E84.0

Billable

Cystic fibrosis with pulmonary manifestations

HCC Category Mapping

V28HCC 277 — Cystic Fibrosis

0.405

V24HCC 110 — Cystic Fibrosis

0.473

ESRDHCC 110 — Cystic Fibrosis

0.000

RxHCCHCC 225 — Cystic Fibrosis

0.000

What This Code Means

Cystic fibrosis is a genetic disorder affecting the lungs and digestive system; this code specifically indicates lung involvement with symptoms like chronic cough and breathing difficulties.

Coding Tips

•Use this code when cystic fibrosis primarily manifests with pulmonary symptoms; document specific lung complications separately if present
•This is the most common presentation of CF; ensure you're not using E84.9 (unspecified) if pulmonary involvement is documented

Clinical Significance

Cystic fibrosis with pulmonary manifestations is the most common presentation of this life-limiting genetic disease, involving chronic progressive lung disease with recurrent infections, bronchiectasis, and eventual respiratory failure. It carries a high RAF weight reflecting the substantial healthcare resource utilization and complexity of care.

Documentation Requirements

✓Confirmed diagnosis of cystic fibrosis (CFTR mutation testing or sweat chloride test)
✓Documentation of specific pulmonary manifestations (chronic bronchitis, bronchiectasis, recurrent pneumonias, lung function decline)
✓Current pulmonary function test results (FEV1)
✓Active treatment regimen (CFTR modulators, airway clearance, inhaled antibiotics, pancreatic enzymes)
✓Pulmonary exacerbation history and hospitalizations
✓Microbiology results (Pseudomonas, MRSA colonization status)

Use Additional Code

code to identify any infectious organism present, such as:
Pseudomonas (B96.5)

Commonly Confused Codes

E84.9 — Cystic fibrosis, unspecified: use only when manifestation type is unknown; E84.0 is preferred when pulmonary involvement is documented E84.11 — Meconium ileus in cystic fibrosis: GI manifestation, not pulmonary E84.19 — Cystic fibrosis with other intestinal manifestations: GI-focused manifestation E84.8 — Cystic fibrosis with other manifestations: for non-pulmonary, non-intestinal manifestations J47.9 — Bronchiectasis, uncomplicated: if caused by cystic fibrosis, use E84.0 instead

Code Hierarchy

└E84Cystic fibrosis └E84.0Cystic fibrosis with pulmonary manifestations

└E84.0Cystic fibrosis with pulmonary manifestations