J43.0
BillableUnilateral pulmonary emphysema [MacLeod's syndrome]
HCC Category Mapping
V28HCC 280 — Chronic Obstructive Pulmonary Disease
0.334V24HCC 111 — Chronic Obstructive Pulmonary Disease
0.334ESRDHCC 111 — Chronic Obstructive Pulmonary Disease
0.000RxHCCHCC 229 — COPD and Chronic Bronchitis
0.000What This Code Means
A condition where one lung develops abnormal air-filled spaces (emphysema) while the other lung remains relatively normal, often appearing as a collapsed lung on imaging.
Coding Tips
- •Specify laterality (right or left) if the documentation indicates which lung is affected
- •This is also known as MacLeod's syndrome and may be associated with childhood infections or unilateral lung hypoplasia
Clinical Significance
Unilateral pulmonary emphysema (MacLeod's syndrome, also known as Swyer-James syndrome) is a rare condition characterized by hyperlucency of one lung due to underdevelopment of pulmonary vasculature, typically following childhood bronchiolitis obliterans. This is distinct from typical smoking-related emphysema and represents a specific developmental/acquired lung abnormality.
Documentation Requirements
- ✓Imaging (CT scan preferred) demonstrating unilateral hyperlucent lung with air trapping
- ✓Documentation specifying unilateral involvement and the affected side
- ✓Clinical history supporting MacLeod's/Swyer-James syndrome (often childhood respiratory infection)
- ✓Pulmonary function testing showing any obstructive pattern
- ✓Current symptoms and functional impact
- ✓Differentiation from other causes of unilateral hyperlucency (pneumothorax, foreign body)
Commonly Confused Codes
J43.9 — Emphysema, unspecified: use when emphysema type is not specified; J43.0 is more specificJ43.1 — Panlobular emphysema: bilateral condition affecting lower lobes, typically alpha-1 antitrypsin deficiencyJ43.2 — Centrilobular emphysema: bilateral upper-lobe predominant, typically smoking-relatedJ93.x — Pneumothorax: can mimic unilateral hyperlucency but is a different condition entirely