Table 1

 Clinical characteristics of patients with idiopathic pulmonary alveolar proteinosis

CaseAge at diagnosis (years)Sex(M/F)Serum LDH(IU/l)*Pao2(kPa)†AaPo2(kPa)†Length of follow up(years)Clinical courseStatus at last follow up
*LDH, lactate dehydrogenase (reference value 95–213 IU/l).
†Pao2 and AaPo2 were measured in the sitting position at room air.
AaPo2  =  Pao2 – Pao2; Pao2  =  (barometric pressure − 47) × Fio2 − Paco2/0.8.
TLL, therapeutic lung lavage; SI, spontaneous improvement; DOE, dyspnoea on exertion.
153M23910.83.44StableSymptom free
240F3896.47.88TLL at diagnosisO2 treatment, repeated TLL
340M26911.31.65StableSymptom free
431M2929.73.34TLL at diagnosisSymptom free
516M2808.85.23SISymptom free
618F28910.83.46SISymptom free
754F24210.72.86StableMild DOE
836F17510.93.13StableSymptom free
959F2658.16.04TLL 2 years after diagnosisSymptom free
1033M19110.13.56SISymptom free
1134M2439.44.12StableMild DOE
1245M3896.58.01TLL at diagnosisSymptom free
1342M3925.29.50.5TLL at diagnosisSymptom free