What Is Idiopathic Pulmonary Fibrosis?
Fibrosis
It was originally thought that Idiopathic Pulmonary Fibrosis was caused by inflammation in the lungs, but this is no longer considered to be the case as anti-inflammatory treatments have little effect on the symptoms of the disease. The term 'idiopathic' actually means 'unknown' and the cause of the disease is as yet unknown, although the symptoms of the disease are sometimes associated with other underlying conditions such as Rheumatoid Arthritis.
Although the cause of the condition is not really understood, there are several factors that are believed to trigger the characteristic inflammation and cell damage in the alveoli tissues of the lung. These include:
1. Viral infections, including Hepatitis C and the Eppstein-Barr virus 2. Environmental pollutants and cigarette smoking 3. Acid reflux disease 4. Genetic predisposition
What are the symptoms of Idiopathic Pulmonary Fibrosis?
Symptoms of Idiopathic Pulmonary Fibrosis usually develop gradually over a long period of time. However, it is not uncommon for a sudden worsening of symptoms to occur, often followed by minor improvements for short periods. The main symptoms of the disease are:
1. Breathlessness - reduced lung efficiency leads to poor oxygenation of the blood and the patient may begin to experience an acute shortage of breath during any kind of physical exertion. 2. Dry cough 3. Increased risk of chest infections 4. 'Clubbing' - swellings at the base of finger or toe nails that are common in patients with heart and lung disease 5. Unexplained tiredness
Chest X-rays will show scarring in the lungs indicative of the disease and if a doctor listens to the patient's chest using a stethoscope, he will be able to hear tell-tale crackling sounds. In order to make a firm diagnosis and rule out any other conditions, a special CT scan and lung biopsy will be carried out so a detailed examination of the lung tissue and alveoli cells can be made.
What is the prognosis for a patient with Idiopathic Pulmonary Fibrosis?
There is currently no cure for Idiopathic Pulmonary Fibrosis, but the symptoms can be improved using steroid medications, immunosuppressant's, and in the later stages of the disease, oxygen treatment. Patients can also help themselves by stopping smoking (if applicable), taking regular exercise and eating a healthy diet.
Not all patients respond very well to treatment and many continue to worsen. In acute cases of the disease (and if a suitable donor is available), a patient may even be offered a lung transplant, although this is fairly rare.
No Answers, No Cure: Cystic Fibrosis
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