Medicare Covered Diagnosis Codes for IVIG: NCD, LCD, and Compendia

Medicare’s list of covered diagnosis codes for IVIG has two tiers. Nationally, every Medicare Administrative Contractor (MAC) must cover 24 ICD-10-CM codes for primary immunodeficiency diseases in the D80–D83 range plus G11.3, and a separate national policy (NCD 250.3) covers autoimmune mucocutaneous blistering diseases as short-term therapy. Every other indication, including chronic inflammatory demyelinating polyneuropathy (CIDP), myasthenia gravis, Guillain-Barré syndrome, multifocal motor neuropathy, and stiff-person syndrome, is covered only when your regional MAC lists the specific ICD-10 code in its Local Coverage Determination (LCD). That means the same diagnosis can be payable in one state and denied in another.

Nationally Covered Codes for Primary Immunodeficiency

Primary immunodeficiency diseases (PIDD) get the broadest IVIG coverage under Medicare. CMS has established national policy for these inherited immune disorders, so every MAC must honor the codes below. These 24 ICD-10-CM codes are listed as supporting medical necessity for IVIG in CMS billing and coding guidance:1Centers for Medicare & Medicaid Services. Billing and Coding: Coverage of Intravenous Immune Globulin

  • D80.0 — Hereditary hypogammaglobulinemia
  • D80.2 — Selective deficiency of immunoglobulin A (IgA)
  • D80.3 — Selective deficiency of immunoglobulin G (IgG) subclasses
  • D80.4 — Selective deficiency of immunoglobulin M (IgM)
  • D80.5 — Immunodeficiency with increased immunoglobulin M (IgM)
  • D80.6 — Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia
  • D80.7 — Transient hypogammaglobulinemia of infancy
  • D81.0 — Severe combined immunodeficiency (SCID) with reticular dysgenesis
  • D81.1 — SCID with low T- and B-cell numbers
  • D81.2 — SCID with low or normal B-cell numbers
  • D81.5 — Purine nucleoside phosphorylase (PNP) deficiency
  • D81.6 — Major histocompatibility complex class I deficiency
  • D81.7 — Major histocompatibility complex class II deficiency
  • D81.89 — Other combined immunodeficiencies
  • D81.9 — Combined immunodeficiency, unspecified
  • D82.0 — Wiskott-Aldrich syndrome
  • D82.1 — DiGeorge syndrome
  • D82.4 — Hyperimmunoglobulin E (IgE) syndrome
  • D83.0 — Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
  • D83.1 — Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
  • D83.2 — Common variable immunodeficiency with autoantibodies to B- or T-cells
  • D83.8 — Other common variable immunodeficiencies
  • D83.9 — Common variable immunodeficiency, unspecified
  • G11.3 — Cerebellar ataxia with defective DNA repair

These conditions share a common thread. The immune system cannot produce enough functional antibodies on its own, and IVIG replaces those missing antibodies. Because they are genetic and lifelong, reauthorization for continued therapy tends to run on longer approval windows than other IVIG indications.

NCD 250.3: Autoimmune Mucocutaneous Blistering Diseases

Outside primary immunodeficiency, the other national coverage policy for IVIG is NCD 250.3, which applies to autoimmune mucocutaneous blistering diseases such as pemphigus vulgaris and bullous pemphigoid.2Centers for Medicare & Medicaid Services. Intravenous Immune Globulin (IVIg) – NCD 250.3 One limitation matters up front: IVIG for these blistering diseases is covered only as short-term therapy to bring the condition under control, not as ongoing maintenance. What counts as “short-term” is left to the discretion of individual MACs, so the allowed number of infusion cycles may differ depending on where you receive treatment.

The specific ICD-10-CM codes falling under NCD 250.3 are published in the associated CMS billing and coding articles. Providers should check the current version of the article linked to NCD 250.3 in the Medicare Coverage Database, because the code list is periodically updated as ICD-10 revisions occur.

Locally Covered Codes for Neurological and Autoimmune Conditions

Most conditions that bring patients to IVIG fall outside national coverage. CIDP, myasthenia gravis, Guillain-Barré syndrome, multifocal motor neuropathy, and stiff-person syndrome are covered through Local Coverage Determinations issued by individual MACs.3U.S. Department of Health and Human Services Office of Inspector General. Local Coverage Determinations Create Inconsistency in Medicare Coverage The exact list of approved ICD-10-CM codes can differ depending on where you live or where your provider bills from.

