Pulmonary Fibrosis

Overview

Idiopathic pulmonary fibrosis is an irreversible, fatal lung disease that plagues adults in their 60s who are otherwise healthy. Fibrotic scar tissue takes over the lungs, progressively decreasing a patient’s ability to transfer oxygen into the bloodstream and causes progressive shortness of breath with activities requiring exertion and, ultimately, while patients are at rest and during conversation.

Related conditions include pulmonary fibrosis associated with rheumatological diseases, such as scleroderma; systemic sclerosis; rheumatoid arthritis; dermato-polymyositis; systemic lupus, such as erythematosus; idiopathic pulmonary fibrosis; idiopathic interstitial pneumonias; interstitial lung disease; pulmonary fibrosis of known origin, such as environmental factors at work and at home, certain medications, asbestosis and silicosis; pulmonary fibrosis of unknown causes, such as sarcoidosis and granulomatous lung diseases; hypersensitivity pneumonitis; nonspecific interstitial pneumonia of the idiopathic variety; and those diseases associated with collagen vascular diseases

Symptoms

Symptoms include shortness of breath with exertion, chronic cough, fatigue, weakness, chest discomfort and eventual loss of appetite and weight loss.

Causes

The cause of pulmonary fibrosis is unknown.

Risk Factors

Risk factors include genetic predisposition, hidden autoimmune problems similar to rheumatological diseases, environmental factors, infections with unknown viruses, micro-aspiration of acid from the stomach, such as abnormal gastroesophageal reflux, into the lungs and cigarette smoking.

Diagnosis

Accurate diagnosis and early treatment increase the likelihood that the disease will respond to medical treatment.

Pulmonary fibrosis is diagnosed based on thorough medical history, family medical history, history of environmental exposures, a physical exam and the results from tests, such as pulmonary function tests, HRCT scans and blood tests, bronchoscopy and surgical lung biopsy.

Complications

Pulmonary fibrosis is an irreversible, fatal lung disease. Most patients do not survive three to five years after diagnosis. Pulmonary hypertension occurs in almost all patients as the disease advances. Acute respiratory decompensation can occur if other complications, such as pneumonia, blood clots, lung collapse, silent aspiration of stomach acid into the lungs and rarely lung cancer. Other complications include treatment associated problems or adverse events. Patients who also have other breathing disorders, such as emphysema and sleep apnea, may have additional complications and poor outcome.

Recovery

Patients with pulmonary fibrosis should be followed for pulmonary care by physicians familiar with idiopathic pulmonary fibrosis and related complications, take medications as directed and eat a healthy diet. Overweight people should decrease their weight so that they have a body mass index below 30. Patients should avoid lung irritants, such as dust, chemicals and cigarette smoke. Patients should work to decrease gastroesophageal reflux. Patients need close monitoring by physicians as their functional impairment may change after a few months.

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