ILD, including but not limited to idiopathic interstitial pneumonias (IIP) such as Idiopathic Pulmonary Fibrosis, exposure-related ILD, and CTD-related interstitial lung diseases such as scleroderma, rheumatoid arthritis, and sjogren’s syndrome. World health organization group 3 includes ILD associated with pulmonary hypertension (PH).
How common is interstitial lung disease
The estimated incidence of ILD is 30 per 100,000 per year, with approximately one-third in the IPF category; the estimated incidence was slightly higher for men than women.1 Incidence of specific ILDs is dependent on the ILD itself. Idiopathic Pulmonary Fibrosis newly occurs in approximately 12 per 100,000 people per year.2
Questions regarding your interstitial lung disease therapy
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症状
What are the symptoms of interstitial lung disease
The symptoms for all types of interstitial lung diseases may be similar, and symptoms are usually more severe as the disease progresses. Symptoms of ILD may include:
- Exertional dyspnea
- Labored breathing
- 干咳
- Chest discomfort
- 食欲不振
- Weight loss (occasionally)
- Bleeding in the lungs
As the disease state progresses, these symptoms will likely intensify in severity. Patients with pulmonary hypertension (PH) associated with an ILD may also experience:
- Lower extremity edema
- Difficulty breathing when lying flat
- Pre-syncope / syncope (fainting)
Additional symptoms are possible based on specific ILD diagnosis.
如何诊断
The diagnosis of interstitial lung disease is challenging as numerous disorders fall under the broad category of ILD. Signs and symptoms can be very similar across the different disorders under ILD. Additionally, other disease states can display signs and symptoms similar to ILD, therefore ruling out other disorders is essential before making a diagnosis.
验血
Blood tests can detect markers of autoimmune diseases or inflammatory responses to environmental exposures that are linked to interstitial lung diseases.
影像检查
CT scans and Echocardiograms are imaging tests that are important in narrowing down the diagnosis of ILD and potentially the more specific ILD in which the patient should be diagnosed. The ability to show fibrosis and details behind the severity of scarring is helpful in determining the treatment regimen and medication(s) the patient should take to assist with their diagnosis.
肺功能检查
这些检查会测量肺部可以容纳的空气量、进出肺部的空气量以及肺部交换氧气的能力。执行这些检查可能会确定其原因。Specific pulmonary function tests include Spirometry and diffusion capacity and Oximetry.
Lung tissue analysis
Tissue samples of a patient’s lung can be helpful in determining diagnosis of interstitial lung disease. Bronchoscopy, bronchoalveolar lavage, and surgical biopsy are particular ways in which tissue samples can be collected.
High resolution computerized tomography
This imaging test is key to, and sometimes the first step in, the diagnosis of interstitial lung disease. CT scanners use a computer to combine X-ray images taken from many different angles to produce cross-sectional images of internal structures. A high-resolution CT scan can be particularly helpful in determining the extent of lung damage caused by interstitial lung disease. It can show details of the fibrosis, which can be helpful in narrowing down the diagnosis and in guiding treatment decisions.
右心导管检查
If the results of initial tests point to PH-associated with a patient’s ILD, your doctor will schedule a right-heart catheterization (commonly referred to as a “right heart cath”). Right-heart catheterization is one of the most accurate and useful tests to get a definitive diagnosis for pulmonary hypertension. This is the only test that directly measures the pressure inside the pulmonary arteries, and it should be done in all patients at least once to confirm a patient’s diagnosis of pulmonary hypertension. 在测试过程中，医生通过患者腹股沟或颈部大静脉插入导管（细橡胶管）。然后将导管送入患者心脏，测量右侧心肺的血压。
药品
Interstitial Lung Disease (PH-associated) Medications
While there is currently no cure for ILD-PH, different treatment options are available to help you manage your disease and feel better day-to-day depending on your type of ILD associated with PH. Your doctor may prescribe other medication(s) as part of your treatment based on your particular ILD diagnosis. The following specialty medication is available at Accredo for the treatment of ILD-PH.
节约护理费用
您还可获得经济援助协调服务，以承担您的部分药品费用，包括生产商和社区计划。Accredo 代表可以帮助您找到适合您的计划。
社区财政资源
- PSI-Patient Services, Inc. | 800-366-7741
- 患者权益基金会 | 800-532-5274
- 援助基金 | 855-845-3663
- Healthwell 基金会 | 800- 675-8416
- Ofev® (nintedanib) Capsules Financial Assistance Information | 855-297-5906
- Tyvaso（吸入曲前列环素）United Therapeutics 生产商援助 | 877-864-8437
财政指导
急救性质的特殊药品很昂贵。通过此视频，了解 Accredo 团队如何帮助个人找到可以负担他们救命药品费用的方式。
查找支持
There are many organizations that support research and advocacy for interstitial lung disease. 下面是其中部分组织。
支持组织
政府组织
团队介绍
Accredo’s Interstitial lung disease (ILD) care team is dedicated to serving you and we understand the complexity of your condition. 我们的特殊培训临床医师每周 7 天、每天 24 小时为您提供服务，以回答您的任何问题。
我们为什么要这样做
Accredo 为慢性和复杂病症患者提供支持，并帮助他们过上最好的生活。请观看我们的视频，了解我们为什么为患者尽心尽力。