Types of ILD
Your doctor will conduct diagnostic tests to determine the specific type of ILD you have to help guide treatment. Common types of ILD include:
Acute Interstitial Pneumonia
Acute interstitial pneumonia (called AIP or Hamman-Rich syndrome) is a form of ILD that involves widespread damage to the alveoli. This injury to the lungs can result in a severe, sudden onset of symptoms, which can progress to respiratory failure. AIP is characterized by a single lesion, which indicates that the lung injury occurred during one particular incident.
Chronic Eosinophilic Pneumonia
Chronic eosinophilic pneumonia (CEP) is caused by an accumulation of eosinophils (a type of white blood cell) in the lungs. This accumulation of white blood cells is usually caused by the body’s response to inflammation caused by allergens or infection. CEP is an example of idiopathic lung disease, meaning that its exact cause is unknown.
Connective tissue and autoimmune disease-related ILD (CTD-ILD)
Patients with connective tissue and autoimmune diseases, such as scleroderma , rheumatoid arthritis or Sjogren’s syndrome , can develop ILD as a result of their condition. Diseases that cause system-wide inflammation can affect the lungs, leading to the scarring that produces ILD.
Cryptogenic organizing pneumonia
Cryptogenic organizing pneumonia produces inflammation in the bronchioles, the alveoli, and the walls of the small bronchi. It is typically a result of an injury to the alveolar wall, although the exact cause is not always known (hence the term “cryptogenic.”
Drug-induced Lung Disease
Bad reactions to medications can sometimes lead to inflammation and scarring in the lungs. When this is the cause of ILD, it’s known as drug-induced lung disease or DILD. Unfortunately, the long-term risks for developing DILD is often something that’s understood only after drugs have been authorized for use.
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) or Familial Interstitial Pneumonia (FIP). 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.
Hypersensitivity Pneumonitis
Hypersensitivity pneumonitis (HP) is an inflammation of the lungs caused by inhaling a foreign substance.
These foreign substances can stimulate an immune reaction in the small airways and air sacs of the lung. This may lead to acute lung disease. Over time and with continued exposure to the causative substance, long-lasting (chronic) lung disease with permanent scarring can occur.
Idiopathic Interstitial Pneumonia
Idiopathic Interstitial Pneumonia (IIP) is a form of pulmonary fibrosis and a subgroup of Interstitial Lung Disease.
When the lung is injured the body responds by healing the wound. Abnormal wound healing can lead to the development of excess scar tissue (fibrosis) and interstitial lung disease.
Idiopathic pulmonary fibrosis, or IPF, is a specific form of idiopathic interstitial pneumonia. It is one of the most common types of ILD and is a progressive scarring of the lung over time.
Lymphangioleiomyomatosis (LAM)
Lymphangioleiomyomatosis (pronounced lim-fan-gee-o-ly-o-my-o-ma-to-sis), or LAM, is a rare disease found almost exclusively in women of all races and ethnic backgrounds.
Pulmonary Langerhans Cell Histiocytosis
Pulmonary Langerhans cell histiocytosis a form of ILD that results from the spread of Langerhans cells (an immune cell) in the lungs. It occurs most often in younger smokers, primarily affecting people aged 20-30 years. When Langerhans cells form granulomas (clusters of white blood cells and tissue), they can damage the bronchioles.
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Sarcoidosis
Sarcoidosis (pronounced SAR-COY-DOE-SIS) is an inflammatory disease characterized by the formation of granulomas—tiny clumps of inflammatory cells—in one or more organs of the body. When the immune system goes into overdrive and too many of these clumps form, they can interfere with an organ’s structure and function. When left unchecked, chronic inflammation can lead to fibrosis, which is the permanent scarring of organ tissue. This disorder affects the lungs in approximately 90% of cases
Smoking-Related ILD
As the name suggests, smoking-related ILD describes ILD that results from cigarette and other inhaled nicotine product users. There are several different types of ILD that can be caused by smoking, including pulmonary Langerhans cell histiocytosis and desquamative interstitial pneumonitis. Smoking cessation [LINK] can greatly reduce the risk of further complications.