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ICD-10/E84.0

E84.0 ICD-10-CM Code: Cystic fibrosis with pulmonary manifestations

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Inpatient Payment Groups (MS-DRG)

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43.0 (FY2026), Appendix B.

  • MS-DRG 177 — RESPIRATORY INFECTIONS AND INFLAMMATIONS WITH MCC (MDC 04)
  • MS-DRG 178 — RESPIRATORY INFECTIONS AND INFLAMMATIONS WITH CC (MDC 04)
  • MS-DRG 179 — RESPIRATORY INFECTIONS AND INFLAMMATIONS WITHOUT CC/MCC (MDC 04)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Risk Adjustment (CMS-HCC)

Payment categories this diagnosis maps to under CMS-HCC V28, payment year 2026.

  • HCC 277 — Cystic Fibrosis (supersedes HCC 278, HCC 279, HCC 280)

Other models: CMS-HCC V22 HCC 110 · RxHCC V08 HCC 225

Risk scores depend on the enrollee's full accepted diagnosis set and segment; a category mapping alone does not determine payment.

Code Set: Valid FY2026 and FY2027. FY2026 took effect October 1, 2025.

Official Registry Overview & Definition

Cystic fibrosis with pulmonary manifestations is a billable ICD-10-CM diagnosis code (E84.0).

Official Tabular Instructional Notes

Sequencing, inclusion, and exclusion notes published for E84.0 in the official ICD-10-CM tabular list.

Includes

Conditions the official ICD-10-CM tabular list includes under this code.

  • mucoviscidosis

Excludes1 — Not Coded Here

Conditions that can never be reported with this code; the two are mutually exclusive.

  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

  • Ehlers-Danlos syndromes (Q79.6-)

Code Also

Additional codes that may be required to fully describe the encounter.

  • exocrine pancreatic insufficiency (K86.81)

Use Additional Code

Supplementary codes the tabular list directs you to add.

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

Nearest Codes in This Family

Official ICD-10-CM classifications closest to E84.0 in its code family, with their registry titles.

  • E84 — Cystic fibrosis
  • 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

View all codes in the E84 family

Indexed Clinical Terms (1)

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code.

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