Therapeutic effectiveness of rituximab in a patient with unresponsive autoimmune pulmonary alveolar proteinosis.

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterised by the accumulation of lung surfactant in the alveoli. In most cases it is an autoimmune disease with antibodies directed against the growth factor granulocyte-macrophage colony stimulating factor (GM-CSF). Standard of care consists of whole lung lavages in symptomatic patients. An alternative treatment is GM-CSF injections. The case history is reported of a patient with PAP and severe dyspnoea and hypoxaemia. Whole lung lavages and GM-CSF initially resulted in partial remission. However, the patient's condition deteriorated and her saturation during rest with high-flow oxygen treatment was 85%. The patient was treated with an anti-CD20 antibody rituximab which resulted in dramatic improvement. Room air saturation increased to 98% with exercise and she no longer required supplemental oxygen. The diffusion capacity for carbon monoxide increased from 27% to 48% of predicted and the chest x-rays improved. Rituximab may be useful in the treatment of patients with unresponsive PAP.

• Authors: Anat Amital, Shlomo Dux, David Shitrit, Ofer Shpilberg, Mordechai R Kramer
• Journal: Thorax (Thorax) Vol. 65 Issue 11 Pg. 1025-6 (Nov 2010) ISSN: 1468-3296 [Electronic] England
• PMID: 20855439 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Antigens, CD20
• Immunosuppressive Agents
• Rituximab

Topics

• Adult
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Antigens, CD20 (immunology)
• Autoimmune Diseases (drug therapy, physiopathology)
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Pulmonary Alveolar Proteinosis (drug therapy, physiopathology)
• Rituximab