Table 1– Demographic characteristics and clinical features of our cases and cases from the literature#
CharacteristicsOur casesLiterature cases
APAPSPAPSPAP
Subjects n2234057
Age yrs50 (9–89)49 (24–77)40 (24–74)
Sex n
 Males1512331
 Females721726
Clinical symptoms
 Asymptomatic70 (32)9 (23)0 (0)
 Symptomatic150 (68)31 (77)21 (100)
  Dyspnoea on effort116 (53)16 (40)10 (48)
  Febrile2 (1)15 (38)14 (67)
Smoking habits
  Never-smoker93 (43)13 (37)NA
  Current or ex-smoker124 (57)22 (63)NA
Underlying diseases
 Haematological disordersNone35 (88)43 (76)
  Myelodysplastic syndrome266
  Chronic myeloid leukaemia216
  Myelofibrosis21
  Acute myeloid leukaemia18
  Acute lymphoid leukaemia13
  Adult T-cell leukaemia10
  Aplastic anaemia11
  Lymphoma13
  Multiple myeloma01
  Plasmacytoma01
  Alymphoplasia01
  Essential thrombocytosis01
 Infectious diseasesNone2 (5)3 (5)
 Autoimmune diseasesNone3 (7)4 (7)
 Post organ transplantationNone04 (7)
 Non-haematological malignancyNone03 (5)
Diagnostic procedure
 Bronchoscopy208 (93)30 (75)27 (47)
 Video-assisted thoracoscopy15 (7)10 (25)9 (16)
 Open lung biopsy0 (0)0 (0)21 (37)
  • Data are presented as median (range) or n (%), unless otherwise stated. APAP: autoimmune pulmonary alveolar proteinosis; SPAP: secondary pulmonary alveolar proteinosis; NA: not applicable. #: cases from the literature were identified using the search methods detailed in the main text.