Pulmonary Alveolar Proteinosis
New pulmonary alveolar proteinosis research, summarized each month in plain language.
2026 so far: So far in 2026 (January 2026 to September 2026), inhaled GM-CSF was linked to better oxygen measures and less breathlessness in autoimmune PAP, while whole-lung lavage approaches (including ECMO support) and fibrosis/infections helped explain differences in outcomes. Read the 2026 review
We also cover clinical trials.
Yearly update
2026 so far
- Inhaled GM-CSF results. A stronger trial summary helped clarify the likely benefits and limits.
- Whole-lung lavage care. Reports added detail on how lavage is performed and monitored.
- Fibrosis and risks. More reports connected complications and scarring with harder-to-treat disease.
- Finding the cause. Reports added options for confirming autoimmune PAP and for genetic workups in children.
- Why severity differs. Early studies pointed to differences in antibody behavior and immune-cell pathways.
New
September 2026
- ECMO during lavage. Lavage with ECMO was linked to larger oxygen and breathing-test gains.
- Daily function. Walking distance and activity scores improved more with ECMO support.
- Antibody levels. Anti-GM-CSF antibody levels fell over years in some people.
August 2026
- Lavage plus sargramostim. One autoimmune PAP case improved after lavage and inhaled sargramostim.
- Need for repeat lavage. Breathing tests and oxygen worsened, so lavage was repeated.
- Mixed lung measures. On nintedanib, lung capacity stayed stable while oxygen transfer fell.
- Related conditions. Reports linked PAP with infection, immune disorders, or autoimmunity.
July 2026
- Treatment outcomes. In autoimmune PAP, inhaled GM-CSF and lung lavage looked similar.
- Lung scarring risk. Lung scarring was linked to less benefit and worse survival.
- Shorter lavage time. A modified lavage method shortened anesthesia time without more complications.
June 2026
- Registry care patterns. A registry found whole-lung lavage recorded in about one-quarter.
- Inhaled GM-CSF use. Case reports noted improvement in some autoimmune PAP patients.
- Possible secondary causes. Reports linked PAP to certain exposures or medicines in some cases.
- Antibody binding strength. Severity may relate to antibody binding strength, not just amount.
May 2026
- Autoimmune PAP antibody test. A newer blood antibody test gave results similar to a reference method for identifying autoimmune PAP.
- Positive tests in infections. Positive antibody results were also reported in some people with serious infections (including disseminated Cryptococcus or Nocardia), which can complicate interpretation.
- Genetic causes in children. In childhood PAP, genetic testing often identified an underlying cause, especially when other organs were also affected.
- Animal studies only. Early animal experiments improved PAP-like markers, but these approaches have not been shown to work in people.
April 2026
- Inhaled GM-CSF and oxygen transfer. In autoimmune pulmonary alveolar proteinosis (PAP), inhaled GM-CSF was linked with improved oxygen-related measures of lung function.
- Breathlessness scores. People receiving inhaled GM-CSF reported lower shortness-of-breath scores.
- Oxygen changes during lavage. During whole lung lavage, oxygen levels may rise during saline filling and fall during drainage, so close monitoring by procedure phase matters.
March 2026
- Whole-lung lavage effects. Whole-lung lavage was often followed by easier breathing and higher oxygen levels.
- More than one lavage. Some people needed repeat lung-wash procedures over time.
- Infection and procedure timing. When a serious infection was present, the timing and results of lung washing were less predictable.
- CT and lab stain support. Certain CT patterns and a Napsin-A stain on bronchoalveolar lavage samples were reported to help support the diagnosis in some settings.
February 2026
- How long improvement lasts. Some difficult PAP cases improved after a suspected immune driver or trigger was addressed, but several reports also described relapse after improvement, so long-term control remains uncertain.
- Possible triggers in context. One secondary PAP case improved soon after stopping tofacitinib, while another report described PAP-like findings during severe flea-borne typhus, making cause-and-effect harder to interpret during acute illness.
- Markers linked to severity. In autoimmune PAP, higher extracellular DNA in lung wash fluid tracked worse oxygen transfer, and very high KL-6 blood levels were reported in a PAP subgroup within a large interstitial lung disease dataset.
- Macrophage biology in models. Animal studies continued to point to lung macrophage function as a key limit on clearing surfactant and lipids, including an inhaled GM-CSF mRNA approach that reduced surfactant buildup in mice but remains preclinical.
January 2026
- Small-cryoprobe lung biopsy. In complex lung disease evaluations, transbronchial cryobiopsy using a very small cryoprobe often provided enough tissue for a clear diagnosis, with mostly mild complications and rare serious events.
- PAP during severe infection. When severe infections occur, pulmonary alveolar proteinosis can be harder to interpret because imaging and test results may not fit together clearly.
- Anti-GM-CSF antibody confirmation. In one complex case, anti-GM-CSF antibody testing confirmed autoimmune pulmonary alveolar proteinosis, and inhaled GM-CSF was followed by partial improvement in scans and oxygen measures.
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