Savara Introduces aPAP ClearPath, a GM-CSF Autoantibody Blood Test to Detect Autoimmune Pulmonary Alveolar Proteinosis (aPAP)

Savara Inc. (Nasdaq: SVRA) (the Company), a clinical stage biopharmaceutical company focused on rare respiratory diseases, recently launched aPAP ClearPath, a new serum-based blood test that can be used by physicians in the U.S. to obtain a definitive diagnosis of aPAP, a rare autoimmune lung disease mediated by autoantibodies targeting GM-CSF. Autoimmune PAP accounts for approximately 90% of all patients with PAP and has an estimated diagnosed prevalence of seven cases per million in the U.S. and similar or higher prevalence reported elsewhere in the world.

The aPAP ClearPath test is a highly sensitive and specific quantitative immunoassay designed to detect aPAP GM-CSF autoantibodies in human serum. The Company partnered with Trillium Health (trillium-health.com), a modern health solutions provider and a Clinical Laboratory Improvement Amendments (CLIA)-certified lab, to develop the simple, no-cost, and noninvasive test. A supporting disease awareness campaign was also launched to improve understanding of aPAP, highlight the hallmark signs and symptoms of the disease, and educate physicians about the need for early testing.

“Our strong operational focus continues as we deliver on our goal of introducing a laboratory-based GM-CSF autoantibody blood test in the U.S. by the end of the year,” said Matt Pauls, Chair and CEO, Savara. “Our commitment to the patient community is underscored by the introduction of this simple, no-cost blood test that U.S. physicians can use to confirm or rule out aPAP. With a few thousand diagnosed patients in the U.S., we suspect the true prevalence of aPAP may be underestimated and, similar to other rare diseases, the introduction of a diagnostic could help decrease the time to diagnosis and increase diagnosed prevalence.”

“Education and widespread blood testing for aPAP is a critical step in shortening the time to diagnosis for patients with aPAP, helping to avoid misdiagnoses and more costly and invasive diagnostic procedures,” said Ali Ataya, M.D., Associate Professor of Medicine, University of Florida, Division of Pulmonary and Critical Care Medicine. “I congratulate Savara for making this simple test broadly available for physicians in the U.S.”

Only a physician or healthcare provider can make a diagnosis of aPAP. To learn more about aPAP ClearPath, the importance of early testing for aPAP, and to order a test kit, please visit www.apapclearpath.com.

The Company expects to roll out a healthcare provider disease awareness campaign and GM-CSF autoantibody blood test in Europe next year.

About aPAP

Autoimmune PAP is a rare lung disease characterized by the abnormal build-up of surfactant sediment in the alveoli (or air sacs) of the lungs. The surfactant consists of proteins and lipids and is an important physiological substance that lines the inside of the alveoli to prevent the lungs from collapsing. In a healthy lung, the old and inactivated surfactant is cleared and digested by immune cells called alveolar macrophages. Alveolar macrophages need to be stimulated by granulocyte-macrophage colony-stimulating factor (GM-CSF) to function properly in clearing surfactant, but in autoimmune PAP, GM-CSF is neutralized by antibodies against GM-CSF, rendering the macrophages unable to perform their tasks. As a result, an excess of surfactant accumulates in the alveoli, causing obstruction of gas exchange, and patients start to experience shortness of breath, often with cough and frequent fatigue. Patients may also experience chronic cough, as well as episodes of fever, chest pain, or coughing blood, especially if secondary lung infection develops. In the long-term, the disease can lead to serious complications, including lung fibrosis and the need for a lung transplant.