What are the causes of restrictive lung disease?

From the Guidelines

Restrictive lung diseases are caused by conditions that limit lung expansion, reducing total lung capacity, and can be broadly categorized into intrinsic and extrinsic factors, including pulmonary fibrosis, sarcoidosis, pneumoconiosis, and inflammatory conditions, as well as chest wall abnormalities, pleural diseases, and neuromuscular disorders, as noted in the most recent study 1 and 1.

Causes of Restrictive Lung Disease

The causes of restrictive lung disease can be divided into two main categories: intrinsic and extrinsic factors.

• Intrinsic causes affect the lung tissue directly and include:
  • Pulmonary fibrosis (which may be idiopathic or related to environmental exposures like asbestos, silica, or radiation)
  • Sarcoidosis
  • Pneumoconiosis
  • Inflammatory conditions like hypersensitivity pneumonitis
  • Infections such as tuberculosis can lead to scarring and restriction
• Extrinsic causes originate outside the lungs and include:
  • Chest wall abnormalities (kyphoscoliosis, obesity, or ankylosing spondylitis)
  • Pleural diseases (effusions, thickening, or fibrosis)
  • Neuromuscular disorders that weaken respiratory muscles (myasthenia gravis, Guillain-Barré syndrome, or muscular dystrophy)
  • Abdominal processes like ascites or pregnancy can also restrict diaphragm movement

Diagnosis and Treatment

Diagnosis of restrictive lung disease involves a combination of clinical evaluation, pulmonary function tests, and imaging studies, as outlined in the American College of Radiology Appropriateness Criteria for diffuse lung disease 1. Treatment depends on addressing the underlying cause, which may include anti-inflammatory medications for inflammatory conditions, oxygen therapy, pulmonary rehabilitation, or in severe cases, lung transplantation, as discussed in the context of osteogenesis imperfecta and pulmonary function guidance 1 and 1. It is essential to consider the most recent and highest quality studies when making a definitive recommendation, and in this case, the studies from 2021 and 2023 provide the most up-to-date guidance on the causes and management of restrictive lung disease.

From the Research

Causes of Restrictive Lung Disease

The causes of restrictive lung disease can be categorized into two main groups: intrinsic and extrinsic.

• Intrinsic causes refer to conditions that affect the lung parenchyma, such as:
  • Interstitial lung diseases (e.g., idiopathic pulmonary fibrosis) 2, 3, 4, 5, 6
  • Idiopathic interstitial pneumonia (IIP) 3
  • Pulmonary fibrosis 3
• Extrinsic causes refer to conditions that affect the chest wall, muscles, or pleura, such as:
  • Obesity 2
  • Neuromuscular disorders 2
  • Chest wall limitations 3
  • Muscle dysfunction 3
  • Pleural disease 3
  • Systemic sclerosis-associated interstitial lung disease 6
  • Interstitial lung disease-associated pulmonary hypertension 6

Underlying Conditions

Certain underlying conditions can increase the risk of developing restrictive lung disease, including:

• Connective tissue disease 3
• Primary lung cancer 3
• Systemic vasculitis 5
• Malnutrition 4

Disease Progression

The progression of restrictive lung disease can vary depending on the underlying condition, with some conditions exhibiting spontaneous reversibility or stabilization, while others may progress to respiratory failure and death 6.