Resumen
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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Síntomas
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
- Tos seca
- Chest discomfort
- Pérdida del apetito
- 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.
Cómo se diagnostica
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.
Análisis de sangre
Blood tests can detect markers of autoimmune diseases or inflammatory responses to environmental exposures that are linked to interstitial lung diseases.
Diagnóstico por imágenes
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.
Pruebas de la función pulmonar
Estas pruebas miden cuánto aire pueden contener sus pulmones, cuánto aire ingresa y sale de sus pulmones, y la capacidad de los pulmones de intercambiar oxígeno. Estas pruebas pueden realizarse para identificar potencialmente la causa. 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.
Cateterismo cardíaco derecho
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. Durante la prueba, los médicos insertan un catéter (un tubo delgado de goma) a través de una vena grande en la ingle o el cuello del paciente. Luego, pasan el catéter hasta el interior del corazón del paciente para medir la presión sanguínea en el lado derecho del corazón y en los pulmones.
Medicamentos
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.
Financiación de su atención
Es posible que haya coordinación de asistencia financiera disponible para ayudar con los costos de sus medicamentos, incluidos programas de los fabricantes y programas comunitarios. Los representantes de Accredo están disponibles para ayudarlo a encontrar un programa que funcione para usted.
Recursos económicos comunitarios
- PSI-Patient Services, Inc. | 800-366-7741
- Fundación para la Defensa del Paciente (Patient Advocate Foundation) | 800-532-5274
- El Fondo de Asistencia (The Assistance Fund) | 855-845-3663
- Fundación Healthwell (Healthwell Foundation) | 800- 675-8416
- Ofev® (nintedanib) Capsules Financial Assistance Information | 855-297-5906
- Asistencia del fabricante United Therapeutics de Tyvaso (treprostinil para inhalación) | 877-864-8437
Orientación financiera
Los medicamentos de especialidad que salvan vidas pueden ser costosos. Conozca en este video cómo los equipos Accredo ayudan a las personas a encontrar formas de costear el medicamento que necesitan para sobrevivir.
Encuentre apoyo
There are many organizations that support research and advocacy for interstitial lung disease. Consulte a continuación algunas de estas organizaciones.
Organizaciones de apoyo
- Asociación Arterial Pulmonar (Pulmonary Arterial Association)
- PHAware
- Pulmonary Fibrosis Foundation
- Instituto Nacional del Corazón, los Pulmones y la Sangre (National Heart, Lung, and Blood Institute, NHLBI)
- Organización Nacional de Trastornos Poco Frecuentes (National Organization of Rare Disorders, NORD)
Organizaciones gubernamentales
Conozca al equipo
Accredo’s Interstitial lung disease (ILD) care team is dedicated to serving you and we understand the complexity of your condition. Nuestros médicos clínicos con formación especializada están disponibles las 24 horas al día, los siete días de la semana, para responder cualquier pregunta.
Por qué lo hacemos
Accredo brinda apoyo a pacientes con afecciones crónicas y complejas, y los ayuda a vivir mejor su vida. Mire nuestro video para conocer por qué hacemos lo que hacemos por nuestros pacientes.