ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group E78 includes disorders of lipoprotein metabolism and other lipidemias such as familial and nonfamilial hypercholesterolemia, hypertriglyceridemia, mixed hyperlipidemia, and other specified or unspecified disorders affecting cholesterol and triglyceride levels. These ICD-10-CM diagnosis codes are used to document clinical scenarios including primary and secondary lipid disorders, associated cardiovascular risk factors, and metabolic contributors affecting disease severity and management. Accurate coding within the ICD-10-CM E78 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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ICD-10-CM E78: Disorders Of Lipoprotein Metabolism And Other Lipidemias Overview

This group includes disorders of lipid and lipoprotein metabolism such as hypercholesterolemia, hypertriglyceridemia, mixed hyperlipidemia, and other specified and unspecified lipidemias that affect serum lipid levels and cardiovascular risk. These conditions target metabolic processes and the cardiovascular system by altering cholesterol and triglyceride profiles. Accurate coding matters for reimbursement because it links documented clinical severity and comorbidities to appropriate billing and claim processing.

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ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

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ICD-10-CM E78: Disorders Of Lipoprotein Metabolism And Other Lipidemias Overview

When to Use

Use a code from this group when a patient is diagnosed with a primary or secondary lipid disorder documented in the medical record, including familial lipid disorders and drug- or disease-related dyslipidemias. Select the specific code that reflects the type of lipid abnormality, documented severity, and any associated conditions or complications.

ICD-10-CM Codes

18 rows

ICD-10-CM Code	Description
E78.00	Pure hypercholesterolemia, unspecified
E78.010	Homozygous familial hypercholesterolemia [HoFH]
E78.011	Heterozygous familial hypercholesterolemia [HeFH]
E78.019	Familial hypercholesterolemia, unspecified
E78.1	Pure hyperglyceridemia
E78.2	Mixed hyperlipidemia
E78.3	Hyperchylomicronemia
E78.41	Elevated Lipoprotein(a)
E78.49	Other hyperlipidemia
E78.5	Hyperlipidemia, unspecified
E78.6	Lipoprotein deficiency
E78.70	Disorder of bile acid and cholesterol metabolism, unspecified
E78.71	Barth syndrome
E78.72	Smith-Lemli-Opitz syndrome
E78.79	Other disorders of bile acid and cholesterol metabolism
E78.81	Lipoid dermatoarthritis
E78.89	Other lipoprotein metabolism disorders
E78.9	Disorder of lipoprotein metabolism, unspecified

DRG Codes

4 rows

DRG Code	Description
564	Other Musculoskeletal System and Connective Tissue Diagnoses with MCC
565	Other Musculoskeletal System and Connective Tissue Diagnoses with CC
566	Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC
642	Inborn and Other Disorders of Metabolism

Typical Clinical Scenarios

A middle-aged patient with a long history of poorly controlled type 2 diabetes and obesity presents for routine follow-up complaining of persistent xanthomas and fasting lipid panel showing markedly elevated LDL-C and mildly elevated triglycerides. The visit is for chronic management of dyslipidemia to reduce cardiovascular risk, and the clinician documents primary hypercholesterolemia as the principal lipid disorder. Typical codes: E78.00, E78.2
A young adult presents to the emergency department with acute pancreatitis confirmed by elevated lipase and abdominal imaging; recent labs reveal severely elevated triglycerides in the thousands mg/dL with a history of alcohol use and familial hypertriglyceridemia. The acute event is attributed to hypertriglyceridemia, so a disorder of elevated triglycerides is coded as the underlying metabolic cause contributing to the pancreatitis. Typical codes: E78.1, E78.2
An older patient with coronary artery disease and prior myocardial infarction comes to the cardiology clinic for medication reconciliation; lipid panel shows controlled LDL but ongoing low HDL and mixed dyslipidemia despite therapy, and the clinician documents other specified lipoprotein abnormalities as comorbidities impacting cardiovascular risk management. Typical codes: E78.49, E78.6

ICD-10-CM Code For Disorders Of Aromatic Amino-Acid Metabolism: Group Overview

E71-DISORDERS-OF-BRANCHED-CHAIN-AMINO-ACID-METABOLISM

ICD-10-CM Code For Disorders Of Branched Chain Amino Acid Metabolism And Fatty Acid Metabolism: Group Overview

E72-OTHER-DISORDERS-OF-AMINO-ACID-METABOLISM

ICD-10-CM Code For Other Disorders Of Amino-Acid Metabolism: Group Overview

E73-LACTOSE-INTOLERANCE

ICD-10-CM Code For Lactose Intolerance: Group Overview

E74-OTHER-DISORDERS-OF-CARBOHYDRATE-METABOLISM

ICD-10-CM Code For Other Disorders Of Carbohydrate Metabolism: Group Overview

E75-DISORDERS-OF-SPHINGOLIPID-METABOLISM

ICD-10-CM Code For Disorders Of Sphingolipid Metabolism And Other Lipid Storage Disorders: Group Overview

E76-DISORDERS-OF-GLYCOSAMINOGLYCAN-METABOLISM

ICD-10-CM Code For Disorders Of Glycosaminoglycan Metabolism: Group Overview

E77-DISORDERS-OF-GLYCOPROTEIN-METABOLISM

ICD-10-CM Code For Disorders Of Glycoprotein Metabolism: Group Overview

E79-DISORDERS-OF-PURINE-AND-PYRIMIDINE-METABOLISM

ICD-10-CM Code For Disorders Of Purine And Pyrimidine Metabolism: Group Overview

E80-DISORDERS-OF-PORPHYRIN-AND-BILIRUBIN-METABOLISM

ICD-10-CM Code For Disorders Of Porphyrin And Bilirubin Metabolism: Group Overview

Showing 1–10 of 16

Frequently Asked Questions

References

1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.

