Notification



Notification Issue Date:



Medicare Advantage Policy

Title:Immune Globulin: Intravenous (IVIG), Subcutaneous (SCIG)
Policy #:MA08.009h

This policy is applicable to the Company’s Medicare Advantage products only. Policies that are applicable to the Company’s commercial products are accessible via a separate commercial policy database.


The Company makes decisions on coverage based on the Centers for Medicare and Medicaid Services (CMS) regulations and guidance, benefit plan documents and contracts, and the member’s medical history and condition. If CMS does not have a position addressing a service, the Company makes decisions based on Company Policy Bulletins. Benefits may vary based on contract, and individual member benefits must be verified. The Company determines medical necessity only if the benefit exists and no contract exclusions are applicable. Although the Medicare Advantage Policy Bulletin is consistent with Medicare’s regulations and guidance, the Company’s payment methodology may differ from Medicare.

When services can be administered in various settings, the Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.


This Policy Bulletin document describes the status of CMS coverage, medical terminology, and/or benefit plan documents and contracts at the time the document was developed. This Policy Bulletin will be reviewed regularly and be updated as Medicare changes their regulations and guidance, scientific and medical literature becomes available, and/or the benefit plan documents and/or contracts are changed.



Policy

Coverage is subject to the terms, conditions, and limitations of the member's Evidence of Coverage.

When services can be administered in various settings, the Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.

MEDICALLY NECESSARY

SUBCUTANEOUS IMMUNE GLOBULIN (SCIG)
Use of subcutaneous immune globulin (SCIG) (Cutaquig, Cuvitru, Hizentra®, HyQvia, Xembify) or subcutaneous administration of certain intravenous immune globulin (IVIG) (e.g., Gammagard Liquid®, Gammaked®, Gamunex-C®) therapy is considered medically necessary and, therefore, covered for the following indications when the dosing and frequency requirements listed in Attachment B and the following criteria are met:

Neurological and Musculoskeletal Disorders

Chronic Inflammatory Demyelinating Polyneuritis (CIDP)

SCIG is considered medically necessary and, therefore, covered for CIPD when the individual has a documented diagnosis of CIDP.

Primary Humoral Immunodeficiencies

SCIG is considered medically necessary and, therefore, covered when used as a replacement therapy in individuals with primary immunodeficiencies, in whom severe impairment of antibody capacity is present in the following conditions*:
    • Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia
    • Cerebellar ataxia with defective DNA repair
    • Common variable immunodeficiency
    • Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
    • Congenital agammaglobulinemia
    • Di George's syndrome
    • Hyperimmunoglobulin E [IgE] syndrome
    • Nonfamilial hypogammaglobulinemia
    • Purine nucleoside phosphorylase [PNP] deficiency
    • Selective deficiency of immunoglobulin A [IgA]
    • Selective deficiency of immunoglobulin M [IgM]
    • Selective deficiency of IgG subclasses
    • Severe combined immunodeficiencies
    • Transient hypogammaglobulinemia of infancy
    • Wiskott-Aldrich syndrome
    • X-linked immunodeficiency with hyper-IgM
    • Deficient qualitative or quantitative antibody production
    • At least one bacterial infection directly attributable to this deficiency
* Coverage for primary immunodeficiencies not included in this list may be reviewed for coverage through applicable Part D benefits. Individual benefits must be verified.

INTRAVENOUS IMMUNE GLOBULIN (IVIG)
Use of intravenous immune globulin (IVIG) therapy (e.g., AscenivTM, Bivigam®, Carimune NF®, Flebogamma®, Gammagard Liquid®, Gammagard S/D®, Gammaked®, Gammaplex®, Gamunex-C®, Octagam®, Panzyga®, Privigen®) is considered medically necessary and, therefore, covered for the following indications when the dosing and frequency requirements listed in Attachment B and the following criteria are met:

Serious Defects of Antibody Function
    • Primary immunodeficiency
    • Immune-mediated thrombocytopenia
    • Kawasaki disease
    • Human immunodeficiency virus (HIV) (individuals younger than 14 years of age)
    • Bone marrow transplantation
    • Chronic B-cell lymphocytic leukemia

