E88.01 Alpha-1-antitrypsin deficiency

Billable ICD-10-CM code, FY2026.

Applicable to

• AAT deficiency

Applies from E88 Other and unspecified metabolic disorders

Excludes1 (never code together)

• histiocytosis X (chronic) (C96.6)

Use additional code

• codes for associated conditions

Applies from E88.0 Disorders of plasma-protein metabolism, not elsewhere classified

Excludes1 (never code together)

• monoclonal gammopathy (of undetermined significance) (D47.2)
• polyclonal hypergammaglobulinemia (D89.0)
• Waldenström macroglobulinemia (C88.00)

Excludes2 (not included here)

• disorder of lipoprotein metabolism (E78.-)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 50 — Amyloidosis, Porphyria, and Other Specified Metabolic Disorders
• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 40 — Alpha-1-Antitrypsin Deficiency
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 273.4 Alpha-1-antitrypsin deficiency

Code annotations containing back-references to E88.01

Back-references to E88.0

• Type 1 Excludes: R77 Other abnormalities of plasma proteins ("disorders of plasma-protein metabolism (E88.0-)")

Back-references to E88

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")

Diagnosis Index entries for E88.01

• Deficiency, deficient › alpha-1-antitrypsin
• Deficiency, deficient › serum antitrypsin, familial

Nearby codes

• E88 Other and unspecified metabolic disorders
• E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
• E88.01 Alpha-1-antitrypsin deficiency (this code)
• E88.02 Plasminogen deficiency
• E88.09 Other disorders of plasma-protein metabolism, not elsewhere classified
• E88.1 Lipodystrophy, not elsewhere classified
• E88.10 Lipodystrophy, unspecified
• E88.11 Partial lipodystrophy