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When to Use

ICD-10-CM Codes

18 rows

ICD-10-CM Code	Description
E78.00	Pure hypercholesterolemia, unspecified
E78.010	Homozygous familial hypercholesterolemia [HoFH]
E78.011	Heterozygous familial hypercholesterolemia [HeFH]
E78.019	Familial hypercholesterolemia, unspecified
E78.1	Pure hyperglyceridemia
E78.2	Mixed hyperlipidemia
E78.3	Hyperchylomicronemia
E78.41	Elevated Lipoprotein(a)
E78.49	Other hyperlipidemia
E78.5	Hyperlipidemia, unspecified
E78.6	Lipoprotein deficiency
E78.70	Disorder of bile acid and cholesterol metabolism, unspecified
E78.71	Barth syndrome
E78.72	Smith-Lemli-Opitz syndrome
E78.79	Other disorders of bile acid and cholesterol metabolism
E78.81	Lipoid dermatoarthritis
E78.89	Other lipoprotein metabolism disorders
E78.9	Disorder of lipoprotein metabolism, unspecified

DRG Codes

4 rows

DRG Code	Description
564	Other Musculoskeletal System and Connective Tissue Diagnoses with MCC
565	Other Musculoskeletal System and Connective Tissue Diagnoses with CC
566	Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC
642	Inborn and Other Disorders of Metabolism

Typical Clinical Scenarios

A middle-aged patient with a long history of poorly controlled type 2 diabetes and obesity presents for routine follow-up complaining of persistent xanthomas and fasting lipid panel showing markedly elevated LDL-C and mildly elevated triglycerides. The visit is for chronic management of dyslipidemia to reduce cardiovascular risk, and the clinician documents primary hypercholesterolemia as the principal lipid disorder. Typical codes: E78.00, E78.2
A young adult presents to the emergency department with acute pancreatitis confirmed by elevated lipase and abdominal imaging; recent labs reveal severely elevated triglycerides in the thousands mg/dL with a history of alcohol use and familial hypertriglyceridemia. The acute event is attributed to hypertriglyceridemia, so a disorder of elevated triglycerides is coded as the underlying metabolic cause contributing to the pancreatitis. Typical codes: E78.1, E78.2
An older patient with coronary artery disease and prior myocardial infarction comes to the cardiology clinic for medication reconciliation; lipid panel shows controlled LDL but ongoing low HDL and mixed dyslipidemia despite therapy, and the clinician documents other specified lipoprotein abnormalities as comorbidities impacting cardiovascular risk management. Typical codes: E78.49, E78.6

ICD-10-CM Code For Disorders Of Aromatic Amino-Acid Metabolism: Group Overview

E71-DISORDERS-OF-BRANCHED-CHAIN-AMINO-ACID-METABOLISM

ICD-10-CM Code For Disorders Of Branched Chain Amino Acid Metabolism And Fatty Acid Metabolism: Group Overview

E72-OTHER-DISORDERS-OF-AMINO-ACID-METABOLISM

ICD-10-CM Code For Other Disorders Of Amino-Acid Metabolism: Group Overview

E73-LACTOSE-INTOLERANCE

ICD-10-CM Code For Lactose Intolerance: Group Overview

E74-OTHER-DISORDERS-OF-CARBOHYDRATE-METABOLISM

ICD-10-CM Code For Other Disorders Of Carbohydrate Metabolism: Group Overview

E75-DISORDERS-OF-SPHINGOLIPID-METABOLISM

ICD-10-CM Code For Disorders Of Sphingolipid Metabolism And Other Lipid Storage Disorders: Group Overview

E76-DISORDERS-OF-GLYCOSAMINOGLYCAN-METABOLISM

ICD-10-CM Code For Disorders Of Glycosaminoglycan Metabolism: Group Overview

E77-DISORDERS-OF-GLYCOPROTEIN-METABOLISM

ICD-10-CM Code For Disorders Of Glycoprotein Metabolism: Group Overview

E79-DISORDERS-OF-PURINE-AND-PYRIMIDINE-METABOLISM

ICD-10-CM Code For Disorders Of Purine And Pyrimidine Metabolism: Group Overview

E80-DISORDERS-OF-PORPHYRIN-AND-BILIRUBIN-METABOLISM

ICD-10-CM Code For Disorders Of Porphyrin And Bilirubin Metabolism: Group Overview

Showing 1–10 of 16

Frequently Asked Questions

References

1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.

Published by

Trek Health powers OpenPayer with a leading commercial payer intelligence platform, seamlessly converting fragmented payer data into actionable insights for provider organizations.

OpenPayer

ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

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ICD-10-CM E78: Disorders Of Lipoprotein Metabolism And Other Lipidemias Overview

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ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

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ICD-10-CM E78: Disorders Of Lipoprotein Metabolism And Other Lipidemias Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

References

ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

Sign up for ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview policy alerts

ICD-10-CM E78: Disorders Of Lipoprotein Metabolism And Other Lipidemias Overview

ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

Sign up for ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview policy alerts

ICD-10-CM E78: Disorders Of Lipoprotein Metabolism And Other Lipidemias Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which conditions are included in ICD-10-CM group `E78`?

How are individual diagnosis codes within the `E78` group formatted in documentation?

Why does accurate coding within `E78` matter for claims?

Which DRGs are related to disorders in the `E78` group?

What clinical scenarios are documented using `E78` diagnoses?

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which conditions are included in ICD-10-CM group `E78`?

How are individual diagnosis codes within the `E78` group formatted in documentation?

Why does accurate coding within `E78` matter for claims?

Which DRGs are related to disorders in the `E78` group?

What clinical scenarios are documented using `E78` diagnoses?

References