Diffuse interstitial lung disease Definition
Diffuse interstitial lung disease is a group of lung disorders in which the deep lung tissues become inflamed and then damaged.
Diffuse parenchymal lung disease; Alveolitis; Cryptogenic fibrosing alveolitis (CFA); Idiopathic pulmonary pneumonitis (IPP)
Causes, incidence, and risk factors
The lungs contain tiny air sacs (alveoli), which is where oxygen is absorbed. These air sacs open up or expand with each breath.
The tissue around these air sacs is called the interstitium. In people with interstitial lung disease, this tissue becomes stiff or scarred, and the air sacs are not able to expand as much. As a result, not as much oxygen can get to your body.
Interstitial lung disease (ILD) can occur without a known cause. This is called idiopathic ILD. Idiopathic pulmonary fibrosis (IPF) is the most common 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
Medications (such as nitrofurantoin, sulfonamides, bleomycin, amiodarone, methotrexate, gold, infliximab, and etanercept)
Radiation therapy to the chest
Working with or around asbestos, coal dust, cotton dust, and silica dust (called occupational lung disease)
Cigarette smoking may increase the risk of developing some forms of ILD and may cause the disease to be more severe.
Symptoms Shortness of breath is a main symptom of interstitial lung disease. You may breathe faster or need to take deep breaths.
At first, shortness of breath may not be severe and you may only notice it with exercise, climbing stairs, and other hard activities.
Over time, you may notice it while bathing or dressing, and then while 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, muscle and joint pain, and fatigue are also present.
People with more advanced ILD may have:
Abnormal enlargement of the base of the fingernails ( clubbing)
Blue color of the lips, skin, or fingernails due to low blood oxygen levels ( cyanosis) Signs and tests
The health care provider will perform a physical exam. Dry, crackling breath sounds may be heard when listening to the chest with a stethoscope.
The following tests may be done:
People who are heavily exposed to known causes of lung disease in the workplace are usually routinely screened for lung disease. These jobs include coal mining, sand blasting, and working on a ship.
Treatment depends on the cause of the disease. Drugs that suppress the immune system and reduce swelling in the lungs are prescribed if an autoimmune disease is causing the problem.
If there is no specific treatment for the condition, therapy is aimed at making you more comfortable and supporting
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 patients with advanced ILD may need a lung transplant.
For additional information and resources, see
lung disease support group.
Some patients develop heart failure and high blood pressure in the blood vessels of their lungs.
Your chances of recovering or of the disease getting worse depend on the cause and how severe the disease was when it was first diagnosed.
Idiopathic pulmonary fibrosis has a poor outlook.
Calling your health care provider
Call your health care 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 References
Raghu G. Interstitial lung disease. In: Goldman L, Schafer AI, eds.
Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders; 2011:chap 92.
Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management.
Am J Respir Crit Care Med. 2011;183:788-824.
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.
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