E84.0

Cystic fibrosis with pulmonary manifestations

FY2027Chapter 4: Endocrine, nutritional and metabolic diseases/E70–E88 Metabolic disorders

BillableComplete code, valid to report

E84.0 (also written E840) is a billable ICD-10-CM diagnosis code for cystic fibrosis with pulmonary manifestations. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 5 codes in category E84 (Cystic fibrosis). The Alphabetic Index lists it under 1 entry. Its coding notes include a Use additional code instruction, a Code also instruction, 6 Excludes1 entries and 1 Excludes2 entry.

Use additional code

Add the listed code as a secondary code, if known.

This code
  • code to identify any infectious organism present, such as:

Code also

Two codes may be needed to describe the condition. This note gives no sequencing order.

Inherited from E84 Cystic fibrosis
  • exocrine pancreatic insufficiency (K86.81)

Excludes1

Not coded here. The listed conditions generally should not be reported together.

Also from chapter/block level (6 notes)
Inherited from E70–E88 Metabolic disorders
  • 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)
Inherited from Chapter 4 Endocrine, nutritional and metabolic diseases
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes2

Not included here. Both codes may be reported if the patient has both conditions.

Also from chapter/block level (1 note)
Inherited from E70–E88 Metabolic disorders
  • Ehlers-Danlos syndromes (Q79.6-)

Care settings

How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.

Home Health Primary

Accepted as the primary (principal) diagnosis on a home health claim. The Home Health Grouper assigns it to the MMTA - Endocrine clinical group.

Source: CMS Home Health Grouper Software v07.2.26, effective October 1, 2026

Hospital MCC

Counts as a major complication or comorbidity (MCC) when reported as a secondary diagnosis on an inpatient claim. It does not count when the principal diagnosis is one of the 5 codes in its exclusion list. Some MS-DRGs also ignore a CC or MCC that defines the DRG.

Source: CMS MS-DRG Definitions Manual v44, effective October 1, 2026, Appendix C

Includes

Further defines or gives examples of the content of the category.

Inherited from E84 Cystic fibrosis
  • mucoviscidosis

Notes

Also from chapter/block level (1 note)
Inherited from Chapter 4 Endocrine, nutritional and metabolic diseases
  • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Alphabetic Index terms

Terms the official Alphabetic Index lists for this code. 1 entry