One code that appears consistently across MAC jurisdictions is G61.81 for chronic inflammatory demyelinating polyneuropathy.4Centers for Medicare & Medicaid Services. Billing and Coding: Immune Globulin Intravenous (IVIg) Even where covered, G61.81 carries a notable exclusion: it is not payable when the polyneuropathy is associated with diabetes, dysproteinemias, renal failure, or malnutrition. If the patient’s CIDP has one of those underlying causes, the claim will be denied even though the code itself is on the covered list.

Each MAC publishes its LCD alongside a companion billing and coding article that spells out every covered and non-covered code. LCD L35093 for Immune Globulin, for example, lists ICD-10-CM codes organized into groups: those that support medical necessity and those that explicitly do not.5Centers for Medicare & Medicaid Services. LCD – Immune Globulin (L35093) The companion billing article for the same LCD adds detail on procedure codes, modifiers, and documentation expectations.6Centers for Medicare & Medicaid Services. Billing and Coding: Immune Globulin (A56786)

How to Find Your MAC’s Covered Codes

CMS divides the country into regional jurisdictions, each managed by a specific MAC. There are 12 Part A/B MAC jurisdictions handling physician and hospital claims, plus four DME MAC jurisdictions handling durable medical equipment claims. The DME MAC list is relevant if you receive IVIG at home through a DME supplier.7Centers for Medicare & Medicaid Services. Who Are the MACs CMS publishes a “MACs by State” document on the same page that maps each state and territory to its assigned contractor.

Once you know your MAC, search the Medicare Coverage Database at cms.gov for that contractor’s LCD on immune globulin. The LCD itself states whether IVIG is covered for a given condition, and the linked billing article lists every ICD-10-CM code the MAC will accept. Check this before every new treatment course. MACs update their LCDs periodically, and a code that was covered last year may have been revised or removed.

Off-Label Uses Covered Through Drug Compendia

IVIG is sometimes prescribed for conditions beyond its FDA-approved labeling. Medicare can still cover these off-label uses if the indication appears in one of several recognized drug compendia, which are reference databases that evaluate whether sufficient clinical evidence supports a particular use.8Centers for Medicare & Medicaid Services. Billing and Coding: Off-Label Use of Intravenous Immune Globulin (IVIG) The compendia Medicare recognizes include AHFS Drug Information, Micromedex DrugDex, Lexi-Drugs, Clinical Pharmacology, and the National Comprehensive Cancer Network guidelines.

If a diagnosis is listed as a supported indication in one of these compendia but is not on the FDA label, the MAC may cover the claim under a separate off-label use policy. The associated CMS billing and coding article lists the compendia-approved ICD-10-CM codes in a distinct group. This is often the path for less common autoimmune and hematologic conditions where IVIG has evidence of benefit but no formal FDA indication.

Why the Correct Code Alone Won’t Get the Claim Paid

A covered ICD-10 code is necessary but not sufficient. The medical record has to demonstrate why IVIG is appropriate for the specific patient. CMS billing guidance calls for documentation that includes a recent history and physical supporting the underlying diagnosis, office and progress notes tracking the patient’s response to treatment, test results and interpretations that substantiate the diagnosis code (immunoglobulin levels, antibody titers, nerve conduction studies), and an accurate weight in kilograms recorded before each infusion, since dosing is calculated per kilogram of body weight.4Centers for Medicare & Medicaid Services. Billing and Coding: Immune Globulin Intravenous (IVIg)

Individual MACs often impose additional requirements beyond this baseline, such as specific timeframes for how recently the history and physical must have been completed, or how far in advance physician orders must be dated. Your provider should consult the LCD and billing article published by the applicable MAC for the full list. Claims submitted without adequate supporting records risk denial for insufficient medical necessity even when the diagnosis code is on the covered list.

What to Do If a Diagnosis Code Denial Arrives

IVIG claims are denied frequently, and many of those denials trace back to coding or documentation. If your claim is denied, the first appeal is a redetermination filed with the MAC that issued the denial. You have 120 days from the date you receive the initial denial notice, which is presumed to be five days after the notice date unless you can show otherwise.9Centers for Medicare & Medicaid Services. First Level of Appeal: Redetermination by a Medicare Contractor

The strongest appeals pair the correct ICD-10 code with robust medical records. If the denial was for insufficient documentation, the most effective move at the first level is to submit the missing records: lab results, progress notes, or a detailed letter of medical necessity from the treating physician. For denials based on the diagnosis code itself not being covered under the MAC’s LCD, the appeal is harder. You may need to show that the patient’s condition actually falls under a covered code that was miscoded on the original claim, or argue that off-label compendia support coverage for the submitted code.