Causes

Pulmonary fibrosis is believed to result from exposure to radiation or inhalation of noxious materials such as silica, asbestos, and coal dust. Pulmonary conditions that can result in

794 Pulmonary Fibrosis pulmonary fibrosis include pneumonia, atelectasis, alveolar cell cancer, pulmonary edema, and lung surgery or trauma. Nonpulmonary causes include neuromuscular disease such as Guillain-Barre syndrome, amyotrophic lateral sclerosis, myasthenia gravis, or muscular dystrophy. Approximately one-third of patients can trace their initial episodes of dyspnea to a viral respiratory illness. Deformities of the bones, such as ankylosing spondylitis and scoliosis, can result in pulmonary fibrosis.

Genetic contribution to pulmonary fibrosis is suggested by familial cases of the disease. Variants in the gene encoding pulmonary surfactant protein SPA1 have increased susceptibility to idiopathic pulmonary fibrosis in nonsmokers. Mutations in the gene encoding surfactant protein C (SFTPC) have been identified in some families with idiopathic pulmonary fibrosis, but not in others.

Although it is possible for pulmonary fibrosis to occur at any age, the average age of patients who are diagnosed with pulmonary fibrosis is 50. Elderly patients or those exposed to risk factors for a prolonged period of time are at the greatest risk. Pulmonary fibrosis occurs in both men and women, and there is no known racial or ethnic predilection.

HISTORY. Establish a history of work or lifestyle that may have caused the disease. Ask if the patient has worked as a coal miner or with materials such as asbestos or silica or whether he or she has lived near industrial plants that use such materials. Determine if the patient has had respiratory complications or conditions such as pneumonia, atelectasis, alveolar cell cancer, pulmonary edema, and lung surgery or trauma. Ask if the patient has experienced pain while breathing or shortness of breath. Ask if the patient smokes cigarettes. There is some suspicion that genetic factors may determine susceptibility to the disease, so take a family history of pulmonary conditions, including pulmonary fibrosis.

PHYSICAL EXAMINATION. Observe the patient's respiratory status, noting rate, depth, rhythm, and ease of breathing. In the initial phases of the disease, the physical examination may be normal and the lungs may be clear on auscultation. As the disease progresses, individuals with pulmonary fibrosis frequently develop shallow, rapid breathing patterns in an attempt to conserve energy. Note any cough. Auscultate the patient's lungs; listen for diminished or absent breath sounds or coarse crackles, particularly at the lung bases on inspiration. Note chest excursion and symmetry. Check for digital clubbing. Because pulmonary fibrosis in its late stages can cause cor pulmonale, check for signs of cardiac failure, such as elevated neck veins, liver distension, and swelling of the lower extremities.

PSYCHOSOCIAL. Patients may experience a lowering of self-esteem with increased dependence on others and changing roles. In addition, shortness of breath and difficulty in breathing usually cause increased anxiety. If the disease has an occupational source, financial concerns may play an important role if the patient is unable to return to work.

Diagnostic Highlights

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