2027 ICD-10-CM Diagnosis Code E88.01Alpha-1-antitrypsin deficiency
ICD-10-CM Codes›E00–E89›E70-E88›E88
- Billable — Valid for Submission
- Risk Adjusts — HCC 50
- 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 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
For Medicare Advantage risk adjustment, E88.01 maps to CMS-HCC Category 50 (Amyloidosis, Porphyria, and Other Specified Metabolic Disorders) under the V28 model, adding a risk factor of about 0.648 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E88.01 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
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
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E88.01: its own notes plus those printed at E88.0, E88, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- AAT deficiency
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- histiocytosis X (chronic) (C96.6)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- disorder of lipoprotein metabolism (E78.-)
- Ehlers-Danlos syndromes (Q79.6-)
Use Additional Code
Add a second code, after this one, to fully describe the condition.
- codes for associated conditions
Notes
General instructions on how these codes are used.
- 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.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E88.01, its category, or a range that includes it.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
alpha 1-Antitrypsin Deficiency
deficiency of the protease inhibitor alpha 1-antitrypsin that manifests primarily as pulmonary emphysema and liver cirrhosis.
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
What is the ICD-10 code for Alpha-1-antitrypsin deficiency?
The ICD-10-CM code for Alpha-1-antitrypsin deficiency is E88.01 (sometimes written as E8801). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
What MS-DRG does E88.01 group to?
When Alpha-1-antitrypsin deficiency is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can E88.01 be reported with D47.2?
Not as a rule. The Excludes1 note at E88.0, which applies to E88.01, lists "monoclonal gammopathy (of undetermined significance) (D47.2)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
Does E88.01 need an additional code?
When the condition applies, yes. The Use Additional Code note at E88, which applies to E88.01, reads: codes for associated conditions. The additional code is reported after E88.01.
What HCC is E88.01?
E88.01 (Alpha-1-antitrypsin deficiency) maps to CMS-HCC Category 50 (Amyloidosis, Porphyria, and Other Specified Metabolic Disorders), commonly written as HCC 50, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 23 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 40.
Does E88.01 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, E88.01 adds a risk adjustment factor of about 0.648 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.362 to 0.883 depending on the payment segment). HCC 50 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 50 category page.
