D80-D89
Medium Complexity

Certain disorders involving the immune mechanism

Primary Specialty: Allergy and Immunology
Last Updated: 2025-09-10

ICD-10 Codes (89)

89 billable
0 category headers
D81
Billable
Combined immunodeficiencies
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D81.0
Billable
Severe combined immunodeficiency [SCID] with reticular dysgenesis
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D81.1
Billable
Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
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D81.2
Billable
Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
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D81.3
Billable
Adenosine deaminase [ADA] deficiency
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D81.30
Billable
Adenosine deaminase deficiency, unspecified
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D81.31
Billable
Severe combined immunodeficiency due to adenosine deaminase deficiency
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D81.32
Billable
Adenosine deaminase 2 deficiency
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D81.39
Billable
Other adenosine deaminase deficiency
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D81.4
Billable
Nezelof's syndrome
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D81.5
Billable
Purine nucleoside phosphorylase [PNP] deficiency
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D81.6
Billable
Major histocompatibility complex class I deficiency
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D81.7
Billable
Major histocompatibility complex class II deficiency
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D81.8
Billable
Other combined immunodeficiencies
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D81.81
Billable
Biotin-dependent carboxylase deficiency
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D81.810
Billable
Biotinidase deficiency
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D81.818
Billable
Other biotin-dependent carboxylase deficiency
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D81.819
Billable
Biotin-dependent carboxylase deficiency, unspecified
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D81.82
Billable
Activated Phosphoinositide 3-kinase Delta Syndrome [APDS]
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D81.89
Billable
Other combined immunodeficiencies
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D81.9
Billable
Combined immunodeficiency, unspecified
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D82
Billable
Immunodeficiency associated with other major defects
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D82.0
Billable
Wiskott-Aldrich syndrome
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D82.1
Billable
Di George's syndrome
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D82.2
Billable
Immunodeficiency with short-limbed stature
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D82.3
Billable
Immunodeficiency following hereditary defective response to Epstein-Barr virus
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D82.4
Billable
Hyperimmunoglobulin E [IgE] syndrome
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D82.8
Billable
Immunodeficiency associated with other specified major defects
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D82.9
Billable
Immunodeficiency associated with major defect, unspecified
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D83
Billable
Common variable immunodeficiency
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D83.0
Billable
Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
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D83.1
Billable
Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
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D83.2
Billable
Common variable immunodeficiency with autoantibodies to B- or T-cells
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D83.8
Billable
Other common variable immunodeficiencies
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D83.9
Billable
Common variable immunodeficiency, unspecified
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D84
Billable
Other immunodeficiencies
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D84.0
Billable
Lymphocyte function antigen-1 [LFA-1] defect
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D84.1
Billable
Defects in the complement system
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D84.8
Billable
Other specified immunodeficiencies
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D84.81
Billable
Immunodeficiency due to conditions classified elsewhere
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D84.82
Billable
Immunodeficiency due to drugs and external causes
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D84.821
Billable
Immunodeficiency due to drugs
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D84.822
Billable
Immunodeficiency due to external causes
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D84.89
Billable
Other immunodeficiencies
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D84.9
Billable
Immunodeficiency, unspecified
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D86
Billable
Sarcoidosis
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D86.0
Billable
Sarcoidosis of lung
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D86.1
Billable
Sarcoidosis of lymph nodes
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D86.2
Billable
Sarcoidosis of lung with sarcoidosis of lymph nodes
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D86.3
Billable
Sarcoidosis of skin
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D86.8
Billable
Sarcoidosis of other sites
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D86.81
Billable
Sarcoid meningitis
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D86.82
Billable
Multiple cranial nerve palsies in sarcoidosis
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D86.83
Billable
Sarcoid iridocyclitis
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D86.84
Billable
Sarcoid pyelonephritis
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D86.85
Billable
Sarcoid myocarditis
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D86.86
Billable
Sarcoid arthropathy
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D86.87
Billable
Sarcoid myositis
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D86.89
Billable
Sarcoidosis of other sites
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D86.9
Billable
Sarcoidosis, unspecified
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D89
Billable
Other disorders involving the immune mechanism, not elsewhere classified
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D89.0
Billable
Polyclonal hypergammaglobulinemia
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D89.1
Billable
Cryoglobulinemia
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D89.2
Billable
Hypergammaglobulinemia, unspecified
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D89.3
Billable
Immune reconstitution syndrome
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D89.4
Billable
Mast cell activation syndrome and related disorders
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D89.40
Billable
Mast cell activation, unspecified
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D89.41
Billable
Monoclonal mast cell activation syndrome
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D89.42
Billable
Idiopathic mast cell activation syndrome
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D89.43
Billable
Secondary mast cell activation
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D89.44
Billable
Hereditary alpha tryptasemia
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D89.49
Billable
Other mast cell activation disorder
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D89.8
Billable
Other specified disorders involving the immune mechanism, not elsewhere classified
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D89.81
Billable
Graft-versus-host disease
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D89.810
Billable
Acute graft-versus-host disease
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D89.811
Billable
Chronic graft-versus-host disease
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D89.812
Billable
Acute on chronic graft-versus-host disease
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D89.813
Billable
Graft-versus-host disease, unspecified
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D89.82
Billable
Autoimmune lymphoproliferative syndrome [ALPS]
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D89.83
Billable
Cytokine release syndrome
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D89.831
Billable
Cytokine release syndrome, grade 1
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D89.832
Billable
Cytokine release syndrome, grade 2
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D89.833
Billable
Cytokine release syndrome, grade 3
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D89.834
Billable
Cytokine release syndrome, grade 4
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D89.835
Billable
Cytokine release syndrome, grade 5
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D89.839
Billable
Cytokine release syndrome, grade unspecified
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D89.84
Billable
IgG4-related disease
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D89.89
Billable
Other specified disorders involving the immune mechanism, not elsewhere classified
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D89.9
Billable
Disorder involving the immune mechanism, unspecified
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Updates & Changes