Dermatologic

Autoimmune Mucocutaneous Blistering Diseases

IVIG is considered medically necessary and, therefore, covered for the treatment of any of the following biopsy-proven conditions (i.e., pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid (a.k.a., cicatricial pemphigoid) benign mucous membrane pemphigold, with or without mention of ocular movement, epidermolysis bullosa acquisita), and meets at least one of the following criteria:
    • Failed conventional therapy
    • Conventional therapy is otherwise contraindicated
    • Rapidly progressive disease in which a clinical response could not be effected quickly enough using conventional agents. In such situations IVIG therapy would be given along with conventional treatment(s), and the IVIG would be used only until the conventional therapy could take effect.
In addition, IVIG for the treatment of autoimmune mucocutaneous blistering diseases must be used only for short-term therapy and not as a maintenance therapy.

Erythema Multiforme Major (Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis)

IVIG is considered medically necessary and, therefore, covered for the one-time treatment of erythema multiforme major (Stevens-Johnson syndrome and toxic epidermal necrolysis) in individuals with a SCORTEN (Severity-of-Illness Score for Toxic Epidermal Necrolysis) level of 3 or greater.

Scleromyxedema

IVIG is considered medically necessary and, therefore, covered for the treatment of scleromyxedema.
Review of medical records should be expected if therapy extends longer than 6 months to assess overall improvement and whether the provider is using the least amount of IVIG to maintain the changes. Long-term treatment is not expected to be seen for this indication.

Hematologic

Idiopathic Thrombocytopenic Purpura (ITP)

For acute ITP, IVIG is considered medically necessary and, therefore, covered for the following indications:
    • Management of acute bleeding due to severe thrombocytopenia (platelet counts usually less than 30,000/uL)
    • To increase platelet counts prior to invasive surgical procedures, e.g., splenectomy
    • Severe thrombocytopenia (platelet counts less than 20,000/uL) considered to be at risk for intracerebral hemorrhage.

Chronic refractory ITP is considered medically necessary and, therefore, covered for individuals meeting all of the following conditions:
    • Prior treatment with corticosteriods and splenectomy, except when contraindicated
    • Duration of illness of greater than six months
    • No concurrent illness/disease explaining thrombocytopenia
    • Platelet counts persistently at or below 20,000/uL

Autoimmune Hemolytic Anemia

IVIG is considered medically necessary and, therefore, covered in individuals with warm-type autoimmune hemolytic anemia that does not respond to corticosteroids or splenectomy, or in individuals for whom corticosteroids or splenectomy are contraindicated.

Rh and ABO incompatibility (moderate to severe)

IVIG is considered medically necessary and, therefore, covered for Rh and ABO incompatibility (moderate to severe) as treatment in addition to phototherapy to prevent or delay exchange transfusion in neonates

Systemic Capillary Leak Syndrome (SCLS) or Clarkson's Disease

IVIG is considered medically necessary and, therefore, covered in individuals with Systemic Capillary Leak Syndrome (SCLS) or Clarkson's Disease when associated with monoclonal gammopathy and used for prophylaxis to increase survival

Immunodeficiency Syndromes, Primary and Secondary

Antibody-Mediated Rejection (AMR)

IVIG is considered medically necessary and, therefore, covered for an individual with antibody-mediated rejection (AMR) after a transplant

Chronic Lymphocytic Leukemia (CLL)

IVIG is considered medically necessary and, therefore, covered when used to prevent recurrent bacterial infections in individuals with B-cell chronic lymphocytic leukemia who meet all of the following criteria:
    • Documented diagnosis of CLL
    • An immunoglobulin G (IgG) level of less than 600 mg/dL
    • Recent history of serious bacterial infection(s) requiring either oral or parenteral antibiotic therapy

Hematopoietic Stem Cell Transplant (HSCT)

IVIG is considered medically necessary and, therefore, covered in an adult, adolescent, or pediatric allogeneic stem cell transplant recipient who experiences severe hypogammaglobulinemia (IgG less than or equal to 400 mg/dL).

