Pulmonary alveolar proteinosis (PAP) is a rare and progressive lung disease in which protein builds up in the air sacs (alveoli) of the lungs, making it very difficult for you to breathe. It typically appears in younger patients, ages 20-50, and more often in men than in women.
Our physicians have extensive experience in diagnosing, treating and helping patients manage PAP. In fact, Penn is the mid-Atlantic regional referral center for the treatment of PAP.
Symptoms of PAP include exercise intolerance, shortness of breath, cough, fatigue, and unintentional weight loss. Some patients experience few or no symptoms.
Comprehensive Diagnosis Leads to Optimal Treatment
Because 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 your lungs by injecting a sterile salt solution into the lungs and then suctioning it out. Following this treatment, you will experience significant improvement in your symptoms.