Idiopathic Pulmonary Fibrosis

Disease characterized by the cells infiltration in interstitial respiratory tissues (interstitial pulmonary disease) with healing of lung parenchyma. This leads to an increase in elastic recoil of the lung and a decrease of compliace, that we known as characteristics of restrictive pulmonary diseases.

Clinical presentation

Also known as criptogenic fibrosing alveolitis, the pulmonary fribosis is an uncommon disease that includes dry and persistent cough with no fever or chest pain. With the progress of the disease the dyspnea proliferates and originates cyanosis. In more advanced stages it occurs pulmonary hypertension that leads to peripheral edema and right cardiac insufficiency.

Phatophysiology

The primary injury that leads to fibrosis remains unknown. Meanwhile it can be observed a series of cellular events that regulates the inflammatory process and the fibrotic answer.

These events are:

- Initial injury of the tissue;

- Damage and vascular activation and trombolises in several degrees;

- Injury and epithelium activation with loss on the barrier integrity and release of proinflammatory mediators;

- Increased adhesion of leukocytes;

- Continuous process of injury and healing characterized by changes in cellular populations, and increased production of matrix with increased deposits of collagen and elastin.

These events lead to a pattern of pulmonary injury that causes an increased of the elastic recoil and changes on  gas exchange and pulmonary and vascular abnormalities.  

Clinical Manifestations

Cough

Dyspnea and tachypnea: It is necessary a larger effort to pulmonary distension. A fast and superficial inspiration pattern decreases the ventilation effort.  

Respiratory rales: Reflect the sucessive opening of respiratory units due to fibrosis and loss of surfactant.

Cardiatic Examination: hypoxemia, pulmonary hypertension, increased jugular venous pressure, tricuspid regurgitation murmur.

Pulmonary function tests: Reduction of CPT, FEV1 and FVC. DLCO progressively reduces.

Arterial blood gas analysis: Hypoxemia due to the increased dead space.