Normal interstitial pneumonia is a lung disease that causes inflammation and scarring of lung tissue, leading to respiratory failure. Symptoms include shortness of breath and a dry cough. Treatment includes corticosteroids and oxygen therapy, but a lung transplant may be necessary. The cause is often unknown, but may be related to smoking, genetic disorders, or exposure to chemicals. Diagnosis is made through chest X-rays and lung tissue biopsy.
Normal interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred. The condition tends to progress rapidly, causing irreparable damage to the lungs in as little as a year from the onset of symptoms and eventually leading to respiratory failure. Doctors may try treating normal interstitial pneumonia with corticosteroids to relieve inflammation, although persistent disease spreads so rapidly that a lung transplant is often needed to prevent life-threatening complications.
The lungs are filled with tiny sacs of air, called alveoli, which expand and contract to oxygenate the blood flow. The alveoli are lined and protected by a membranous tissue called the interstitium. Normal interstitial pneumonia begins when the alveoli become inflamed, causing the interstitium to harden. The interstitium further tightens as the disease progresses, eventually leading to scarring and significantly reducing the lungs’ ability to oxygenate the blood.
Symptoms are usually not apparent until the disease is past its early stages. As interstitial pneumonia progresses, an individual is likely to experience shortness of breath and a dry cough that gets worse after engaging in physical activity. The disease can eventually become so severe that breathing becomes very difficult even when a person is at rest.
Most cases of usual interstitial pneumonitis are idiopathic, which means that doctors cannot determine why the inflammation and scarring occur. Some cases may be related to underlying causes, such as smoking, genetic connective tissue disorders, or long-term exposure to industrial chemicals and silica. The condition is slightly more common in men than in women, and most patients diagnosed with this condition are over the age of 60.
A doctor can diagnose normal interstitial pneumonia by taking chest X-rays to look for signs of scarring. When signs of lung disease are present, the doctor usually collects a blood sample to check for traces of toxic chemicals, bacteria, and other abnormalities. A biopsy of lung tissue may be needed to confirm the diagnosis and rule out other forms of lung disease, including cancer.
Once a diagnosis has been made, a patient who has mild symptoms is typically given an oxygen machine, prescribed corticosteroids, and instructed to limit their physical activity. A severe case generally requires immediate hospitalization and oxygen therapy to prevent total respiratory failure. The condition cannot be cured and even with intensive medical treatment, patients tend to get worse over time. The most effective treatment for normal interstitial pneumonia is a lung transplant when a donor is available.
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