2026 ICD-10-CM Diagnosis Code E84.0Cystic fibrosis with pulmonary manifestations

ICD-10-CM CodesE00–E89E70-E88E84

ICD-10-CM E84.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E84.0 is a billable ICD-10-CM diagnosis code for cystic fibrosis with pulmonary manifestations. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 177 through 179. Coders also document this condition as cystic fibrosis of the lung. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Cystic fibrosis.

Code Identity

ICD-10-CM Code
E84.0
Billable Status
Yes — Valid for Submission
Code Describes
Cystic fibrosis with pulmonary manifestations
Short Description
Cystic fibrosis with pulmonary manifestations
Same as the full description in the CMS dataset.
Parent Code
Cystic fibrosis

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE84Cystic fibrosis
This CodeE84.0Cystic fibrosis with pulmonary manifestations

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Cystic fibrosis of the lung

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Use Additional Code

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

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

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

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END012
Cystic fibrosis
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Calgranulin A

    a 10.8-kda member of the s-100 family of calcium-binding proteins that can form homo- or heterocomplexes with calgranulin b and a variety of other proteins. the calgranulin a/b heterodimer is known as leukocyte l1 antigen complex. calgranulin a is found in many cell types including granulocytes; keratinocytes; and myelomonocytes, and has been shown to act as a chemotactic substance for neutrophils. because it is present in acute inflammation but absent in chronic inflammation, it is a useful biological marker for a number of pathological conditions.
  • Cystic Fibrosis

    an autosomal recessive genetic disease of the exocrine glands. it is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator expressed in several organs including the lung, the pancreas, the biliary system, and the sweat glands. cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in airway obstruction; chronic respiratory infections; pancreatic insufficiency; maldigestion; salt depletion; and heat prostration.
  • Cystic Fibrosis Transmembrane Conductance Regulator

    a chloride channel that regulates secretion in many exocrine tissues. abnormalities in the cftr gene have been shown to cause cystic fibrosis. (hum genet 1994;93(4):364-8)

Patient EducationClinical

Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E84.0 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.02 Cystic fibros w pul man
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E84.0Overview

Is E84.0 (Cystic fibrosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report cystic fibrosis with pulmonary manifestations on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does E84.0 group to?

When cystic fibrosis with pulmonary manifestations is the principal diagnosis on an inpatient stay, it groups to MS-DRG 177, 178, 179, with relative weights from 0.7550 to 1.5627 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of E84.0?

Under the General Equivalence Mappings, cystic fibrosis with pulmonary manifestations converts to ICD-9-CM 277.02 (cystic fibros w pul man). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.