Medical

Everything about Idiopathic Pulmonary Fibrosis

IPF is a rare and progressive lung disease with unknown origins. Understand more about the disease, how it manifests and how to manage it effectively.

By URLife Team

23 Feb 2024

Idiopathic pulmonary fibrosis is a rare interstitial lung disease that causes scar tissues to grow inside your lungs and affect the respiratory system. This condition develops when the lung tissue becomes thick and stiff for unknown reasons. The symptoms may develop immediately or may take years to reflect. Pulmonary fibrosis usually thickens the lung tissue and impacts the connecting tissue in the lung and alveoli (air sacs inside the lungs). Why it occurs and what causes IPF is still a mystery amongst Doctors. The word idiopathic means ‘unexplained’ and in medical terms, it is used when doctors can’t get to the cause of your condition.

According to a 2023 study published in The Lancet, the median age of onset is approximately 60 years of age and its incidence and prevalence increase dramatically after the sixth decade of life.

The progression of IPF varies among individuals, with scarring that may happen slowly or quickly. Some people maintain a stable condition for years, while others experience rapid deterioration. Acute exacerbations can also occur, intensifying symptoms suddenly. Complications such as pulmonary hypertension and respiratory failure may arise, limiting oxygen delivery to organs.

While there's no cure for IPF, certain treatments can slow its progression and enhance lung function, potentially extending lifespan and improving the quality of life for those with the disease.

The prevalence rate of IPF is 13 to 20 per 1,00,000 people worldwide. Also, as per the 2020 reports familial pulmonary fibrosis is less common than the sporadic form of the disease. Only a small percentage of cases of idiopathic pulmonary fibrosis appear to run in families.

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Symptoms of IPF

Chest pain or tightness
Persistent dry cough
Difficulty in breathing while walking or performing basic chores
Rapid, shallow breathing
Leg swelling
Loss of appetite
Chronic fatigue
Joint and muscle aches
Weight loss
Clubbing (when the tips of your fingers and toes get wider)
Cyanosis (bluish skin in fair-skinned people or grey or white skin around the mouth or eyes in dark-skinned people)

Risk Factors Affecting IPF

While certain factors like smoking can heighten the likelihood of developing pulmonary fibrosis, having these risk factors doesn't guarantee the onset of the disease.
Other risk factors include:

Older age: Pulmonary fibrosis is commonly observed in the second half of life, typically between ages 50 and 70.
Male biological sex: More men than women are affected by pulmonary fibrosis, although cases in women have been increasing in recent years.
Smoking: The risk of pulmonary fibrosis is elevated among individuals who smoke cigarettes.
Occupational exposure: Working in environments with dust or fumes, where regular inhalation of chemicals or hazardous substances occurs, can damage the lungs. Occupations such as farming, ranching, hairdressing, stone cutting/polishing, and metalworking may pose an increased risk.
Certain infections: Infections like flu, Epstein-Barr virus, hepatitis, and herpes can increase the risk of IPF, though they are not direct causes. People with IPF may also have acid reflux, and it's suspected that breathing in tiny drops of stomach acid into the lungs over time may cause damage, though it's not clear if one condition directly causes the other.

Diagnosis of Idiopathic Pulmonary Fibrosis

To diagnose IPF, your doctor may recommend several tests and procedures depending on your family history and the severity of your symptoms.

Chest Scans

A high-resolution chest CT scan (HRCT) is commonly used to capture detailed images of your lungs, identifying signs of scarring or inflammation. CT scans aid in distinguishing between different lung diseases, with IPF typically exhibiting a distinctive honeycomb pattern.

Lung Biopsy

A lung biopsy may be performed to examine lung tissue for indications of inflammation, scarring, or other alterations. This procedure, often conducted through thoracoscopic surgery involving small incisions and a camera-guided approach, can help confirm the diagnosis of IPF.

Chest X-Ray

Additionally, a chest X-ray may be employed to capture images of the lungs and detect evidence of inflammation or damage, including scarring. While X-rays provide a general view, they offer less detail compared to high-resolution CT scans.

Breathing Tests

Pulmonary function tests, also known as lung function tests, involve various devices to assess how well your lungs function and their capacity.

Oxygen Desaturation Test

An oxygen desaturation study measures blood oxygen levels in your blood. In this, the patient is asked to walk for six minutes with a probe attached to your finger or forehead.

Idiopathic Pulmonary Fibrosis Treatment

While there is no treatment that can reverse or cure the condition, they can slow down the progression of the disease and can make it easier for you to breathe. The treatment might include a combination of medication, oxygen therapy and pulmonary rehabilitation.

Medication

There are two approved drugs, nintedanib (Ofev) and pirfenidone (Esbriet), for treating IPF. They work by blocking a lung scarring process, helping to slow down IPF progression and improve breathing. Additional medications like corticosteroids (e.g., prednisone), immune system-slowing drugs and the antioxidant N-acetylcysteine (NAC or Mucomyst) may be prescribed.

Oxygen Therapy

For individuals with advanced IPF or low blood oxygen levels, oxygen therapy is often recommended. This involves providing supplemental oxygen to ensure the body receives an adequate supply, relieving symptoms and enhancing overall well-being.

Pulmonary Rehabilitation

Pulmonary rehabilitation programs play a crucial role in managing IPF. These programs include a combination of exercise, education, and support to improve physical conditioning, enhance breathing techniques, and promote overall health and quality of life for individuals with IPF.

Lung Transplant

In severe cases where conservative treatments are insufficient, lung transplantation may be considered. Lung transplant surgery involves replacing the damaged lungs with a healthy donor lung, providing a potential cure for advanced stages of IPF. However, this option is reserved for carefully selected candidates due to its complexities and associated risks.

Idiopathic pulmonary fibrosis is a lung problem that causes scarring and breathing issues. With treatments ranging from medication to advanced interventions like lung transplantation, managing IPF requires a multifaceted approach to enhance patients' quality of life.

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Regular health checks are essential for everyone, but they are particularly important for individuals who are at risk of or already have lung conditions. Taking regular health checks can help detect the condition at an early stage when it is easier to manage and treat. With the UR.Life HRA, we help you to invest in your well-being through seamless interventions and targeted medical treatments. Our holistic wellness approach caters to all aspects of your well-being. We ensure that you can bring your whole self to work.

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