ICD-10 Code E88.01 – Alpha-1-antitrypsin deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E88.01 – Alpha-1-antitrypsin deficiency
What it is
Alpha-1-antitrypsin deficiency is an inherited disorder where the body makes too little of a protective protein. It can lead to lung disease, liver disease, or both.
Clinical signs
Clinical features vary; refer to documentation. Common findings include early emphysema, chronic dyspnea, wheezing, or liver abnormalities such as hepatomegaly, jaundice, or cirrhosis.
When to use this code
Use E88.01 when the provider documents alpha-1-antitrypsin deficiency as a confirmed diagnosis. It applies whether the condition is found during evaluation of lung disease, liver disease, or family screening. Do not assume the code from low protein levels alone; confirm the documented diagnosis.
Do not use for
Do not use this code for nonspecific chronic obstructive pulmonary disease or unspecified liver disease without documented alpha-1-antitrypsin deficiency. Check documentation if the record only mentions carrier status or family history.
Coding tip
Assign E88.01 only when the chart clearly states alpha-1-antitrypsin deficiency, and code any documented organ manifestations separately.