Autoimmune Pulmonary Alveolar Proteinosis When Plasmapheresis Has Not Reduced GM-CSF Antibody Titres
Clinical scenario
This protocol applies to patients with confirmed autoimmune pulmonary alveolar proteinosis (aPAP) who have raised GM-CSF autoantibody titres and whose disease remains active or continues to worsen. Objective GM-CSF antibody measurement underpins both diagnosis and the decision to escalate treatment in this setting.
Prior treatment — Plasmapheresis did not achieve its goals
This is the next-line protocol for patients in whom Plasmapheresis failed to meet the following targets:
- Reduction in GM-CSF antibody titres
- Improvement in symptoms, oxygenation, radiological findings, or lung function
Next-line approach
For patients whose disease continues to progress despite prior treatment lines and who meet established eligibility criteria, a surgical intervention targeting the lungs may be considered. Full eligibility requirements and the complete protocol are available via the link below.
References
DOI: 10.1183/20734735.0224-2024
- Treatment is indicated in patients with active or worsening disease.
- GM-CSF antibody measurement is objective, reproducible and has high accuracy for diagnosing aPAP with a level of 10.2 µg·mL−1 or above the threshold of the individual laboratory references.
- We suggest lung transplantation for patients with PAP progressing despite WLL and/or pharmacological treatment, who fulfil the International Society for Heart and Lung Transplantation (ISHLT) criteria for patients with ILD (conditional recommendation, very low certainty of evidence).
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