وصف
Interstitial lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue. The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream. It can be caused by long-term exposure to hazardous materials, such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. In some cases, however, the causes remain unknown. Once lung scarring occurs, it's generally irreversible. Medications may slow the damage of interstitial lung disease, but many people never regain full use of their lungs. Lung transplant is an option for some people who have interstitial lung disease.
ملخص
- Interstitial lung diseases (ILDs) encompass a diverse group of disorders characterized by generalized involvement of the lung interstitium, the connective tissue between alveolar sacs and secondary pulmonary lobes. These diseases share common clinical, radiological, and histological manifestations, requiring a careful and systematic approach for accurate diagnosis.
- ILDs are broadly categorized into idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs. IPF is the most important subgroup due to its poor prognosis, typically with a mean survival of around three years. Classifying ILDs as IPF or non-IPF is crucial for guiding treatment strategies and anticipating disease progression.
- Diagnosis of ILDs often involves a multidisciplinary approach, including clinical evaluation, radiological imaging, and pathological assessment. A thorough history focusing on chronic exertional dyspnea, cough, and fatigue is essential. Physical examination may reveal velcro-like crackles on auscultation and clubbing of the fingers.
- Radiological imaging, particularly high-resolution computed tomography (HRCT), plays a critical role in diagnosing ILDs. Key features to identify on CT scans include septal thickening, traction bronchiectasis, and honeycombing. Understanding the anatomy of secondary pulmonary lobules is crucial for interpreting CT patterns.
- IPF is characterized by a specific HRCT pattern known as usual interstitial pneumonia (UIP), featuring subpleural and basal predominant distribution, heterogeneous reticular opacities, and honeycombing with or without traction bronchiectasis. The presence of UIP pattern on HRCT can be highly suggestive of IPF, especially when alternative diagnoses are ruled out.
- Management of IPF primarily focuses on slowing disease progression and improving quality of life. Approved antifibrotic medications include pirfenidone and nintedanib, which can slow the decline in forced vital capacity (FVC). Supportive care, such as oxygen therapy and pulmonary rehabilitation, also plays a crucial role.
- Other idiopathic interstitial pneumonias include nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), cryptogenic organizing pneumonia (COP), and acute interstitial pneumonia (AIP). These entities have varying clinical presentations, radiological features, and prognoses.
- Drug-induced ILDs can occur as a result of exposure to certain medications, particularly anti-cancer drugs and others. It is imperative to take drug history before labeling the patient as I to avoid the problems associated with drug induced ILD. Recognizing and discontinuing the causative agent is essential for management.
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