The Novel Use of Daratumumab in the Treatment of Refractory Autoimmune Pulmonary Alveolar Proteinosis
ABSTRACT Autoimmune pulmonary alveolar proteinosis (aPAP) is caused by circulating anti‐granulocyte‐macrophage colony‐stimulating factor (GM‐CSF) auto‐antibodies that impair alveolar macrophage and neutrophil function. Macrophage dysfunction leads to accumulation of protein‐ and lipid‐rich surfactan...
Saved in:
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2025-06-01
|
| Series: | Respirology Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/rcr2.70246 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| Summary: | ABSTRACT Autoimmune pulmonary alveolar proteinosis (aPAP) is caused by circulating anti‐granulocyte‐macrophage colony‐stimulating factor (GM‐CSF) auto‐antibodies that impair alveolar macrophage and neutrophil function. Macrophage dysfunction leads to accumulation of protein‐ and lipid‐rich surfactant within alveoli, impairing gas exchange and leading to respiratory failure. Standard treatments include whole lung lavage (WLL) as first‐line therapy, nebulised replacement of GM‐CSF, and in refractory cases, lymphocyte depletion, immunosuppression, or lung transplantation. We present a case of a 51‐year‐old patient with severe, treatment‐refractory aPAP failing all standard PAP therapies. A compassionate access program enabled commencement of Daratumumab, a CD38‐directed monoclonal antibody (mAb), targeting long‐lived plasma cells and providing a novel approach to treatment‐resistant autoimmune conditions. One year post completion of Daratumumab, the patient remained in remission with clinical and radiological improvement. There was a corresponding reduction in anti‐GM‐CSF antibody levels and improvement in gas exchange on pulmonary function testing. |
|---|---|
| ISSN: | 2051-3380 |