​Pathology - Emphysema

​Pathology - Emphysema
Linked to both smoking and genetic alpha-antitrypsin deficiency
Pathophysiology refers to the study of the abnormal physiological processes that occur in the body as a result of a disease or disorder. Elastase enzymes degrade elastin in the alveolar wall, unless counteracted by alpha-1-antitrypsin. Smoking deactivates alpha-1-antitrypsin and attracts neutrophils, which are a source of elastase.

Gross pathology: The presence of enlarged air gaps and damage of the alveolar wall are observed. There are four distinct patterns that can be identified. (1) Centriacinar: The respiratory bronchioles, particularly in the upper lobes, are enlarged, a condition commonly observed in individuals who smoke; (2) Panacinar: The entire acinus, which refers to the functional unit of the lung, is dilated throughout the entire lung. This condition is typically associated with a lack of alpha-1-antitrypsin; (3) Paraseptal: The distal alveoli, located near the pleura and interlobar septa, are dilated. In some cases, huge blebs may be visible. (4) Irregular: The acinus is affected in an irregular manner, sometimes accompanied by scarring and inflammation.

The individual exhibits symptoms such as "pink puffers" (a decrease in the speed of forced expiration through pursed lips), difficulty breathing (dyspnea), bluish discoloration of the skin (cyanosis), rapid heart rate (tachycardia), a chest that seems round and barrel-shaped, faint breath noises, and the usage of additional respiratory muscles.

Complications encompass pneumothorax resulting from bulla rupture, persistent bronchitis, and cor pulmonale.
Imaging reveals hyperinflation and bulla, together with a flattened diaphragm, as observed on the chest X-ray.
Pulmonary function test results indicate the presence of hypoxia, elevated residual volume and total lung capacity, and reduced forced expiratory volume and forced vital capacity ratio.

Therapeutic interventions 
Methods for quitting smoking; oxygen therapy; inhaled medications that block the effects of acetylcholine and stimulate S-receptors; inhaled corticosteroids
Emphysema is a long-term respiratory condition characterized by obstructed airflow in the lungs, known as chronic obstructive pulmonary disease (COPD).
Hepatic cirrhosis is also caused by hereditary alpha antitrypsin deficiency.