E84.0Cystic fibrosis with pulmonary manifestations

E84.0 is the ICD-10-CM diagnosis code for cystic fibrosis with pulmonary manifestations. It belongs to category E84 (cystic fibrosis), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E84 (cystic fibrosis), E84.0 is specifically for cystic fibrosis with pulmonary manifestations. Related codes cover with intestinal manifestations (E84.1), with other manifestations (E84.8) and unspecified (E84.9).

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

As a secondary diagnosis it is an MCC (major complication or comorbidity), which can move an inpatient stay into the highest-paying "with MCC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 177 (Respiratory Infections and Inflammations with MCC, relative weight 1.6092), DRG 178 (Respiratory Infections and Inflammations with CC, relative weight 0.9744) and DRG 179 (Respiratory Infections and Inflammations without CC/MCC, relative weight 0.7345), in MDC 04 (Diseases and Disorders of the Respiratory System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 277.02 (cystic fibrosis with pulmonary manifestations).