Pulmonary fibrosis - Vejthani Hospital | JCI Accredited International Hospital in Bangkok, Thailand.

Pulmonary fibrosis

Overview

Pulmonary fibrosis is a progressive lung disease characterized by the scarring and thickening of lung tissue, including the alveoli (air sacs) and connective tissue. This condition deteriorates over time, causing increasing shortness of breath. While some individuals may remain stable for extended periods, others may experience a rapid decline in lung function.

The exact causes of pulmonary fibrosis are often unclear, and when no specific cause is identified, the condition is referred to as idiopathic pulmonary fibrosis. This form of the disease typically affects middle-aged and older adults, although it is rare in children and infants.

Unfortunately, the lung damage from pulmonary fibrosis is irreversible. Treatment options, such as medications and therapies, are aimed at slowing the progression of the disease, alleviating symptoms, and enhancing the quality of life. In certain cases, a lung transplant may be considered.

Symptoms

Pulmonary fibrosis manifests differently in each individual, and its symptoms can often resemble those of common, more readily treatable ailments such as the common cold or an upper respiratory infection.

The symptoms of pulmonary fibrosis may include:

  • Rapid, shallow breathing.
  • Muscle and joint aches.
  • Persistent dry cough.
  • Severe fatigue, regardless of the amount of rest.
  • Shortness of breath, especially during or soon after physical activity.
  • Unexplained and unintended weight loss.

As the condition progresses, additional symptoms may develop, including:

  • Clubbing, characterized by the widening and rounding of the tips of the fingers or toes.
  • Cyanosis, seen as a bluish hue to the skin in fair-skinned people or a gray or white discoloration around the mouth or eyes in darker-skinned individuals, resulting from low oxygen levels in the blood.

Causes

Pulmonary fibrosis results from lung damage caused by various factors such as radiation therapy, certain medications, prolonged exposure to specific toxins, and certain medical conditions. These changes impede the blood’s ability to absorb oxygen. In some cases, the origin of pulmonary fibrosis may be unknown.

  • Work and environment: Pulmonary fibrosis may be caused entirely or in part by your place of work and residence. Wearing protective gear is especially important if you have frequent or continuous contact with toxins or pollutants, which are substances that degrade the quality of water, air, or land. Among the examples are:
    • Asbestos fibers.
    • Bird and animal poop.
    • Dust from wood, coal or grain.
    • Metal dusts.
    • Mold.
    • Silica dust.
  • Radiation treatments: When receiving radiation therapy to the chest for conditions like lung or breast cancer, some patients experience lung damage months or even years following the treatment. The following will determine the severity of the damage:
    • Administered chemotherapy.
    • Duration of exposure to radiation.
    • Overall radiation dose that was provided.
    • Underlying lung disease.
  • Medicines: Many medications might harm the lungs. Among the examples are:
    • Chemotherapy: Lung tissue can be damaged by medications meant to destroy cancer cells, such as bleomycin, cyclophosphamide, and methotrexate.
    • Heart medicines: Lung tissue may be damaged by certain medications used to treat irregular heartbeats, such as amiodarone.
    • Antibiotics: Damage to the lungs can result from antibiotics such nitrofurantoin or ethambutol.
    • Anti-inflammatory medicines: Lung damage can result from taking some anti-inflammatory medications such sulfasalazine or rituximab.
  • Medical conditions: There are also other disorders that can cause damage to the lungs, such as:
    • Dermatomyositis: The skin rash and weakening of the muscles are indications of an inflammatory condition.
    • Lupus: A condition brought on by the immune system of the body attacking its own tissues and organs.
    • Mixed connective tissue disease: Exhibits a variety of symptoms common to several illnesses, including polymyositis, scleroderma, and lupus.
    • Pneumonia: An infection that causes inflammation in one or both of the lungs’ air sacs.
    • Polymyositis: A condition that is inflammatory and results in bilateral muscular weakening.
    • Rheumatoid arthritis: A condition characterized by inflammation that impacts the body’s processes and joints.
    • Sarcoidosis: An inflammatory condition that typically affects the lymph nodes and lungs.
    • Scleroderma: A group of rare diseases that cause internal body issues in addition to skin tightness and hardening.
  • Idiopathic pulmonary fibrosis: Pulmonary fibrosis can be caused by a wide range of medications and conditions. However, the cause is never identified in a great number of cases. Even if the etiology of the condition cannot be determined, risk factors like smoking or exposure to air pollution may be linked to it. Idiopathic pulmonary fibrosis is pulmonary fibrosis without a known etiology.

A common condition in many cases of idiopathic pulmonary fibrosis is gastroesophageal reflux disease, or GERD. When stomach acid refluxes back into the esophagus, it causes this disease. Idiopathic pulmonary fibrosis may be made worse more quickly by GERD, or it may be a risk factor for the condition. However, additional research is required.

Risk factors

The following risk factors that may increase your risk of pulmonary fibrosis includes:

  • Age: Although it is uncommon, pulmonary fibrosis has been observed in newborns and children. The majority of those who get pulmonary fibrosis do so in their later years, typically between the ages of 50 and 70.
  • Cancer treatments: Pulmonary fibrosis can be more likely to occur if you use certain chemotherapy medications or receive radiation treatments to the chest.
  • Gender: More males than women are affected by pulmonary fibrosis. Still, the number of female cases has increased recently.
  • Genetics: Genes may be involved in some forms of pulmonary fibrosis since they run in families.
  • Occupation: If you are employed in construction, farming, or mining, your risk of acquiring pulmonary fibrosis is increased. Additionally, there is an increased risk if you come into constant or frequent contact with contaminants that are known to harm the lungs.
  • Smoking: Compared to those who have never smoked, those who smoke currently or in the past are more likely to develop lung fibrosis. Moreover, those who have emphysema are more vulnerable.