TABLE 1.
Comparison of baseline characteristics between patients without fibrosis and patients with fibrosis on initial and on follow-up computed tomography (CT)
| Patients without fibrosis (n=45) | Patients with fibrosis on initial CT (n=5) | Patients with fibrosis on final CT (n=11) | Patients with fibrosis on initial or on final CT (n=16) | |
|---|---|---|---|---|
| Baseline characteristics at aPAP diagnosis | ||||
| Age, years | 41.0 (33.0–49.0) | 48.0 (47.0–56.0) | 41.0 (39.5–54.0)* | 47.6 (40.5–55.0)# |
| Male gender | 26 (58%) | 4 (80%) | 7 (64%) | 11 (69%) |
| GM-CSF titre | 600 (245–1155) | 300 (115–850) | 300 (100–750) | 300 (115–850) |
| Comorbidities | ||||
| Active smokers | 20 (47%) | 3 (60%) | 6 (55%) | 9 (56%) |
| COPD | 2 (4.5%) | 0 (0%) | 1 (9.1%) | 1 (6.2%) |
| Asthma | 4 (9.5%) | 0 (0%) | 1 (9.1%) | 1 (6.2%) |
| Dust exposure¶ | 12 (28%) | 3 (60%) | 7 (64%)* | 10 (62%)# |
| Diabetes mellitus | 3 (6.8%) | 0 (0%) | 2 (18%) | 2 (12%) |
| High blood pressure | 10 (23%) | 0 (0%) | 4 (36%) | 4 (25%) |
| Dyslipidaemia | 4 (9.1%) | 0 (0%) | 1 (9.1%) | 1 (6.2%) |
| Symptoms at diagnosis | ||||
| No symptoms | 5 (13%) | 0 (0%) | 1 (9.1%) | 1 (6.2%) |
| Cough | 20 (51%) | 3 (60%) | 7 (64%) | 10 (62%) |
| Dyspnoea | 28 (65%) | 5 (100%) | 10 (91%)* | 15 (94%)# |
| Sputum | 6 (15%) | 3 (60%) | 2 (18%) | 5 (31%) |
| Digital clubbing | 1 (2.6%) | 1 (20%) | 1 (9.1%) | 2 (12%) |
| Fever | 4 (10%) | 0 (0%) | 1 (9.1%) | 1 (6.2%) |
| Weight loss | 12 (32%) | 0 (0%) | 2 (18%) | 2 (13%) |
| Oxygen need | 8 (18%) | 2 (40%) | 3 (27%) | 5 (31%) |
| Functional data at aPAP diagnosis | ||||
| FVC (% predicted) | 82.5 (78.0–91.8) | 88.4 (80.0–90.0) | 86.5 (71.2–101) | 87.9 (73.2–103) |
| FEV1 (% predicted) | 83.2 (76.2–91.5) | 90.0 (87.0–90.8) | 85.0 (75.0–95.0) | 88.8 (80.0–97.0) |
| DLCO (% predicted) | 53.0 (36.8–70.0) | 60.0 (47.5–65.0) | 58.4 (44.0–63.5) | 59.0 (42.0–65.0) |
| 6MWT, m | 510 (472–521) | 490 (468–499) | 498 (470–509) | −2.00 (−4.00–9.00) |
| Desaturation during 6MWT | −0.500 (−6.50–5.75) | −8.00 (2.00–8.00) | −2.00 (−3.00–14.0) | 7.00 (2.00–9.00) |
| PaO2, mmHg | 72.0 (64.0–88.0) | 59.0 (47.0–61.0) | 68.0 (57.0–83.0) | 67.0 (56.0–82.0) |
| PCO2, mmHg | 34.7 (32.0–39.0) | 35.0 (34.2–35.8) | 36.0 (32.5–37.5) | 35.0 (32.8–37.2) |
| Treatments | ||||
| Whole-lung lavage | 25 (57%) | 3 (60%) | 7 (64%) | 10 (62%) |
| Inhaled GM-CSF therapy | 8 (21%) | 0 (0%) | 4 (36%) | 4 (25%) |
| Subcutaneous injection of GM-CSF | 15 (36%) | 1 (20%) | 4 (36%) | 5 (31%) |
| Rituximab | 12 (29%) | 2 (40%) | 1 (9.1%) | 3 (19%) |
| Other immunosuppressive treatment | 1 (2.4%) | 0 (0%) | 1 (10%) | 1 (7.1%) |
| Plasmapheresis | 0 (0%) | 0 (0%) | 1 (10%) | 1 (6.7%) |
| Infections | ||||
| All infections | 19 (42%) | 4 (80%) | 7 (64%) | 11 (69%) |
| Non-opportunistic infections | 16 (36%) | 2 (40%) | 6 (55%) | 8 (50%) |
| Opportunistic infections | 9 (20%) | 3 (60%) | 4 (36%) | 7 (44%) |
| Nocardia spp. | 4 (8.9%) | 0 (0%) | 1 (9.1%) | 1 (6.2%) |
| Clinical outcomes | ||||
| Complete resolution | 13 (32%) | 3 (60%) | 3 (30%) | 6 (40%) |
| All-cause mortality | 2 (4.4%) | 1 (20%) | 3 (27%)* | 4 (25%)# |
Data are expressed as median (interquartile range) or number (%). aPAP: autoimmune pulmonary alveolar proteinosis; GM-CSF: granulocyte–monocyte colony-stimulating factor; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide; 6MWT: 6-min walking test; PaO2: arterial oxygen tension; PCO2: carbon dioxide tension. ¶: Dust exposure includes patients who had worked in the building sector. *: p<0.05 compared with the column of 45 patients without fibrosis. #: p<0.05 compared with the column of 45 patients without fibrosis. Bold indicates significant values.