Human Immunodeficiency Virus (HIV) Infection

IVIG is considered medically necessary and, therefore, covered to reduce significant bacterial infection for individuals infected with HIV who meet all of the following conditions:
    • Individual is younger than 14 years of age
    • Evidence of either qualitative or quantitative humoral immunologic defects
    • Current bacterial infections, despite appropriate antimicrobial prophylaxis

Multiple Myeloma

IVIG is considered medically necessary and, therefore, covered for an individual with multiple myeloma and associated hypogammaglobulinemia who has a high risk of recurrent infections despite prophylactic antibiotic therapy

Primary Humoral Immunodeficiencies

IVIG is considered medically necessary and, therefore, covered when used as a replacement therapy in individuals with primary immunodeficiencies in whom severe impairment of antibody capacity is present in the following conditions*:
    • Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia
    • Cerebellar ataxia with defective DNA repair
    • Common variable immunodeficiency
    • Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
    • Congenital agammaglobulinemia
    • Di George's syndrome
    • Hyperimmunoglobulin E [IgE] syndrome
    • Nonfamilial hypogammaglobulinemia
    • Purine nucleoside phosphorylase [PNP] deficiency
    • Selective deficiency of immunoglobulin A [IgA]
    • Selective deficiency of immunoglobulin M [IgM]
    • Selective deficiency of IgG subclasses
    • Severe combined immunodeficiencies
    • Transient hypogammaglobulinemia of infancy
    • Wiskott-Aldrich syndrome
    • X-linked immunodeficiency with hyper-IgM
    • Deficient qualitative or quantitative antibody production
    • At least one bacterial infection directly attributable to this deficiency
* Coverage for primary immunodeficiencies not included in this list may be reviewed for coverage through applicable Part D benefits. Individual benefits must be verified.

Solid Organ Transplant

IVIG is considered medically necessary and, therefore, covered for a solid organ transplant (e.g., kidney, heart, lung, liver) when the individual has had a transplant.

Neurological and Musculoskeletal Disorders

Chronic Inflammatory Demyelinating Polyneuritis (CIDP)

IVIG is considered medically necessary and, therefore, covered for CIPD when the individual has a documented diagnosis of CIDP.

Dermatomyositis, Polymyositis

IVIG is considered medically necessary and, therefore, covered in individuals who meet all of the following indications:
    • Individual has refractory myopathy, defined as a disease that is unresponsive or poorly responsive to high-dose steroids either alone or in combination with other immunosuppressive agents (azathioprine, cyclophosphamide, methotrexate). Also included in this definition are individuals who are responsive to but intolerant of continual high-dose steroids as reflected by severe adverse side effects (e.g., steroid myopathy or severe osteoporosis) in whom trials of other immunosuppressive agents, unless contraindicated, have been unsuccessful in achieving significant long-term steroid dose reductions.
    • Individual has biopsy-proven disease (or unequivocal diagnostic features through history, exam, and EMG/NCS studies)
    • Individual is refractory to, intolerant of, or has a contraindication to, a four-month trial of prednisone or prednisone combination therapies.
    • Individual lacks response or has poor response to therapies as reflected by persistently elevated serum creatine kinase (CK) levels and/or lack of improvement on muscle strength improvement scale.

Guillain-Barre Syndrome

IVIG is considered medically necessary and, therefore, covered for Guillain-Barre syndrome when the individual's function is impaired (e.g., unable to stand or walk without aid), measured by a standard clinical scale and/or objective findings on physical exam at the time of initial therapy.

Lambert-Eaton Myasthenic Syndrome

IVIG is considered medically necessary and, therefore, covered for Lambert-Eaton Myasthenic syndrome when the individual is unresponsive/refractory to, intolerant of, or has a contraindication to, other standard therapies (e.g., plasma exchange, corticosteroids or other immunosuppressants, cholinesterase inhibitors, or 3,4-diaminopyridine [DAP]).

Multifocal Motor Neuropathy

IVIG is considered medically necessary and, therefore, covered as initial therapy in individuals who have progressive, symptomatic, multifocal motor neuropathy that has been diagnosed on the basis of electrophysiologic findings that rule out other possible conditions that may not respond to this treatment.

Multiple Sclerosis (MS)

IVIG is considered medically necessary and, therefore, covered in individuals as a second-line therapy in acute relapses of relapsing remitting MS, but is generally not considered effective for maintenance therapy of MS or in slowing disease progression.

