बढ़े हुए GM-CSF ऑटोएंटीबॉडी के साथ ऑटोइम्यून PAP — जब रिटुक्सिमैब अपेक्षित लक्ष्य प्राप्त नहीं कर सका

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 the use of plasmapheresis for patients with confirmed aPAP who remain symptomatic, requiring high flow of supplemental oxygen (≥4 L · min−1) or two or more WLL over a period of a year, despite receiving exogenous GM-CSF and rituximab, or having previously failed these treatments (conditional recommendation, very low certainty).

Plasmapheresis was effective in four cases where symptom, oxygenation, radiological or lung function improvements were described.

Most patients (five of nine) had a reduction in GM-CSF antibody titres after plasmapheresis.