What is idiopathic interstitial pneumonia?

It is a chronic inflammatory interstitial lung disease of unknown cause, characterized by progressive decline in lung function and irreversible respiratory failure, predominantly affecting middle-aged and elderly individuals. Currently, there is no effective treatment, and the mortality rate is high.

What are the causes of idiopathic interstitial pneumonia?

The exact cause of idiopathic interstitial pneumonia remains unclear. Known risk factors associated with the disease include smoking, viral infections, environmental pollution, chronic aspiration, gastroesophageal reflux disease (GERD), genetic predisposition, and certain medications.

Who is most commonly affected by idiopathic interstitial pneumonia?

Commonly affected groups include males aged 50–70 years, long-term smokers, individuals working in mining or construction industries, those with GERD, people with chronic hepatitis C or EBV infection, and those with a family history of idiopathic interstitial pneumonia.

What are the main symptoms of idiopathic interstitial pneumonia?

Patients often experience gradually worsening shortness of breath and dry cough during exertion or activity, which becomes particularly noticeable after walking or exercise. Some may also suffer from fatigue, muscle or joint pain, and unexplained weight loss.

In advanced stages, patients may develop clubbing of fingers and toes. Physical examination often reveals crackles at the lung bases.

How is idiopathic interstitial pneumonia diagnosed?

1. Medical history

Focuses on identifying high-risk factors such as older age, male gender, long-term smoking, occupational exposure, and family history.

2. Clinical manifestations

Include exertional dyspnea, dry cough, fatigue, muscle or joint pain, and clubbing of fingers.

3. Blood tests

Evaluate inflammatory markers in peripheral blood to assess the presence of inflammation.

4. Pulmonary function tests

Help determine the type and severity of lung damage and guide treatment.

5. Imaging studies

Include chest X-rays and high-resolution CT scans. X-rays provide a general assessment of lung abnormalities, while CT scans offer detailed evaluation of lesion extent and characteristic features.

6. Pathological examination

Involves obtaining lung tissue samples via bronchoscopy or bronchoalveolar lavage for microscopic analysis to assess alveolar damage and fibrosis.

How is idiopathic interstitial pneumonia treated?

Current treatments include symptomatic management, medications, and surgery.

• Symptomatic treatment includes smoking cessation, oxygen therapy, mechanical ventilation, and pulmonary rehabilitation.
• Medications aim to reduce lung injury and fibrosis.
• Surgical treatment primarily involves lung transplantation, which carries risks such as rejection and infection, and is limited by organ availability and matching.

What is the prognosis for idiopathic interstitial pneumonia?

Patients experience progressive decline in lung function, with respiratory failure or death occurring approximately 4 years after initial diagnosis on average. The median survival is 2–5 years, which is worse than most malignancies.

The disease severely impacts quality of life, leading to worsening dyspnea, loss of self-care ability, and eventual respiratory failure.

How can idiopathic interstitial pneumonia be prevented?

Since the exact cause is unknown, prevention focuses on managing risk factors, such as quitting smoking, using dust protection in high-risk occupations, treating GERD and viral infections, and maintaining immunity through regular exercise.