From the Guidelines
Interstitial thickening is most commonly caused by interstitial lung diseases, such as idiopathic pulmonary fibrosis, as well as connective tissue diseases, and can be diagnosed through a combination of imaging findings, clinical presentation, and pulmonary function tests, as recommended by the most recent guidelines 1.
Causes of Interstitial Thickening
The causes of interstitial thickening can be varied and include:
- Interstitial lung diseases, such as idiopathic pulmonary fibrosis, desquamative interstitial pneumonia, and respiratory bronchiolitis-associated interstitial lung disease, as described in the American Thoracic Society guidelines 1
- Connective tissue diseases, such as systemic sclerosis, rheumatoid arthritis, and systemic lupus erythematosus, which can cause interstitial lung disease, as discussed in the Portuguese Pulmonology Society guidelines 1
- Other conditions, such as pulmonary edema, sarcoidosis, and hypersensitivity pneumonitis, which can also cause interstitial thickening
Diagnosis of Interstitial Thickening
Diagnosis of interstitial thickening typically requires:
- Correlation of imaging findings, such as chest X-rays or CT scans, with clinical presentation and pulmonary function tests, as recommended by the American Thoracic Society guidelines 1
- Sometimes, lung biopsy may be necessary to confirm the diagnosis, as discussed in the European Respiratory Society guidelines 1
- The pattern of interstitial thickening, whether smooth, nodular, or reticular, and its distribution, whether upper or lower lung predominance, can provide important diagnostic clues to the specific underlying condition, as described in the Autoimmunity Reviews article 1
Treatment of Interstitial Thickening
Treatment of interstitial thickening depends on the underlying cause, and may include:
- Diuretics, such as furosemide, for pulmonary edema, as recommended by the American Thoracic Society guidelines 1
- Corticosteroids, such as prednisone, for inflammatory conditions, as discussed in the Portuguese Pulmonology Society guidelines 1
- Antifibrotics, such as pirfenidone or nintedanib, for fibrotic diseases, as recommended by the American Thoracic Society guidelines 1
From the Research
Causes of Interstitial Thickening (Interstitial Lung Disease)
The causes of interstitial thickening, also known as interstitial lung disease (ILD), are complex and multifaceted. Some of the key factors that contribute to the development of ILD include:
- Inflammation and scarring of the lung interstitium 2
- Fibrosis of the interstitial space, which can lead to progressive lung damage 3
- Idiopathic pulmonary fibrosis (IPF), which is the most common form of ILD 4, 5
- Connective tissue disease-associated ILD, such as scleroderma-associated ILD 6
- Hypersensitivity pneumonitis, which is caused by an allergic reaction to certain substances 6
- Genetic predisposition, environmental factors, and exposure to certain toxins or pollutants 6
Types of Interstitial Lung Disease
There are several types of ILD, including:
- Idiopathic pulmonary fibrosis (IPF) 4, 5
- Hypersensitivity pneumonitis 6
- Connective tissue disease-associated ILD, such as scleroderma-associated ILD 6
- Interstitial lung disease associated with progressive pulmonary fibrosis of any cause 6
- Other rare forms of ILD, such as lymphangioleiomyomatosis and pulmonary alveolar proteinosis 6
Pathophysiology of Interstitial Lung Disease
The pathophysiology of ILD involves inflammation and scarring of the lung interstitium, which can lead to progressive lung damage and fibrosis. This can result in: