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INTERSTITIAL LUNG DISEASE
Interstitial lung disease, or diffuse parenchymal lung disease, is a group of respiratory diseases affecting the interstitium and space around the alveoli of the lungs.
It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process is disrupted, and the tissue around the air sacs becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years.

ILDs encompass over 200 different conditions that primarily affect the interstitial space of the lungs, which is the tissue and space around the air sacs. Common types include idiopathic pulmonary fibrosis, sarcoidosis, pneumoconiosis (often related to occupational exposures), and connective tissue disease-related lung disease. Symptoms typically include persistent dry cough, shortness of breath on exertion, and fatigue. The exact cause of many ILDs remains unclear, but factors such as environmental exposures, autoimmune diseases, and genetic predisposition play a role.

In the UK, the prevalence of ILD is rising, in part due to an aging population and improved diagnostic techniques. It is estimated that around 5-10 people per 100,000 suffer from idiopathic pulmonary fibrosis alone. Other forms of ILD are often underdiagnosed, as symptoms can mimic other respiratory conditions.

Diagnosis of ILD typically involves a comprehensive approach, including a detailed patient history, physical examination, pulmonary function tests (PFTs), imaging studies (such as high-resolution computed tomography scans), and sometimes lung biopsies. The multidisciplinary approach is crucial due to the variety of conditions under the ILD umbrella. Specialist centers in the UK are increasingly collaborating across disciplines to improve diagnosis and patient care.

Though there is no cure for most ILDs, management focuses on slowing disease progression and maintaining quality of life. Treatment options may include:

  1. Medications: Anti-fibrotic drugs like pirfenidone and nintedanib have been shown to slow the progression of idiopathic pulmonary fibrosis. Corticosteroids and immunosuppressive drugs are also utilized for autoimmune-related ILDs.

  2. Oxygen Therapy: Supplementing oxygen can help alleviate respiratory symptoms and improve the quality of life in patients experiencing significant hypoxemia.

  3. Pulmonary Rehabilitation: Tailored physical therapy can enhance functional capacity, endurance, and overall well-being.

  4. Lung Transplantation: In severe cases, lung transplantation may be the only option, especially for younger patients with idiopathic pulmonary fibrosis.