Early symptoms of IPF

symptoms ipf

The early symptoms of IPF (idiopathic pulmonary fibrosis) may not be perceived at first due to their gradual development. Moreover, they may not be interpreted as a symptom at all. For example, one of the main and earliest signs of IPF is shortness of breath. However, this symptom may appear in the beginning as part of an activity in which it might be expected, such as exercise. As the disease advances and becomes more serious, shortness of breath will occur even at rest. Another telling sign is a dry, hacking cough that doesn’t improve but actually worsens to the point that the patient will experience uncontrollable fits of coughing.

Other symptoms of idiopathic pulmonary fibrosis are:

·         Fatigue.

·         Malaise.

·         Weight loss.

·         Muscle and joint pain.

·         Rapid and shallow breathing.

·         Clubbing of fingertips (enlargement, widening and rounding of the tips of fingers or toes).

Farmers, ranchers, hairdressers, stone cutters/polishers, metal workers, miners, and other individuals whose work exposes them to dust or fumes such as silica dust, asbestos fibers, grain dust, and bird and animal droppings, are at a slightly higher risk of experiencing these symptoms. Other risk factors include:

·         Smoking.

·         Age and gender (men between the ages of 40 and 70 are more commonly diagnosed with IPF).

·         Taking nitrofurantoin, amiodarone, methotrexate, bleomycin, and other drugs that cause IPF in some people.

·         Cancer radiation treatments to the chest.

·         Viral infections like Epstein-Barr virus, influenza A virus, hepatitis C virus, HIV, and herpes virus 6.

·         Other conditions like tuberculosis, pneumonia, systemic lupus erythematosus, sarcoidosis, and scleroderma.

Additionally, idiopathic pulmonary fibrosis can be genetically inherited. Furthermore, there may be a link between IPF and GERD (grastroesophageal reflux disease). Some researchers believe that people with GERD may breathe small droplets of acid from their stomachs which can damage their lungs and contribute to idiopathic pulmonary fibrosis. More often than not, though, the cause of IPF is not clearly established.

Just like there are several early symptoms of IPF, there are also a number of tests that can help to diagnose this condition.


IPF diagnostic tests

Imaging tests. These types of testing discards other conditions, and help provide early and sometimes definitive diagnosis, as well as show the extent of damage.

·         Chest x-ray. Shows the scar tissue characteristic of IPF, though some patients may have normal x-rays and require further tests.

·         Computerized tomography. Also known as CT scan, it is more detailed than an x-ray and may help identify specific types of fibrosis.

·         Echocardiogram. A heart sonogram that can assess the amount of pressure in the right side of the heart.


Lung function tests. They use a device such as a spirometer, pulse oximeter, or treadmill to gauge breathing capacity.

·         Spirometry. Performed with a device known as a spirometer, it measures how much air the patient can inhale and exhale.

·         Pulse oximetry. Employs a pulse oximeter to assess blood oxygen saturation levels.

·         Exercise stress test. Monitors lung function during physical activity using a stationary bike or a treadmill.


Lung biopsy. Lung tissue samples are collected and examined to measure the condition’s activity, progression, and response to treatment.

·         Bronchoscopy. A thin, flexible tube with a tiny camera on top is inserted through the nose or mouth, down the throat, and into the airways, followed by a forceps to collect tissue samples.

·         Bronchoalveolar lavage. During the above procedure, a saline solution may be injected into and suctioned out of the lungs to retrieve air sac cells.

·         Surgical biopsy. An invasive and complicated procedure but the only one that can produce a large enough sample for precise diagnosis.

·         Thoracoscopy. Similar to bronchoscopy only the tube is inserted through small incisions between the ribs.

·         Thoracotomy. Small tissue samples are removed through a cut in the chest all between the ribs.



Other diagnostic tests include urinalysis, full blood count, differential blood count, urea measurement, electrolytes, creatinine levels, liver function tests, autoantibody tests, arterial blood gas tests, and skin tests for tuberculosis.


Diagnosing IPF and determining its progression can influence the type of treatment. For instance, some people with idiopathic pulmonary fibrosis receive oxygen therapy only when they sleep or exercise, while others receive it 24/7, meaning they are either confined to a single place or have to carry an oxygen cylinder wherever they go. The benefits of oxygen therapy include:

·         Allowing the patient to breathe easier and exercise.

·         Decreasing blood pressure in the right side of the heart.

·         Improving sleep and wellbeing.

Oxygen therapy can also prevent or alleviate the complications of IPF, including:

·         Pulmonary hypertension.

·         Respiratory failure.

·         Lung cancer.


Medical treatment for idiopathic pulmonary fibrosis is complemented with pulmonary rehabilitation, a set of guidelines aimed at enhancing the quality of life of people with IPF. Pulmonary rehab involves:

·         Physical exercise to increase endurance.

·         Breathing techniques to increase lung efficiency.

·         Nutritional advice.

·         Techniques to conserve energy.

·         Education on lung condition and management.

·         Additional support and counseling.

The last resort treatment for idiopathic pulmonary fibrosis is a lung transplant surgery. Such a procedure has the potential of considerably improving quality and length of life, but there is more demand for lungs than there is offer, though the FDA recently approved a device that can preserve lungs for transplantation.

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