PT  - JOURNAL ARTICLE
AU  - Anat Amital
AU  - Shlomo Dux
AU  - David Shitrit
AU  - Ofer Shpilberg
AU  - Mordechai R Kramer
TI  - Therapeutic effectiveness of rituximab in a patient with unresponsive autoimmune pulmonary alveolar proteinosis
AID  - 10.1136/thx.2010.140673
DP  - 2010 Nov 01
TA  - Thorax
PG  - 1025--1026
VI  - 65
IP  - 11
4099  - http://thorax.bmj.com/content/65/11/1025.short
4100  - http://thorax.bmj.com/content/65/11/1025.full
SO  - Thorax2010 Nov 01; 65
AB  - 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.