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Table 2 Main diseases that can benefit from NIPPV classified according to the cause and progressiveness of the respiratory impairment

From: Clinical review: Long-term noninvasive ventilation

Parietal disorders (PFT abnormal; ↓ VC, ↓ FEV1, → FEV1/VC, ↓ RV, ↓ TLC)

   Chest wall



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)


Continuous worsening

   Bronchiectasis, cystic fibrosis

Continuous worsening

Predominant ventilatory control abnormalities (PFT normal)

   Ondine's curse


   Cheyne-Stokes breathing

Depends on heart failure

Upper airway abnormalities (PFT normal)

   Obstructive sleep apnea

No worsening

  1. Symbols indicate actual compared to theoretical values: ↓, decrease; ↑, increase; →, normal. COPD, chronic obstructive pulmonary disease; FEV1, forced expiratory volume in 1 second; NIPPV, noninvasive positive pressure ventilation; PFT, pulmonary function test; RV, residual volume; TLC, total lung capacity; VC, vital capacity.