Myasthenia Gravis Syndrome

IVIG is considered medically necessary and, therefore, covered for myasthenia gravis syndrome when the individual is refractory to other standard therapies (e.g., plasmapheresis) or to standard pharmacological therapies (e.g., corticosteroids, azathioprine, cyclosporine, cyclophosphamide, cholinesterase inhibitors) given in therapeutic doses over at least 8 weeks, or is intolerant of, or has a contraindication to, standard therapies.

Myasthenic Crisis

IVIG is considered medically necessary and, therefore, covered for myasthenic crisis (i.e., an acute episode of respiratory muscle weakness) when there is a contraindication to plasma exchange.

Stiff-Person Syndrome

IVIG is considered medically necessary and, therefore, covered for Stiff-Person syndrome in individuals who are refractory to other standard therapies (e.g., muscle relaxants, benzodiazepines, and gabapentin-related medications).

Systemic Lupus Erythematosus (SLE)

IVIG is considered medically necessary and, therefore, covered for individuals with severe active Systemic Lupus Erythematosus (SLE) for whom other interventions have been unsuccessful, have become intolerable, or are contraindicated

CONTINUATION OF THERAPY FOR IVIG, SCIG

Once treatment with immune globulin (IVIG, SCIG) is initiated, documentation of the individual's progress is required. If there is initial improvement and continued treatment is necessary, then objective clinical assessment to monitor the progress is required. Objective monitoring may be done with any accepted clinical method such as the Medical Research Council (MRC) scale, Rankin score, Activities of Daily Living (ADL) scores, and/or objective findings on physical exam. Changes in these measures and the relationship of the change to immune globulin use are expected to be documented clearly. Subjective improvement is insufficient to continue immune globulin treatment. Clinical monitoring takes clear precedence over laboratory monitoring. If clinical improvement is evident, then laboratory monitoring solely to guide immune globulin therapy is not necessary.

EXPERIMENTAL/INVESTIGATIONAL (SCIG, IVIG)

All other uses for SCIG, IVIG are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the Company medical policy on off-label coverage for prescription drugs and biologics.

NONCOVERED (IVIG)

Treatment of inclusion body myositis with IVIG is not covered by the Company because this indication is not covered by Medicare. Per Medicare, this condition is generally refractory to all therapies and its rate of progression appears to be unaltered by most therapies; therefore, it is not eligible for reimbursement consideration.

Immune modulation of highly sensitized individuals prior to transplantation with IVIG has not been proven safe and effective and is, therefore, not covered.

NOT ELIGIBLE FOR REIMBURSEMENT

Vivaglobin® is no longer manufactured and has been withdrawn from the market; therefore, it is not eligible for reimbursement.

DOSING AND FREQUENCY REQUIREMENTS

The Company reserves the right to modify the Dosing and Frequency Requirements listed in this Policy to ensure consistency with the most recently published recommendations for the use of IVIG and SCIG. Changes to these guidelines are based on a consensus of information obtained from resources such as, but not limited to, the US Food and Drug Administration (FDA), drug manufacturer’s guidelines, Company-recognized authoritative pharmacology compendia, or published peer-reviewed clinical research. The professional provider must supply supporting documentation (i.e., published peer-reviewed literature) in order to request coverage for an amount of IVIG and SCIG outside of the Dosing and Frequency Requirements listed in this policy. For a list of Company-recognized pharmacology compendia and criteria for peer-reviewed clinical research, view the policy on off-label coverage for prescription drugs and biologics.

Accurate member information is necessary for the Company to approve the requested dose and frequency of these drugs. If the member’s dose, frequency, or regimen changes (based on factors such as changes in member weight or incomplete therapeutic response), the provider must submit those changes to the Company for a new approval based on those changes as part of the precertification process. The Company reserves the right to conduct post-payment review and audit procedures for any claims submitted for IVIG and SCIG.

Refer to Attachment B for dosing and frequency requirements for IVIG and SCIG.

REQUIRED DOCUMENTATION

When coverage of IVIG and SCIG is requested outside of the Dosing and Frequency Guidelines listed in this policy, the prescribing professional provider must supply documentation (i.e., published peer-reviewed literature) to the Company that supports this request.

