Prevalence of aPAP

Autoimmune PAP is a disease that belongs to a family of distinct, rare lung diseases collectively referred to as PAP. Autoimmune PAP represents about 90% of all patients with PAP.

The estimated prevalence of aPAP is 7 cases per million people in the U.S. and 6 cases per million in Japan, with similar or higher prevalence reported elsewhere in the world.

Japan, a country that has a more centralized approach to diagnosing and treating aPAP, has seen a consistent increase in patients being diagnosed with the disease. It is now estimated the prevalence in Japan could be 4 times the original estimate of 6 cases per million in that country. Read more here.

aPAP most commonly affects men in early middle age, but both sexes and patients of any age can be affected.

Burden of aPAP

Most patients must learn how to manage the burden of aPAP symptoms and the impacts the disease has on everyday activities.

As a result of surfactant accumulation, gas exchange in the lung is impaired and patients may start to experience shortness of breath and decreased exercise tolerance.

At first, shortness of breath typically occurs upon exertion, but as the disease progresses, it can occur even when a person is at rest. Some patients experience significantly reduced exercise capacity that can dramatically impact the simplest of daily activities, e.g., becoming winded simply walking up a flight of stairs.

Patients may experience cough and episodes of fever—especially if secondary lung infection develops.

In the long-term, the disease can lead to serious complications, including lung fibrosis and the need for lung transplant.

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