Table 1.
Demographics of the 81 subjects with surfactant associated disorders
n. | % | |
---|---|---|
Classification |
|
|
Primary PAP |
|
|
Idiopathic + autoimmune1 |
73 |
90 |
Hereditary2 |
1 |
1 |
Secondary PAP3 |
3 |
4 |
PAP-like4 |
4 |
5 |
Gender |
|
|
Male |
54 |
66.5 |
Female |
27 |
33.5 |
Smoking status |
|
|
Current |
18 |
22 |
Former |
34 |
42 |
Never | 29 | 36 |
1 We began to measure serum GMAb level in 2004, thus for accuracy in this group we included patients with idiopathic PAP (n = 42, those diagnosed before 2004) and patients with autoimmune PAP (n = 31, those diagnosed after 2004), although currently they are considered synonymous. 2 This subject was affected by PAP due to a GM-CSF receptor αchain mutation [17]. 3 This group includes two subjects with PAP secondary to hematologic disorders (1 chronic myeloid leukemia and 1 myelodysplastic syndrome) and 1 subject affected by lysinuric protein intolerance (the latter’s case history has already been reported) [18,19]. 4 This group includes four subjects with a mutation in the ABCA3 gene (manuscript in preparation).