The Company may conduct reviews and audits of services to our members regardless of the participation status of the provider. Medical record documentation must be maintained on file to reflect the medical necessity of the care and services provided. These medical records may include but are not limited to: records from the professional provider’s office, hospital, nursing home, home health agencies, therapies, and test reports.

PRESCRIPTION (ORDER) REQUIREMENTS
Before submitting a claim to the Company, the supplier must have on file a timely, appropriate, and complete order for each item billed that is signed and dated by the professional provider who is treating the member. Requesting a provider to sign a retrospective order at the time of an audit or after an audit for submission as an original order, reorder, or updated order will not satisfy the requirement to maintain a timely professional provider order on file.

PROOF OF DELIVERY
Medical record documentation must include a contemporaneously prepared delivery confirmation or member’s receipt of supplies and equipment. The medical record documentation must include a copy of delivery confirmation if delivered by a commercial carrier and a signed copy of delivery confirmation by member/caregiver if delivered by the DME supplier/provider. All documentation is to be prepared contemporaneous with delivery and be available to the Company upon request.

CONSUMABLE SUPPLIES
The durable medical equipment (DME) supplier must monitor the quantity of accessories and supplies an individual is actually using. Contacting the individual regarding replenishment of supplies should not be done earlier than approximately seven days prior to the delivery/shipping date. Dated documentation of this contact with the individual is required in the individual’s medical record. Delivery of the supplies should not be done earlier than approximately five days before the individual would exhaust their on-hand supply.

If required documentation is not available on file to support a claim at the time of an audit or record request, the durable medical equipment (DME) supplier may be required to reimburse the Company for overpayments.
Policy Guidelines

This policy is consistent with Medicare’s coverage determination. The Company’s payment methodology may differ from Medicare.

BLACK BOX WARNINGS

Refer to the specific manufacturer's prescribing information for any applicable Black Box Warnings.

SCORTEN SCALE

The SCORTEN scale (SCORe of Toxic Epidermal Necrosis) is a tool that assesses 7 independent risk factors for high mortality. Factors include: age above or below 40 years, if an associated malignancy is present, heart rate above or below 120 beats/min, serum BUN above or below 27 mg/dL, if the detached/compromised body surface impacts greater than or less than 10% of the body, serum bicarbonate level above or below 20 mEq/L, and serum glucose above or below 250 mg/dL. A high SCORTEN score, usually leads to a high mortality rate.

BENEFIT APPLICATION

Subject to the terms and conditions of the applicable Evidence of Coverage, SCIG and IVIG are covered under the medical benefits of the Company’s Medicare Advantage products when the medical necessity criteria, dosing and frequency requirements, and precertification/preapproval requirements listed in this medical policy are met.

Certain drugs may be available through either the member's medical benefit (Part B benefit) or pharmacy benefit (Part D benefit), depending upon how the drug is prescribed and dispensed or administered. This medical policy only addresses instances when IVIG and SCIG may be covered under a member's medical benefit. It does not address instances when IVIG and SCIG may be covered under a member’s pharmacy benefit.

For individuals with primary immunodeficiency, IVIG and SCIG in the home setting may be covered under the medical benefit (Part B benefit). All other clinical indications in the home setting may be covered under the pharmacy benefit (Part D benefit). Refer to the policy entitled "Medicare Part B vs. Part D Crossover Drugs - MA08.007" for appropriate benefit application.

US FOOD AND DRUG ADMINISTRATION (FDA) STATUS

The FDA has approved numerous formulations of SCIG and IVIG.

Description

Intravenous immune globulin (IVIG) is a blood product prepared from the pooled plasma of donors. It has been used to treat a variety of autoimmune diseases, including mucocutaneous blistering diseases. It has fewer side effects than steroids or immunosuppressive agents.

Intravenous immune globulin (IVIG) can replace missing antibodies and decrease infection in primary immune deficiency and chronic lymphocytic leukemia, increase platelets in idiopathic thrombocytopenic purpura, prevent complications in Kawasaki disease, and possibly decrease morbidity in some other conditions.

IVIG is the preferred treatment method for patients who require an immediate increase in intravascular immunoglobulin antibody levels and are unable to produce sufficient amounts of immunoglobulin G (IgG) antibodies. The therapeutic effect of IVIG is immediate, well tolerated, and less likely to produce side effects if infused at the properly indicated rate(s). Sensitivity to these reactions is usually related to the infusion rate. Caution should be exercised in the administration of intravenous immune globulin: reactions may cause a rapid fall in blood pressure and clinical anaphylaxis.

