Table 2 Main diseases that can benefit from NIPPV classified according to the cause and progressiveness of the respiratory impairment
Parietal disorders (PFT abnormal; ↓ VC, ↓ FEV1, → FEV1/VC, ↓ RV, ↓ TLC) | |
   Chest wall |  |
Kyphoscoliosis | No worsening |
Sequelae of tuberculosis | Slow worsening |
Obesity hypoventilation syndrome | Depends on obesity |
   Neuromuscular disorders |  |
Spinal muscular atrophy | No worsening |
Acid maltase deficit | Slow worsening (>15 years) |
Duchenne muscular dystrophy | Intermediate worsening (5 to 15 years) |
Myotonic myopathy | Intermediate worsening (5 to 15 years) |
Amyotrophic lateral sclerosis | Rapid worsening (0 to 3 years) |
Lung diseases (PFT abnormal; → or ↓ VC, ↓ FEV1, ↓ FEV1/VC, ↑ RV, ↑ TLC) | |
   COPD | Continuous worsening |
   Bronchiectasis, cystic fibrosis | Continuous worsening |
Predominant ventilatory control abnormalities (PFT normal) | |
   Ondine's curse | Improvement? |
   Cheyne-Stokes breathing | Depends on heart failure |
Upper airway abnormalities (PFT normal) | |
   Obstructive sleep apnea | No worsening |