Clinical and bronchoalveolar lavage findings before and during a 12 week treatment course with daily subcutaneous granulocyte-macrophage colony-stimulating factor (GM-CSF) for the treatment of idiopathic pulmonary alveolar proteinosis
| Before GM-CSF | 6 weeks GM-CSF | 12 weeks GM-CSF | |
|---|---|---|---|
| VC = vital capacity; FEV1 = forced expiratory volume in 1 second; TLC = total lung capacity; Tlco = carbon monoxide transfer factor; Po2, Pco2 = oxygen and carbon dioxide tensions; A–ao2 = alveolar-arterial oxygen gradient; Vo2max = maximal oxygen uptake; a = distinction between foamy and ghost-like macrophages not possible because of cell damage and debris. *BAL of two lingular segments pooled. †By haemocytometer. ‡Manual count of 2 × 200 cells, Giemsa. §5000 cells counted by flow cytometry. | |||
| Lung volumes | |||
| VC , l (% pred) | 1.52 (55%) | 2.16 (78%) | 2.76 (99%) |
| FEV1, l (% pred) | 1.35 (57%) | 1.73 (73%) | 2.17 (91%) |
| TLC, l (% pred) | 2.17 (51%) | 2.80 (66%) | 3.42 (81%) |
| Tlco, mmol/kPa/min (% predicted) | 2.3 (31%) | 3.4 (44%) | 4.9 (64%) |
| Blood gases at rest | |||
| Po2 (kPa) | 5.7 | 8.7 | 10.7 |
| Pco2 (kPa) | 5.0 | 4.9 | 5.6 |
| A–ao2 (kPa) | 7.3 | 4.1 | 1.2 |
| Peak performance (cycle ergometer) | |||
| Vo2max, ml/kg/min (% pred) | 16 (48%) | 24 (71%) | 28 (86%) |
| Work rate, watt (% pred) | 64 (65%) | 84 (84%) | 113 (114%) |
| A–ao2 (kPa) at peak performance | 7.3 | 6.8 | 4.8 |
| Bronchoalveolar lavage* | |||
| Recovery (% of 500 ml) | 80% | 76% | 68% |
| Total protein, g/l | 2.0 | 0.2 | 0.2 |
| Total cell count†, cells/μl | 600 | 500 | 400 |
| Macrophages‡, cells/μl (%) | 300 (50%) | 295 (59%) | 236 (59%) |
| Small monocyte-like cells | 0% | 33% | 10% |
| Foamy macrophages | a | 62% | 90% |
| Ghost-like huge macrophages | a | 3% | 0% |
| Granulocytes‡, cells/μl (%) | 24 (4%) | 15 (3%) | 4 (1%) |
| Lymphocytes‡, cells/μl (%) | 276 (46%) | 190 (38%) | 160 (40%) |
| CD3+§ | 96% | 97% | 96% |
| CD4+§ | 75% | 55% | 82% |
| CD8+§ | 20% | 39% | 12% |
| Anti-GM-CSF antibody titre | |||
| Serum (μg/ml) | 35.2 | 31.0 | 20.6 |
| BAL fluid (μg/ml) | 4.4 | 0.14 | 0.13 |