The US Food and Drug Administration (FDA) has approved the subcutaneous infusion preparation of an immune globulin product (SCIG) for the prevention of serious infections in those with primary immunodeficiency disease (PIDD). SCIG is given under the skin using an infusion pump, usually on a weekly basis, in CIDP and PIDD. SCIG may be an option for individuals who have received IVIG and wish to transition to the subcutaneous infusion route. Subcutaneous infusion of immune globulin allows for an alternative route of administration in those who may have problems with chronic IV administration, and enables individuals to self-administer the product at home.

Note: There are a few formulations of IVIG that may be administered via subcutaneous infusion for the indication of PIDD (e.g., Gammagard Liquid®, Gamunex-C®, Gammaked®).

OFF-LABEL INDICATIONS

There may be additional indications contained in the Policy section of this document due to evaluation of criteria highlighted in the Company’s off-label policy, and/or review of clinical guidelines issued by leading professional organizations and government entities.
References

American Hospital Formulary Service (AHFS). Drug Information 2019. Immune Globulin. updated 12/04/17. [Lexicomp Online Web site]. Available at: http://online.lexi.com/lco/action/home [via subscription only]. Accessed March 11, 2019.

Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 2000 Aug;115(2):149-53.

Centers for Medicare and Medicaid Services (CMS). Medicare Benefit Policy Manual. Chapter 15 – Covered Medical and Other Health Services. 50.6 – Coverage of Intravenous Immune Globulin for Treatment of Primary Immune Deficiency Diseases in the Home. Issued 09/03/14. Available at: http://www.cms.gov/Regulations-and-Guidance/Guidance/Manuals/Internet-Only-Manuals-IOMs-Items/CMS012673.html . Accessed February 7, 2019.

Centers for Medicare and Medicaid Services (CMS). National Coverage Determination (NCD). 250.3 NCD for intravenous immune globulin for the treatment of autoimmune mucocutaneous blistering diseases. [CMS Web Site]. 10/01/02. Available at: http://www.cms.gov/medicare-coverage-database/details/ncd-details.aspx?NCDId=158&ncdver=1&CoverageSelection=Both&ArticleType=All&PolicyType=Final&s=Pennsylvania&KeyWord=Immune+Globulin&KeyWordLookUp=Title&KeyWordSearchType=And&bc=gAAAABAAAAAAAA%3d%3d& . Accessed February 7, 2019.

Cuvitru, Immune Globulin Subcutaneous (Human), 20% Solution approval and prescribing information. Baxalta US Inc.: Lexington, MA. 07/2018. Available at: https://www.cuvitru.com/ . Accessed February 11, 2019.

Elsevier’s Clinical Pharmacology Compendium. Immune Globulin IV, IVIG, IGIV. [Clinical Key Web site]. 01/15/19. Available at: https://www.clinicalkey.com/#!/content/drug_monograph/6-s2.0-311 [via subscription only]. Accessed March 7, 2019.

Gammagard Liquid package insert. Baxalta US Inc.: Westlake, CA. 06/2016. Available at: https://www.shirecontent.com/pi/pdfs/gamliquid_usa_eng.pdf . Accessed February 11, 2019.

Gammaked®. package insert. Grifols Therapeutics Inc. Kedrion Biopharma Inc; Fort Lee, NJ. Revised 03/2017. Available at: http://www.gammaked.com/ . Accessed February 11, 2019.

Gamunex-C package insert. Research Triangle Park, NC: Grifols Therapeutics LLC. 06/2018. Available at: https://www.gamunex-c.com/en/hcp . Accessed February 11, 2019.

Hizentra package insert. Kankakee, IL: CSL Behring LLC. 03/2018. Available at:
https://www.hizentra.com/prescribing-information . Accessed February 6, 2019.

HyQvia. package insert. Baxalta US Inc.: Lexington, MA. 01/2019. Available at: http://www.hyqvia.com/ . Accessed February 11, 2019.

