Interstitial Lung DiseaseSymptoms, Doctors, Treatments, Advances & More
Learn About Interstitial Lung Disease
Interstitial lung disease (ILD) is a group of lung disorders in which the lung tissues become inflamed and then damaged.
Diffuse parenchymal lung disease; Alveolitis; Idiopathic pulmonary pneumonitis (IPP)
The lungs contain tiny air sacs (alveoli), which is where oxygen is absorbed. These air sacs expand with each breath.
The tissue around these air sacs is called the interstitium. In people with interstitial lung disease, this tissue becomes inflamed, stiff, or scarred, and the air sacs are not able to expand as much. As a result, not as much oxygen can get to the body.
ILD can occur without a known cause. This is called idiopathic ILD. Idiopathic pulmonary fibrosis (IPF) is the most common disease of this type.
There are also dozens of known causes of ILD, including:
- Autoimmune diseases (in which the immune system attacks the body) such as lupus, rheumatoid arthritis, sarcoidosis, and scleroderma.
- Lung inflammation due to breathing in a foreign substance such as certain types of dust, fungus, or mold (hypersensitivity pneumonitis).
- Medicines (such as nitrofurantoin, sulfonamides, bleomycin, amiodarone, methotrexate, gold, infliximab, etanercept, and some chemotherapy medicines).
- Radiation treatment to the chest.
- Working with or around asbestos, coal dust, cotton dust, and silica dust (called occupational lung disease).
- Infection and partial recovery from diseases like COVID-19.
Cigarette smoking may increase the risk of developing some forms of ILD and may cause the disease to be more severe.
Shortness of breath is a main symptom of ILD. You may breathe faster or need to take deep breaths:
- At first, shortness of breath may not be severe and is only noticed with exercise, climbing stairs, and other activities.
- Over time, it can occur with less strenuous activity such as bathing or dressing, and as the disease worsens, even with eating or talking.
Most people with this condition also have a dry cough. A dry cough means you do not cough up any mucus or sputum.
Over time, weight loss, fatigue, and muscle and joint pain are also present.
People with more advanced ILD may have:
- Abnormal enlargement and curving of the base of the fingernails (clubbing).
- Blue color of the lips, skin, or fingernails due to low blood oxygen levels (cyanosis).
- Symptoms of other diseases such as arthritis or trouble swallowing (scleroderma), associated with ILD.
Treatment depends on the cause and duration of the disease. Medicines that suppress the immune system and reduce swelling in the lungs are prescribed if an autoimmune disease is causing the problem. For some people who have IPF or certain other autoimmune diseases, pirfenidone and nintedanib are two medicines that may be used to slow worsening of the disease. If there is no specific treatment for the condition, the aim is to make you more comfortable and support lung function:
- If you smoke, ask your provider about how to stop smoking.
- People with low blood oxygen levels will receive oxygen therapy in their home. A respiratory therapist will help you set up the oxygen. Families need to learn proper oxygen storage and safety.
Lung rehabilitation can provide support, and help you learn:
- Different breathing methods
- How to set up your home to save energy
- How to eat enough calories and nutrients
- How to stay active and strong
Some people with advanced ILD may be candidates for a lung transplant.
Johns Hopkins Bayview Medical Center
Dr. Danoff is an Assistant Professor in the Division of Pulmonary and Critical Care Medicine and Co-Director of the Hopkins Interstitial Lung Disease Clinic. She is a specialist in diseases causing fibrosis or scarring in the lung, particularly those associated with autoimmune diseases including myositis. Dr. Danoff is rated as an Elite provider by MediFind in the treatment of Interstitial Lung Disease. She is also highly rated in 13 other conditions, according to our data. Her clinical expertise encompasses Interstitial Lung Disease, Acute Interstitial Pneumonia, Pulmonary Fibrosis, Lung Transplant, and Endoscopy. Dr. Danoff is board certified in American Board Of Internal Medicine.
UPMC Comprehensive Lung Center - Simmons Center
Kevin Gibson, MD, specializes in interstitial lung disease and is board-certified in pulmonary disease and internal medicine by the American Board of Internal Medicine. He practices at UPMC Comprehensive Lung Center - Simmons Center and is affiliated with University of Pittsburgh Physicians, Department of Medicine and UPMC Presbyterian. Dr. Gibson completed his medical degree at University of Medicine and Dentistry of New Jersey, residency at Emory University School of Medicine, and fellowship at the University of Pittsburgh School of Medicine.Dr. Gibson specializes in treating patients with idiopathic pulmonary fibrosis, sarcoidosis, autoimmune lung disease, and occupational lung disease. Dr. Gibson is rated as an Elite provider by MediFind in the treatment of Interstitial Lung Disease. He is also highly rated in 11 other conditions, according to our data. His clinical expertise encompasses Pulmonary Fibrosis, Idiopathic Pulmonary Fibrosis, Acute Interstitial Pneumonia, Interstitial Lung Disease, and Lung Transplant. Dr. Gibson is board certified in American Board Of Internal Medicine and American Board Of Internal Medicine. Dr. Gibson is currently accepting new patients.
Baylor College Of Medicine - Pulmonary
Ivan Rosas, MD, is dedicated to delivering high-quality, compassionate care to Houston and nearby communities. Ivan Rosas specializes in Pulmonary Disease and works at BCM - Faculty Group Practice. To make an appointment, please call (713) 798-2400. To review all accepted insurance carriers, please visit: https://www.stlukeshealth.org/patients-visitors/patients/billing-insurance/insurances-accepted. Dr. Rosas is rated as an Elite provider by MediFind in the treatment of Interstitial Lung Disease. He is also highly rated in 13 other conditions, according to our data. His clinical expertise encompasses Pulmonary Fibrosis, Acute Interstitial Pneumonia, Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis, and Lung Transplant. Dr. Rosas is currently accepting new patients.
You can ease the stress of illness by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone.
The chance of recovering or ILD getting worse depends on the cause and how severe the disease was when it was first diagnosed.
Some people with ILD develop heart failure and high blood pressure in the blood vessels of their lungs (pulmonary hypertension).
Idiopathic pulmonary fibrosis has a poor outlook.
Contact your provider if:
- Your breathing is getting harder, faster, or more shallow than before
- You cannot get a deep breath, or need to lean forward when sitting
- You are having headaches more often
- You feel sleepy or confused
- You have a fever
- You are coughing up dark mucus
- Your fingertips or the skin around your fingernails is blue
Summary: This study is a prospective observational study for subjects with idiopathic pulmonary fibrosis (IPF) or non-IPF interstitial lung diseases (ILD). The purpose of this study is to compare whether imaging patterns from high-resolution computed tomography (HRCT) at baseline can predict worsening. Single Time point Prediction (STP) is a score derived from an artificial intelligenc/ machine learning (A...
Summary: This is a Ph 2, randomized, double-blind, placebo-controlled global multicenter study to evaluate the efficacy, safety, tolerability, and pharmacokinetics (PK) of PIPE-791 in subjects with a diagnosis of Idiopathic Pulmonary Fibrosis (IPF) with or without background treatment.
Published Date: October 14, 2025
Published By: Andrew Schriber, MD, FCCP, Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, New Jersey. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Corte TJ, Wells AU. Connective tissue diseases. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 92.
Graney BA, Lee JS, King TE. Nonspecific interstitial pneumonitis and other idiopathic interstitial pneumonias. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 90.
Raghu G, Martinez FJ. Interstitial lung disease. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 80.