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Therapeutic effectiveness of rituximab in a patient with unresponsive autoimmune pulmonary alveolar proteinosis
  1. Anat Amital1,
  2. Shlomo Dux2,5,
  3. David Shitrit3,5,
  4. Ofer Shpilberg4,5,
  5. Mordechai R Kramer1,5
  1. 1Pulmonary Institute Rabin Medical Center Beilinson Campus, Petah Tiqwa, Israel
  2. 2Department of Internal Medicine C, Rabin Medical Center Beilinson Campus, Petah Tiqwa, Israel
  3. 3Pulmonary Department, Meir Medical Center, Kfar Saba, Israel
  4. 4Department of Hematology, Rabin Medical Center Beilinson Campus, Petah Tiqwa, Israel
  5. 5Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
  1. Correspondence to Anat Amital, Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petah Tiqwa 49100, Israel; aamital{at}


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.

  • Alveolar proteinosis

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  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval Treatment was approved by the hospital drug approval committee.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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