Central & Eastern European ILDs Interstitial Lung Diseases  Patient Network

Central european ILDs Patient network

Severe types of ILDs

Out of the more than 200 types of interstitial lung disease some are more severe than others. A number of types of ILD get worse over time because more and more of the lung tissue is scarring and stiffening. Those diseases are more challenging for patients to live with. They show a progressive fibrosing behavior and are therefore called Progressive Fibrosing ILD (PF-ILD). The following diseases belong to that group:

Autoimmune ILDs

If the body’s own immune system is attacking the lung and therefore causing a patient to develop pulmonary fibrosis, it is called an Autoimmune ILD. There are many conditions that can cause pulmonary fibrosing: Interstitial Pneumonia with Autoimmune Features (IPAF), Rheumatoid Arthritis ILD (RA-ILD), Sjögren’s Syndrome ILD, Systemic Lupus Erythematous ILD, Polymyositis and Dermatomyositis ILD, Polymyositis and Dermatomyositis ILD, Systemic Sclerosis ILD (SSc-ILD) and Other Connective Tissue Disease ILDs. Diagnosing an Autoimmune ILD can be achieved testing a blood sample for certain proteins, antibodies, or other markers of an autoimmune disease.

Hypersensitivity Pneumonitis

This rare immune system disorder affects the lungs and is caused by breathing in dusts or molds. These organic substances cause the immune system of some patients to react much stronger than others. Hypersensitive patients suffer from inflammation of the airspaces and small airways, leading to the development of pulmonary fibrosis if it is not detected early. There are more than 300 known substances that can cause hypersensitivity pneumonitis. Common examples are mold that grows on hay, straw and grain, particles from feathers or droppings of many species of birds or fungus growing in humidifiers, air conditioners and heating systems.


This inflammatory disease causes the immune system to overreact. As a result, clusters of inflamed tissue forms in the body’s organs. Sarcoidosis most commonly affects the lungs, leading to pulmonary fibrosis. While the cause for this disease is still unknown, scientists assume it to have both a genetic and an environmental component. People who work in dusty or moldy environments are at greater risk to develop sarcoidosis.