2026 ICD-10-CM Diagnosis Code E88.01Alpha-1-antitrypsin deficiency
ICD-10-CM Codes›E00–E89›E70-E88›E88
- Billable — Valid for Submission
- Chronic Condition
E88.01 is a billable ICD-10-CM diagnosis code for Alpha-1-antitrypsin deficiency. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
Code Identity
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Alpha-1-antitrypsin deficiency
- Alpha-1-antitrypsin hepatitis
- Panniculitis due to action of lipolytic enzymes
- Panniculitis due to alpha-1 anti-trypsin deficiency
- Pulmonary emphysema in alpha-1 PI deficiency
Tabular List NotesGuidance
Coding notes and annotation back-references applicable to this code.
Inclusion Terms
- AAT deficiency
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
- alpha-1-antitrypsin - E88.01
- serum antitrypsin, familial - E88.01
External Cause of Injuries IndexGuidance
References for this code in the External Cause of Injuries Index.
- Deficiency, deficient
- alpha-1-antitrypsin
- Deficiency, deficient
- serum antitrypsin, familial
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Patient EducationClinical
Alpha-1 Antitrypsin Deficiency
Alpha-1 antitrypsin deficiency (AAT deficiency, or AATD) is an inherited condition that raises your risk for lung and liver disease. If you have this condition, your body doesn't make enough alpha-1 antitrypsin (AAT).
The full article covers:
- What is alpha-1 antitrypsin deficiency (AAT deficiency)?
- What causes alpha-1 antitrypsin deficiency (AAT deficiency)?
- What are the symptoms of alpha-1 antitrypsin deficiency (AAT deficiency)?
- How is alpha-1 antitrypsin deficiency (AAT deficiency) diagnosed?
- What are the treatments for alpha-1 antitrypsin deficiency (AAT deficiency)?
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E88.01 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E88.01Overview
Is E88.01 a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report Alpha-1-antitrypsin deficiency on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
What is the ICD-9 equivalent of E88.01?
Under the General Equivalence Mappings, Alpha-1-antitrypsin deficiency converts to ICD-9-CM 273.4 (Alpha-1-antitrypsin def). 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.
