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Pulmonary fibrosis is a condition in which the lungs become scarred over time. [1] Symptoms include shortness of breath , a dry cough, feeling tired, weight loss, and nail clubbing . [ 1 ] Complications may include pulmonary hypertension , respiratory failure , pneumothorax , and lung cancer .
515 Postinflammatory pulmonary fibrosis; 516 Other alveolar and parietoalveolar pneumonopathy. 516.3 Idiopathic fibrosing alveolitis. Hamman-Rich syndrome; 517 Lung involvement in conditions classified elsewhere 517.1 Rheumatic pneumonia; 517.2 Lung involvement in systemic sclerosis; 517.3 Acute chest syndrome; 517.8 Lung involvement in other ...
The median survival in idiopathic pulmonary fibrosis is 3-3.5 years. In those who receive a lung transplant, the medial survival in idiopathic pulmonary fibrosis is 5.2 years, as compared to 6.7 years in those with other types of ILD. [8] ILD is associated with a 3-fold increased risk of lung cancer. [8]
Idiopathic pulmonary fibrosis (IPF) synonymous with cryptogenic fibrosing alveolitis [5] is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.
While a lung biopsy is the gold standard, some clinicians opt against this due to the risks of the procedure. Lung biopsies performed on patients with NSIP reveal two different disease patterns – cellular and fibrosing – which are associated with different prognoses. The cellular pattern displays chronic inflammation with minimal fibrosis.
Appearance of usual interstitial pneumonia (UIP) in a surgical lung biopsy at low magnification. The tissue is stained with hematoxylin (purple dye) and eosin (pink dye) to make it visible. The pink areas in this picture represent lung fibrosis (collagen stains pink). Note the "patchwork" (quilt-like) pattern of the fibrosis.
Medical treatment for restrictive lung disease is normally limited to supportive care since both the intrinsic and extrinsic causes can have irreversible effects on lung compliance. [10] The supportive therapies focus on maximizing pulmonary function and preserving activity tolerance through oxygen therapy, bronchodilators, inhaled beta ...
Although fibrosis is not a normal feature of these subtypes, repair strategies in end-stage emphysema may lead to pulmonary fibrosis. [14] The fourth subtype is known as paracicatricial emphysema or irregular emphysema, involves the acinus irregularly and is associated with fibrosis. [17]