Diseases Treated in the Interstitial Lung Disease Clinic at National Jewish Health (partial list)
The Interstitial lung diseases (ILD) are a collection of more than 150 acute and chronic pulmonary disorders. Scarring or fibrosis of the lungs characterizes them. Basic information on some of the specific types of ILD is offered below.
Autoimmune Related Lung Diseases
BOOP - Bronchiolitis Obliterans Organizing Pneumonitis
Bronchiolitis
Connective Tissue Disease Related Interstitial Lung Disease
DIP - Desquamative Interstitial Pneumonia
HP - Hypersensitivity Pneumonitis
IPF - Idiopathic Pulmonary Fibrosis (Cryptogenic Fibrosing Alveolitis)
FPF - Familial Pulmonary Fibrosis
LAM - Lymphangioleiomyomatosis
LIP - Lymphocytic Interstitial Pneumonia
NSIP - Non-Specific Interstitial Pneumonia
PAP - Pulmonary Alveolar Proteinosis
Pulmonary Langerhans Cell Histiocytosis (Eosinophilic Granuloma)
Pulmonary Vasculitis and Alveolar Hemorrhage Syndromes
Scleroderma
Wegeners Granulomatosis
Autoimmune Related Lung Diseases
The body has an immune system that defends the body by fighting off infection. An autoimmune disease occurs when the immune system malfunctions and attacks the body itself. This can result in inflammation of an organ. If the inflammation occurs in the lungs, an autoimmune related interstitial lung disease may develop. This may occur with rheumatoid arthritis, sarcoidosis, scleroderma, Sjogren’s Syndrome, systemic lupus erythematosus (SLE) and Wegener’s Granulomatosis.
BOOP - Bronchiolitis Obliterans Organizing Pneumonitis
This is an inflammatory process that occurs in the lung as a result of lung injury. This can occur in the small airways and their surrounding tissue. It may involve limited areas in the lung or the whole lung. It is not an infectious process and is not contagious. The cause is not always known but may include radiation, some medications and some of the connective tissue diseases. Flu like symptoms may occur and frequently last less than two months.
Bronchiolitis
This fibrotic lung disease affects small airways in the lung called bronchioles. As a result of injury such as infection, organ transplantation, drug reaction, connective tissue and other diseases or exposure to some types of fume, the lining of these airways are become fibrotic. The exact cause of the injury may be unknown. There are a variety of types of bronchiolitis. Symptoms include may occur slowly or rapidly and include cough or shortness of breath.
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Connective Tissue Related Interstitial Lung Disease
There are many types of connective tissue disease. The whole lung can be involved in these diseases due to the large amount of connective tissue and blood supply in the lungs. Many connective tissue diseases show a dysfunction of the immune system. Some of the connective tissue related interstitial lung diseases include systemic lupus erythematosus, rheumatoid arthritis and scleroderma. Understanding lung involvement in the connective tissue diseases is still under investigation.
DIP- Desquamative Interstitial Pneumonia
This is a chronic inflammatory process in the walls of the alveoli that usually affects smokers in there 40s or 50s, though non-smokers may also be involved. Men are affected twice as frequently as women with symptoms of cough and shortness of breath, possible weight loss and chest pain.
HP- Hypersensitivity Pneumonitis
This condition is the result of repeated inhalations of very fine particles such as animal and bird proteins, fungi, bacteria and chemical proteins. This inhalation causes inflammation of the small airways and other functional parts of the lung. It may result in damage that is not reversible. Only 5-15% of people exposed to high levels of these particles will develop the disease.
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IPF- Idiopathic Pulmonary Fibrosis (Cryptogenic Fibrosing Alveolitis)
The American Thoracic Society has defined IPF as “a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic appearance of usual interstitial pneumonia on surgical (thorascopic or open) lung biopsy.” IPF accounts for up to 45% of all interstitial lung diseases. It occurs more frequently in men with the average age of diagnosis being the mid 60s. The chance of occurrence increases with age. Some potential risk factors for this disease include cigarette smoking, chronic aspiration, exposure to antidepressants, environmental factors, infectious agents and genetics. The prognosis for this condition is poor with the median survival being 2.5-3 yrs. See clinical trials for information on studies relating to IPF being performed at National Jewish Health.
