title: Experience with treatment of pulmonary alveolar proteinosis from a tertiary care centre in north India.
abstract: BACKGROUND
  Pulmonary alveolar proteinosis (PAP) is a disorder characterised by accumulation of lipids and proteins in the alveoli, with the resultant symptoms ranging from indolent subclinical disease to progressive respiratory failure.
  
  
  METHODS
  We retrospectively studied five patients with PAP managed at our center between January 2007 and April 2010, with whole lung lavage (WLL) and/or subcutaneous granulocyte macrophage-colony stimulating factor (GM-CSF) therapy. Patients undergoing WLL under general anaesthesia were supplemented with three months of GM-CSF therapy. Pre- and post-lavage symptom assessment was performed with a 10-point, symptom-based visual analogue scale.
  
  
  RESULTS
  Their mean age was 37.6-7.0 years; there were four males. Diagnosis of PAP [idiopathic (n=3); secondary to Nocardia (n=1)] was established by surgical lung biopsy in four patients who presented with respiratory failure. Three patients with idiopathic PAP (n=3) were treated with a combination of GM-CSF and WLL; one patient with secondary PAP was treated with antibiotics alone. In another patient transbronchial lung biopsy was used to diagnose PAP and GM-CSF alone was administered. All patients were followed up for a median period of two years (range 0.5-3 years). Significant improvement was achieved in all the patients with therapeutic WLL and/or GM-CSF.
  
  
  CONCLUSIONS
  Whole lung lavage appeared to be an effective and safe therapy in patients with PAP. Efficacy of simultaneous administration of GM-CSF and WLL in the treatment of PAP merits further study.
authors.affiliation: Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India. ashutosh@indiachest.org
authors.forename: A
authors.initials: A
authors.lastname: Khan
authors.forename: R
authors.initials: R
authors.lastname: Agarwal
authors.forename: A N
authors.initials: AN
authors.lastname: Aggarwal
authors.forename: Amanjit
authors.initials: A
authors.lastname: Bal
authors.forename: Indu
authors.initials: I
authors.lastname: Sen
authors.forename: L N
authors.initials: LN
authors.lastname: Yaddanapuddi
authors.forename: G D
authors.initials: GD
authors.lastname: Puri
mesh_terms: D000328:Adult; D018893:Bronchoalveolar Lavage; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D007194:India; D008297:Male; D011649:Pulmonary Alveolar Proteinosis / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies
chemical_list: D016178:Granulocyte-Macrophage Colony-Stimulating Factor