E84.0 Cystic fibrosis with pulmonary manifestations

Billable ICD-10-CM code, FY2026. Acts as a Major Complication or Comorbidity (MCC) as a secondary diagnosis.

Use additional code

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

Applies from E84 Cystic fibrosis

Includes

• mucoviscidosis

Code also

• exocrine pancreatic insufficiency (K86.81)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 277 — Cystic Fibrosis
• CMS-HCC V22: HCC 110 — Cystic Fibrosis
• RxHCC V08: HCC 225 — Cystic Fibrosis
• ESRD V24: HCC 110

MS-DRG index

Defines the logic of DRG(s) 177-179 in MDC 04.

Approximate ICD-9-CM (GEMs)

• 277.02 Cystic fibrosis with pulmonary manifestations

Code annotations containing back-references to E84.0

Back-references to E84.0

• Code Also: I27.23 Pulmonary hypertension due to lung diseases and hypoxia ("cystic fibrosis with pulmonary manifestations (E84.0)")
• Type 1 Excludes: J99 Respiratory disorders in diseases classified elsewhere ("cystic fibrosis with pulmonary manifestations (E84.0)")
• Type 1 Excludes: P78 Other perinatal digestive system disorders ("cystic fibrosis (E84.0-E84.9)")
• Type 1 Excludes: Q33.0 Congenital cystic lung ("cystic fibrosis (E84.0)")
• Type 1 Excludes: Q45.3 Other congenital malformations of pancreas and pancreatic duct ("cystic fibrosis (E84.0-E84.9)")

Back-references to E84

• Code First: E08 Diabetes mellitus due to underlying condition ("cystic fibrosis (E84.-)")
• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Code First: J93.12 Secondary spontaneous pneumothorax ("cystic fibrosis (E84.-)")
• Type 2 Excludes: K86 Other diseases of pancreas ("fibrocystic disease of pancreas (E84.-)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: Q45.3 Other congenital malformations of pancreas and pancreatic duct ("fibrocystic disease of pancreas (E84.-)")
• Code First: Q55.3 Atresia of vas deferens ("any associated cystic fibrosis (E84.-)")
• Code Also: Z71.87 Encounter for pediatric-to-adult transition counseling ("cystic fibrosis (E84.-)")

Diagnosis Index entries for E84.0

• Fibrosis, fibrotic › cystic (of pancreas) › with › pulmonary manifestations

Nearby codes

• E84 Cystic fibrosis
• E84.0 Cystic fibrosis with pulmonary manifestations (this code)
• E84.1 Cystic fibrosis with intestinal manifestations
• E84.11 Meconium ileus in cystic fibrosis
• E84.19 Cystic fibrosis with other intestinal manifestations
• E84.8 Cystic fibrosis with other manifestations
• E84.9 Cystic fibrosis, unspecified