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Whether you’re newly diagnosed, supporting a loved one, or seeking deeper insight into Pulmonary Alveolar Proteinosis (PAP), you’ve come to the right place. The Education Hub is your starting point for trustworthy, up-to-date information from leading experts, real patients, and partner organizations. Get the facts, understand your options, and take the next step with confidence.

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What Is Pulmonary Alveolar Proteinosis?

PAP is a rare lung disease in which surfactant, a naturally occurring substance meant to keep your lungs open, builds up abnormally in the air sacs (alveoli). This buildup prevents oxygen from passing into the bloodstream, making breathing increasingly difficult.

The most common form of PAP is autoimmune PAP (aPAP) which is caused by the presence of autoantibodies to granulocyte/macrophage colony stimulating factor (GM-CSF) which prevents cells in the lung (macrophages) from removing the excess surfactant. There are other types of PAP (secondary PAP, hereditary PAP, congenital PAP) that are linked to hematologic disorders, environmental exposures, or genetic factors.

Key Facts About PAP

7 in 1 million

people are affected by PAP worldwide.

30-50 years old

is the age when PAP is most commonly diagnosed.

~90% of cases

are Autoimmune Pulmonary Alveolar Proteinosis (aPAP).

PAP Symptoms

Common symptoms overlap with asthma, pneumonia, or COPD and some people show no symptoms in early stages.

  • Shortness of breath (dyspnea)

  • Chronic cough

  • Fatigue

  • Unexplained weight loss

  • Chest discomfort

  • Less frequently reported symptoms include coughing up blood, fever, chest discomfort, and fatigue, and roughly one-third of patients may be asymptomatic in the early stages of the disease.

Diagnosis & Testing

Imaging Tests

Chest X-rays and CT scans often reveal a “crazy-paving” pattern unique to PAP.

Pulmonary (Lung) Function Tests

Pulmonary Function Tests are used to assess how well the lungs are functioning. Results can support overall evaluation but do not confirm a PAP diagnosis.

Bronchoalveolar Lavage (BAL)

Bronchoscopy with Bronchoalveolar Lavage: A minimally invasive procedure that allows clinicians to collect a small fluid sample from the lungs for laboratory testing. It is often considered the most reliable method for confirming a diagnosis.

Blood tests

Particularly important in detecting autoimmune PAP (GM-CSF antibodies). There is a free, simple GM-CSF autoantibody blood test available called aPAP ClearPathTM that can help confirm or rule out if you have aPAP. Your provider may get your blood sample through blood drawn from a vein or a finger prick. You can ask your healthcare provider about this test and learn more by contacting the Lung HelpLine.

Treatment

Treatment options vary by PAP type and patient need. Ongoing monitoring is essential.

Whole Lung Lavage (WLL)

The most common treatment for moderate/severe PAP – this can be used for aPAP and hereditary PAP.

Sargmalin (available in Japan)

Sargmalin has been used as a granulocyte–macrophage colony-stimulating factor (GM-CSF)–based therapy to help restore alveolar macrophage function and improve surfactant clearance in patients with PAP.

GM-CSF Therapy

Targets the underlying macrophage dysfunction in PAP. May involve GM-CSF therapy using a nebulizer.

Oxygen

Supplemental oxygen is an important supportive treatment option for patients with pulmonary alveolar proteinosis (PAP), helping to alleviate hypoxemia and improve exercise tolerance while definitive therapies are pursued.

Clinical Trials

Clinical trials represent an important treatment option for patients with pulmonary alveolar proteinosis (PAP), offering access to investigational therapies that target the underlying disease mechanisms. These studies may evaluate novel GM-CSF–based approaches, immunomodulatory therapies, or other emerging strategies aimed at improving alveolar macrophage function and gas exchange.
clinicaltrials.gov/find-studies

Savara EAP

Savara is sponsoring the Savara Early Access Program (EAP) to provide potential access to its

investigational drug, molgramostim, a nebulizer solution, for patients with autoimmune pulmonary

alveolar proteinosis (aPAP) living in the United States who meet the program’s eligibility criteria.

 Living with PAP can feel isolating—but you're not alone. We’ve gathered practical tools and links to help you get the support you need, from insurance assistance to finding a clinical trial.

Take Action. Find Support.

Listen to our founder.

Kelsea Arford joins Immunity Pulse Podcast to talk all things lung lavage.