A Woman’s Suspected Vaping Lung Injury Was Hiding a Rare Autoimmune Disease That Clogged Her Air Sacs

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Doctors suspected a vaping-related lung injury. But tests eventually pointed to a second, far rarer problem: autoimmune pulmonary alveolar proteinosis, a disease in which surfactant, a slippery fat-and-protein mixture, builds up in the lungs’ tiny air sacs and blocks oxygen from reaching the blood.

The case, published October 5, 2026, in Oxford Medical Case Reports by physicians at TriHealth Good Samaritan Hospital in Cincinnati and colleagues, ended with the patient’s death. The authors called it a diagnostic dilemma, with two overlapping lung conditions obscuring each other.

The woman had chronic obstructive pulmonary disease and acid reflux and smoked tobacco daily. Before her hospitalization, she had been treated twice as an outpatient with steroids, the second time alongside an antibiotic, without improvement.

By the time she was admitted, she had fever, chills, and weakness and needed supplemental oxygen. Her white blood cell count and C-reactive protein, a marker of inflammation, were high, but tests for viral and bacterial infection came back negative. CT scans showed extensive hazy, ground-glass areas in both lungs that were still there on a repeat scan eight days later.

Those findings, combined with her vaping, raised suspicion for e-cigarette or vaping product use-associated lung injury, known as EVALI. The report does not say what type of vaping product she used.

In classic pulmonary alveolar proteinosis, fluid washed from the lungs during bronchoscopy looks milky. Hers did not. Her first PAS stain, a test that highlights the built-up material, was negative, and a repeat procedure was not definitive.

The fluid was dominated by neutrophils and macrophages, two types of immune cells, and fungal studies were negative.

The decisive clue came from a blood test. She had elevated antibodies, 610 ng/mL against a reference value below 338 ng/mL, against granulocyte-macrophage colony-stimulating factor, or GM-CSF. Immune cells in the lungs need this signaling protein to clear away surfactant. When antibodies block it, surfactant accumulates.

According to StatPearls, a reference hosted by the National Library of Medicine, autoimmune disease accounts for about 90% of documented pulmonary alveolar proteinosis cases. The case report’s authors note that the disease affects only about six to seven people per million and cite research findings that roughly 79% of patients are active smokers, as she was.

High-dose intravenous steroids, a standard treatment for EVALI, brought no response. She was transferred for whole lung lavage, a procedure that washes out one lung at a time under anesthesia and is the standard treatment for pulmonary alveolar proteinosis.

Her oxygen levels kept falling. She was placed on ECMO, a machine that oxygenates blood outside the body, and developed bloodstream infections related to her lines. After discussions with her care team and family, she was moved to comfort care and died.

An autopsy supported the diagnosis while revealing additional damage. In the authors’ words, “Post-mortem pathology confirmed surfactant accumulation and foamy macrophages but also showed features of diffuse alveolar damage.”

The authors stopped short of blaming vaping. They wrote that there is currently no definitive evidence connecting vaping to the development of anti-GM-CSF antibodies, and they described her lung injury as likely multifactorial. EVALI was suspected but never formally confirmed.

A 2021 report in Respirology Case Reports described a 33-year-old man who vaped, smoked, and used cannabis and developed autoimmune pulmonary alveolar proteinosis. His breathing and oxygen levels improved dramatically after whole lung lavage, and those authors also treated a link to vaping as only possible.

EVALI drew national attention in 2019 and 2020. As of February 18, 2020, the CDC had recorded 2,807 hospitalized cases or deaths and 68 confirmed deaths, with vitamin E acetate, an additive found in some THC vaping products, strongly linked to the outbreak.

Vaping remains common among young people. The 2025 National Youth Tobacco Survey, released by the FDA in June 2026, found that 5.2% of U.S. middle and high school students currently used e-cigarettes, down from 5.9% in 2024.

For people with autoimmune pulmonary alveolar proteinosis, treatment options may soon expand. Savara’s inhaled GM-CSF therapy, molgramostim, is under FDA review. The agency refused to file the company’s first application in 2025. Savara resubmitted it in December 2025, and the FDA accepted it for priority review in February 2026. In April, the agency extended its review by three months, setting a decision date of November 22, 2026. The drug is not yet approved.

The authors said the possible relationship between vaping-related lung injury and surfactant disorders merits further study. Anyone who vapes and develops worsening shortness of breath, cough, or fever should seek medical care and tell clinicians about their vaping history.

It is a rare lung disease in which surfactant builds up in the air sacs and interferes with oxygen exchange. Most cases are autoimmune.

Her recent vaping and lung scans pointed toward vaping-related lung injury, and her lung fluid lacked the classic milky look of the rarer disease.

The authors said there is no definitive evidence that vaping causes the antibodies behind autoimmune pulmonary alveolar proteinosis, and they described her lung injury as likely multifactorial.

How is autoimmune pulmonary alveolar proteinosis treated?

Whole lung lavage is the standard treatment. An inhaled therapy called molgramostim is under FDA review, with a decision expected by November 22, 2026.

When should someone who vapes see a doctor?

Worsening shortness of breath, persistent cough, chest pain, or fever warrant prompt medical care, along with full disclosure of vaping history.