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Orphan lung disease definition

Pulmonary vasculitis refers to inflammation of the small blood vessels in the lungs. It is usually part of a wider condition affecting the whole body, resulting in inflammation in the vessels within several organs. It can be treated with corticosteroids, immunosuppressive drugs, and the monoclonal antibody rituximab.

Alveolar haemorrhage syndromes

The main symptoms are coughing up blood and rapidly increasing anaemia (low numbers of red blood cells). Alveolar haemorrhage syndromes are usually diagnosed when blood is found in the fluid taken during a procedure known as bronchoalveolar lavage. There are numerous causes of alveolar haemorrhage syndrome, including rare infectious diseases such as leptospirosis.


Bronchiolitis refers to inflammation of the small airways in the lungs. It can be caused by inhaling toxins, gases and dusts or by lung transplantation. It is also linked with other inflammation disorders such as rheumatoid arthritis and interstitial diseases. It can lead to airway obstruction and is diagnosed by lung function tests and CT scans.

Idiopathic eosinophilic pneumonia

This may occur as a result of taking medication for other conditions or it may be linked to a disease caused by a worm infestation. It results in breathlessness and high levels of eosinophils – a type of white blood cell. It may also be associated with asthma. People often have a dramatic response to the use of corticosteroids to treat the condition, but relapses are very common.

An acute, or short-term, form of the condition can occur, which is similar to adult respiratory distress syndrome. This can improve with or without corticosteroids and often affects people who have just started smoking.

Pulmonary alveolar proteinosis

These disorders are caused by an abnormal immune response from the body when a type of protein builds up in the air sacs (alveoli) of the lungs, making breathing difficult. Symptoms may include cough, wheeze and shortness of breath. It is usually diagnosed when milky fluid is found during a procedure known as bronchoalveolar lavage. This procedure involves squirting fluid into the lungs via a long tube called a bronchoscope, and recollecting it to examine.

Whole-lung lavage is the standard treatment for pulmonary alveolar proteinosis. This involves requires inserting a tube into the lungs, which allows ventilation of one lung while the other lung is repeatedly filled with saline and drained to clean out the material from the air spaces. A further treatment, which involves inhaling a protein called GM-CSF has also become an efficient therapy for this condition.

Idiopathic tracheopathies

This term refers to disorders of the trachea, or windpipe, usually causing a chronic- cough or recurrent lung infections. They are diagnosed by CT scans or endoscopy a procedure involving a thin, long, flexible tube that can be inserted into the airways (bronchoscopy).

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