2026 ICD-10-CM Changes

Highlights of the code changes in ICD-10-CM taking effect 10/1/2025.

The ICD-10-CM updates for 2026 are here, and with over 480 new codes, 38 revisions, and 28 deletions, it's more important than ever for medical coders and healthcare professionals to understand what’s changed and why it matters. These updates reflect evolving clinical practices, research needs, and the growing importance of data specificity in patient care and reimbursement.

Why These Updates Matter

Every year, changes to the ICD-10-CM code set are designed to improve the accuracy of medical coding, support evolving clinical knowledge, and enable better data collection for research and public health. The 2026 updates especially emphasize:

Greater clinical detail
Genetic and hereditary conditions
Social determinants of health
Emerging diseases and treatments

Let’s break down the most significant changes and what coders should look out for.

Guideline Changes: The Foundation of Accurate Coding

Clarifying “Multiple Sites”

When a condition affects two or more anatomical locations, coders must determine whether to code each site individually or use a “multiple sites” code. If documentation specifies each site, code them separately. Use the multiple sites code only if the provider does not specify.

HIV Coding Revisions

The updated guidance clarifies when to assign:

B20: HIV disease or AIDS, or any HIV-related condition
Z21: Asymptomatic HIV positive
R75: Inconclusive serology

Once a patient has HIV-related symptoms, they are coded as B20 permanently. For pregnant patients, HIV codes are sequenced with pregnancy codes first. The update also introduces Z29.81 for pre-exposure prophylaxis (PrEP) encounters.

Highlight: New Code for Type 2 Diabetes in Remission

A major addition is E11.A, indicating type 2 diabetes in remission. This distinguishes it from active disease or the less precise term “resolved.” Coders should confirm provider documentation specifically states “remission” to use this code. It reflects a more nuanced understanding of disease management and tracking.

Hypertension and Heart Disease

The new guidance strengthens the rules for linking hypertension with heart conditions such as heart failure or myocarditis. If the provider documents a connection, assign I11 codes. If the heart condition is clearly unrelated, code them separately. This change encourages more reliance on provider documentation and avoids assumptions.

Pertinent Code Changes

Infectious Diseases

Expanded codes for HIV-related conditions and infestation by Demodex mites.

Neoplasms

New codes for inflammatory breast cancers, reflecting laterality and alignment with cancer research classifications.

Blood and Endocrine Disorders

Abnormal autoantibody findings (rheumatoid factor, anti-CCCP) without clinical RA
Hyperoxaluria, now with detailed primary, secondary, and dietary-related forms
Familial hypercholesterolemia, now split into homozygous and heterozygous
Lipodystrophy, with several subtypes including HIV-associated

Neurology and Genetic Conditions

Multiple Sclerosis

Greater detail for relapsing and progressive MS with active or inactive status. This helps coders more accurately reflect prognosis and treatment.

Primary Apraxia of Speech

New code supports data collection in speech therapy and neurology for this rare disorder.

Limb-Girdle Muscular Dystrophy

A new genetic-specific code highlights advances in molecular diagnosis.

Eyes, Ears, and Congenital Conditions

Thyroid eye disease and secondary angle-closure glaucoma now have laterality-specific codes
Usher syndrome gets its own type-specific codes
New codes for Kabuki, CTNNB1, Howell Foundation, and other congenital syndromes
Genetic neurodevelopmental disorders now reflect specific gene variants like FOXG1, DLG4, and SLC6A1

These updates underscore how genetic insights are transforming diagnosis and documentation.

Skin and Ulcers

Chronic non-pressure ulcers of the abdomen, face, arms, and groin now have severity-specific codes. This supports better clinical management and reimbursement. Additionally, there are new codes for abscesses, contusions, and puncture wounds of the flank, groin, and abdominal wall.

Pain and Lab Findings

Expanded codes for abdominal and flank pain, now with laterality
New code for cannabis hyperemesis syndrome (R11.16)
Addition of costovertebral angle tenderness
Abnormal immunological lab findings without a clinical diagnosis are now codable

External Causes and Social Determinants of Health

New codes for injuries such as fishing hook piercings and sharp objects entering through orifices
Additions for Gulf War illness and war-related exposures
More precise capture of prophylactic surgeries (even in absence of family history)
New Z codes for financial insecurity and utility access – an acknowledgment of how social factors affect health outcomes

Final Thoughts

The 2026 ICD-10-CM updates continue the trend toward greater specificity in medical coding. From genetic disorders to social determinants of health, coders must now connect clinical documentation to increasingly detailed codes. This demands close attention to detail, proactive querying, and ongoing education.

To stay compliant and effective, coders should:

Review guideline changes each year
Train providers on the importance of clear documentation
Validate coding decisions with current source documents
Be prepared to adjust workflows as specificity increases

As ICD-10-CM continues to evolve, coders are more important than ever in ensuring the integrity of clinical data.