Understanding Immunodeficiency
A Guide to Immunodeficiency
What is Immunodeficiency?
Defining Immunodeficiency
Immunodeficiency or an immunocompromised state refers to a weakened immune system that results in more frequent infections that are more severe and last longer than usual.
There are two types of immunodeficiency disorders: Primary and Secondary.
- Primary disorders are usually present at birth and are often hereditary. The International Union of Immunological Societies recognizes nine classes of primary immunodeficiencies, totaling approximately 430 conditions.
- Secondary disorders are acquired conditions related to chronic conditions, medical therapy, or other external factors.
Diagnostic Criteria
- Review of past medical history of medical conditions and medicine therapy to evaluate if at risk.
- Complete blood count (CBC)- Cytopenia/Cell abnormalities
- Immunoglobulin levels (IgG, IgM, IgA and IgE)
- Bone marrow or lymph node biopsy to determine specific immunodeficiency disorder
Coding Considerations
- D84.81, Immunodeficiency due to condition classified elsewhere, was created for immunocompromised state due to specific medical conditions such as cancers and genetic disorders. HIV is an excluded condition as it is considered inherent to the disease per Coding Clinic.
- D84.821, Immunodeficiency due to drugs is a code for immunodeficiency due to medications that interfere with the immune system.
- D84.822, Immunodeficiency due to external causes such as radiation therapy or bone marrow transplant.
- Consider Coding Clinics:
- AHA Coding Clinic ICD-10-CM/PCS, First Quarter 2021, p.52 Correction notice: Immunocompromised State due to Human Immunodeficiency Virus Disease
- AHA Coding Clinic ICD-10-CM/PCS, Fourth Quarter, 2020, p. 10 ICD-10 CM New/Revised Codes: Immunodeficiency Status
CDI Practice Considerations
Most Immunodeficiency codes provide a CC. With the new CMS V28 Risk Adjustment models, Immunodeficiency codes will no longer map to an HCC for Medicare Advantage patients. However, other Risk Adjustment models (Affordable Care Act and Medicaid) will continue to map to an HCC.
Be aware of Secondary Disorders of Immunodeficiency that are treated with immunosuppressant drugs:
- Autoimmune Diseases
- Lupus
- Rheumatoid Arthritis
- Crohn’s disease
- Multiple Sclerosis
- Psoriasis
- Other Immunocompromised Conditions
- S/P Organ Transplant
- Cancer patients receiving chemotherapy or radiation therapy
- Common Immunosuppressants
- Corticosteroids (Prednisone, Budesonide, prednisolone)
- Janus kinase inhibitors (Xeljanz)
- Calcineurin inhibitors (cyclosporine, tacrolimus)
- mTOR inhibitors (Rapamune, Afinitor, Zortress)
- IMDH inhibitors (Imuran, CellCept, Myfortic)
- Biologics (Orencia, Humira, Embrel, Remicade, Rituxan, Cosentyx)
Other Immunocompromised Conditions
- S/P Organ Transplant
- Cancer patients receiving chemotherapy or radiation therapy
- Common Immunosuppressants
- Corticosteroids (Prednisone, Budesonide, prednisolone)
- Janus kinase inhibitors (Xeljanz)
- Calcineurin inhibitors (cyclosporine, tacrolimus)
- mTOR inhibitors (Rapamune, Afinitor, Zortress)
- IMDH inhibitors (Imuran, CellCept, Myfortic)
- Biologics (Orencia, Humira, Embrel, Remicade, Rituxan, Cosentyx)
Ensure that the documentation accurately reflects the underlying etiology of the immunodeficient state, enabling the assignment that truly represents the patient’s clinical condition.
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The information and opinions presented here are based on the experience, training, and interpretation of the author. Although the information has been researched and reviewed for accuracy, e4health does not accept any responsibility or liability regarding errors, omission, misuse, or misinterpretation. This information is intended as a guide; it should not be considered a legal/consulting opinion or advice.