FY 2026 Updates

Current Year

Deleted Codes

No codes deleted in this range for FY 2026

No significant changes for FY 2026

This range maintains stability with current coding practices

Historical Changes

  • •FY 2025: Routine maintenance updates with minor terminology clarifications
  • •FY 2024: Enhanced specificity requirements for certain code ranges
  • •FY 2023: Updated documentation guidelines for improved clarity

Upcoming Changes

  • •Proposed updates pending review by Coordination and Maintenance Committee
  • •Under consideration: Enhanced digital health integration codes

Implementation Guidance

  • •Review all FY 2026 updates for D80-D89 codes before implementation
  • •Always verify the most current codes in the ICD-10-CM manual
  • •Ensure clinical documentation supports the selected diagnosis codes
  • +3 more guidance items...

Range Overview

high priority

The D80-D89 range in ICD-10 pertains to disorders involving the immune mechanism. This includes both primary and secondary immunodeficiencies, autoimmune diseases, and other diseases mediated by immune mechanisms. These codes are crucial for accurately documenting patient conditions and ensuring appropriate treatment and reimbursement.

Key Usage Points:

  • •Always specify the type of immunodeficiency (primary or secondary) when coding.
  • •For autoimmune diseases, indicate the organ or system involved.
  • •Use additional codes to identify any associated conditions or complications.
  • •Remember that some conditions have both an underlying etiology and multiple body system manifestations.
  • •Always review the latest updates and changes in the ICD-10 guidelines.

Coding Guidelines

When to Use:

  • ✓When a patient has a diagnosed immunodeficiency disorder.
  • ✓When a patient presents with symptoms suggestive of an immune disorder.
  • ✓When a patient has an autoimmune disease affecting a specific organ or system.
  • ✓When a patient has a disease mediated by immune mechanisms, such as graft-versus-host disease.

When NOT to Use:

  • ✗When a patient has an immune response to an infection, but no underlying immune disorder.
  • ✗When a patient has a condition that is not primarily due to an immune mechanism.
  • ✗When a patient has a temporary immune response due to medication or treatment.
  • ✗When a patient has a condition that falls under another ICD-10 code range.

Code Exclusions

Always verify exclusions using the latest ICD-10 guidelines and updates.

Documentation Requirements

Accurate documentation is crucial for correct coding. It should include a clear diagnosis, details about the type of immune disorder, any associated conditions or complications, and the impact on the patient's health status.

Clinical Information:

  • •Specific diagnosis
  • •Type of immune disorder (primary or secondary)
  • •Organ or system affected (for autoimmune diseases)
  • •Associated conditions or complications
  • •Impact on patient's health status

Supporting Evidence:

  • •Laboratory test results
  • •Imaging studies
  • •Clinical notes
  • •Specialist reports
Good Documentation Example:

Patient diagnosed with primary immunodeficiency disorder, specifically common variable immunodeficiency. Regular infections noted. Impact on health status significant.

Poor Documentation Example:

Patient has immune disorder.

Common Documentation Errors:

  • âš Not specifying the type of immune disorder
  • âš Not indicating the organ or system affected
  • âš Not documenting associated conditions or complications
  • âš Not detailing the impact on the patient's health status

Range Statistics

7
Total Codes
89
Billable
Complexity:
Medium
Primary Use:Clinical Documentation
Chapter:3

Coding Complexity

Medium
Complexity Rating

The complexity of coding within this range is considered medium. This is due to the need for detailed documentation, the specificity required in coding, and the need to keep up with updates and changes in the ICD-10 guidelines.

Key Factors:
  • â–¸Need to specify the type of immune disorder
  • â–¸Need to indicate the organ or system affected
  • â–¸Need to document associated conditions or complications
  • â–¸Need to keep up with updates and changes in the ICD-10 guidelines

Specialty Focus

These codes are most commonly used in specialties dealing with immunology, such as Allergy and Immunology, Rheumatology, and Infectious Disease.

Primary Specialties:
Allergy and Immunology
40%
Rheumatology
30%
Infectious Disease
30%
Clinical Scenarios:
  • • A patient with recurrent infections diagnosed with common variable immunodeficiency.
  • • A patient with systemic lupus erythematosus presenting with kidney involvement.
  • • A patient with graft-versus-host disease following a bone marrow transplant.
  • • A patient with secondary immunodeficiency due to long-term corticosteroid use.

Resources & References

Several resources are available to assist with coding within this range. These include the official ICD-10 guidelines, clinical reference sources, and educational materials.

Official Guidelines:

  • Official ICD-10-CM Guidelines for Coding and Reporting
  • American Health Information Management Association (AHIMA) resources
  • Centers for Disease Control and Prevention (CDC) resources
  • World Health Organization (WHO) resources

Clinical References:

  • American Academy of Allergy, Asthma & Immunology (AAAAI) resources
  • American College of Rheumatology (ACR) resources

Educational Materials:

  • AHIMA ICD-10-CM Coding Workbook
  • ICD-10-CM Expert for Physicians

Frequently Asked Questions

Can I use these codes for patients with temporary immune responses?

No, these codes are for disorders involving the immune mechanism, not temporary immune responses.