ICD-10 Code E84.0 – Cystic fibrosis with pulmonary manifestations (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E84.0 – Cystic fibrosis with pulmonary manifestations
What it is
E84.0 identifies cystic fibrosis when the disease is documented with lung or airway involvement. Use it when the record links cystic fibrosis to pulmonary manifestations such as chronic respiratory disease.
Clinical signs
Clinical features vary; refer to documentation. Common pulmonary findings in cystic fibrosis include chronic cough, recurrent respiratory infections, wheezing, and thick airway secretions.
When to use this code
Use E84.0 when the provider documents cystic fibrosis with pulmonary manifestations or a respiratory complication clearly attributed to cystic fibrosis. It fits encounters focused on chronic lung disease, airway clearance problems, or recurrent chest infections in a patient with cystic fibrosis.
If the note mentions cystic fibrosis but does not specify pulmonary involvement, check documentation before coding. Assign the code only when the respiratory manifestation is documented or clearly linked to the diagnosis.
Do not use for
Do not use this code for cystic fibrosis without pulmonary manifestations. Do not assign it when the respiratory problem is documented separately and not linked to cystic fibrosis.
Coding tip
Verify that the pulmonary manifestation is explicitly documented with cystic fibrosis before assigning E84.0.