Paul F. Harron, Jr. Lung Center

Pulmonary Alveolar Proteinosis

Overview

Penn Lung Center physicians have extensive experience in diagnosing, treating and helping patients manage pulmonary alveolar proteinosis (PAP). Penn is the mid-Atlantic regional referral center for the treatment of PAP.

PAP is a rare lung disease in which protein builds up in the air sacs (alveoli) of the lungs, making breathing difficult. It typically appears in younger patients, ages 20-50, and more often in men than in women. PAP is a progressive disease.

Symptoms of PAP include:

  • Exercise intolerance
  • Shortness of breath
  • Cough
  • Fatigue
  • Unintentional weight loss

Some patients experience few or no symptoms.

Comprehensive Diagnosis Leads to Optimal Treatment

Because its symptoms are similar to those of other lung diseases, PAP is usually diagnosed through a combination of tests, including:

  • Bronchoscopy
  • Chest X-rays
  • Pulmonary function tests

In some cases, a lung biopsy may be necessary for definitive diagnosis.

PAP can be the result of infection, an immunologic issue, exposure to high levels of dust or blood cancers. However, in many cases, there is no predisposing cause for the disease.

The Latest Treatment

In addition to treating any potential, predisposing causes, whole-lung lavage is the standard treatment for PAP. During whole-lung lavage, surgeons wash the protein from a patient's lungs by injecting a sterile salt solution into the lungs and then suctioning it out. The vast majority of patients experience significant improvement in symptoms after this treatment.