FPF - Familial Pulmonary Fibrosis
When two or more members within the same family have Idiopathic Pulmonary Fibrosis (IPF) or any other form of Idiopathic Interstitial Pneumonia (IIP), it is called Familial Pulmonary Fibrosis (FPF). The most common definition of FPF is two or more primary biological family members (parent, child, or sibling) with the diagnosis of IPF or IIP. However, family members can be second degree as well, for example they can be an aunt or uncle, cousins, etc.
LAM - Lymphangioleiomyomatosis
This rare, multi-system disease may lead to cystic lung disease. The average age of diagnosis is the mid 30s though symptoms are usually present for up to 3 yrs. prior. There is no racial, ethnic or smoking prevalence. Infectious or inflammatory agents due no appear to cause this disease. It is thought that estrogen plays a role in the onset and/or progression on the disease.
LIP-Lymphocytic Interstitial Pneumonia
This disease results when the alveoli are compressed by lymphocytes. The infiltrates can be in a specific area of the lung or spread throughout the lung. The disease is rare in occurrence but may be associated with other diseases. It occurs more frequently in women with the mean age of onset being 56 years old. The most common symptoms of cough and shortness of breath may occur long before diagnosis.
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NSIP-Non-Specific Interstitial Pneumonia
This is a disease of exclusion, which means it is defined by what diseases it is not. The cause of NSIP has not been firmly defined. The rate of occurrence is not known. The mean age of onset is between 39 and 60 years old. Most patients are current or former cigarette smokers. Symptoms occur approximately 11 months prior to diagnosis with cough and shortness of breath being most common. Symptoms of fule, fatigue and weight loss are also seen.
PAP-Pulmonary Alveolar Proteinosis
This group of diseases results when protein material is deposited in the alveolar spaces of the lung. The cause of PAP is usually unknown, it is thought that there may be more then one cause. Some conditions associated with PAP are the result of environmental exposures, tissue, blood and immune disorders. It occurs more frequently in men then women with the mean age at diagnosis being 36. Cough and shortness of breath are the main symptoms, sometimes following an upper respiratory tract infection. 8-30% of patients with PAP will have a spontaneous remission.
Pulmonary Langerhans cell histiocytosis (Eosinophilic Granuloma)
This disease is the result of infiltration of an organ with specific type of cell called a Langerhans cell. The organs usually affected include the skeleton, lungs, central nervous system and skin. The occurrence of this disease is not known. It occurs most commonly in smokers between 30 and 50 years old. 15-30% of patients do not have symptoms when the disease is diagnosed. When symptomatic, dry cough, shortness of breath with exertion, weight loss, fever, night sweats and anorexia are the most common complaints. There may also be symptoms that results from other organ involvement.
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Pulmonary Vasculitis and Alveolar Hemorrhage Syndromes
There are many forms of vasculitis. The lung is one of the many organs that can be involved in vasculitis. The most common types of vasculitis that result in pulmonary disorders are Wegener’s granulomatosis, mictoscopic polyangiitis and Churg-Strauss syndrome. Vasculitis of the lungs may occur secondary to other conditions that result in vasculitis such as systemic lupus erythematosus. When the lung is involved, it is the result of inflammation of the pulmonary blood vessels.
Scleroderma
Also referred to as Progressive Systemic Sclerosis, this rare disease can involve the skin and many other organs of the body. Women are diagnosed more frequently then men though men frequently have more extensive disease. Lung involvement is the 4th most common organ involved after skin, blood vessels and the esophagus. 80% of scleroderma patients have lung involvement. Lung involvement frequently occurs throughout the lung. The most common symptoms include shortness of breath with exertion that may progress to shortness of breath at rest. A dry cough may be present.
Wegener’s Granulomatosis
This disease can affect many organs of the body. It usually affects the upper airways or lung first then may progress to include other organs. This progression does not always occur. In relation to lung involvement, inflammatory lesions may heal and cause scarring. Pulmonary symptoms may include shortness of breath, stridor and pneumonia.
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