Lexi-Drugs Compendium. Immune Globulin. [Lexicomp Online Web site]. 03/05/19. Available at: http://online.lexi.com/lco/action/home [via subscription only]. Accessed March 11, 2019.

Noridian Healthcare Solutions. Local Coverage Determination (LCD and Policy Article). L33794 & A52507. External infusion pumps. L33794 Original 10/01/2015, Revised 03/29/2018. A52507 Original 10/01/2015, Revised 01/01/2018. Available at: https://med.noridianmedicare.com/web/jadme/policies/lcd/active or https://med.noridianmedicare.com/documents/2230703/7218263/External+Infusion+Pumps+LCD . Accessed February 8, 2019.

Noridian Healthcare Solutions. Local Coverage Determination (LCD and Policy Article). L33610 & A52509. Intravenous Immune Globulin. Original 10/01/2015, Revised 01/01/2017. Available at: https://med.noridianmedicare.com/web/jadme/policies/lcd/active or https://med.noridianmedicare.com/documents/2230703/7218263/Intravenous+Immune+Globulin+LCD. Accessed February 7, 2019.

Novitas Solutions, Inc. Local Coverage Article (A53127). Self Administered Drug Exclusion List. [Novitas Solutions Web site]. Original 10/01/2015, Revised 03/15/2018.
Available at: https://www.cms.gov/medicare-coverage-database/details/article-details.aspx?articleId=53127&ver=51&CoverageSelection=Both&ArticleType=All&PolicyType=Final&s=Pennsylvania&KeyWord=Self+Administered+Drug+Exclusion+list&KeyWordLookUp=Title&KeyWordSearchType=And&bc=gAAAACAAAAAA& . Accessed February 7, 2019.

Novitas Solutions, Inc. Local Coverage Article(LCD)A56786 - Billing and Coding: Intravenous Immune Globulin (IVIG). [Novitas Solutions Web site]. Original 08/08/2019, Revised 08/13/2019. Available at: https://www.cms.gov/medicare-coverage-database/details/article-details.aspx?articleId=56786&ver=5&name=331*1&UpdatePeriod=847&bc=AAAAEAAAAAAA& . Accessed August 23, 2019.

Novitas Solutions, Inc. Local Coverage Determination (LCD)L35093 - Intravenous Immune Globulin (IVIG). [Novitas Solutions Web site]. Original 10/01/2015, Revised 08/22/2019. Available at: https://www.cms.gov/medicare-coverage-database/details/lcd-details.aspx?LCDId=35093&ver=97&articleId=56860&name=331*1&UpdatePeriod=847&bc=AAAAEAAAAAAA& . Accessed August 23, 2019.

Truven Health Analytics Inc. Micromedex® Solutions. DrugDex®. [Internet database]. Immune Globulin. 01/25/19. Available at:
http://www.micromedexsolutions.com/micromedex2/librarian/ [via subscription only]. Accessed March 11, 2019.

US Food and Drug Administration (FDA). Vaccines, Blood & Biologics. Cutaquig. Immune Globulin Subcutaneous (Human), 16.5% Solution. Approval and prescribing information. 12/12/2018. Available at: https://www.fda.gov/BiologicsBloodVaccines/ucm628258.htm . Accessed February 8, 2019.

US Food and Drug Administration (FDA). Vaccines, Blood & Biologics. Xembify. Immune Globulin Subcutaneous, human-klhw, 20% Solution. Approval and prescribing information. 07/03/2019. Available at: https://www.fda.gov/vaccines-blood-biologics/xembify . Accessed July 12, 2019.

US Food and Drug Administration (FDA). Vaccines, Blood & Biologics. Immune Globulin Intravenous (Human)-slra (Asceniv) prescribing information. Updated: 04/02/2019. [FDA Web site]. Available at: https://www.fda.gov/BiologicsBloodVaccines/ucm634956.htm . Accessed April 8, 2019.

US Food and Drug Administration (FDA). Vaccines, Blood & Biologics. Panzyga. Immune Globulin Intravenous, human-ifas, 10% Solution. Approval and prescribing information. 08/02/2018. Available at: https://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm615698.htm . Accessed February 6, 2019.

US Food and Drug Administration (FDA). Vaccines, Blood & Biologics. Immune Globulin Intravenous (Human) approved products. Updated: 02/09/2019. [FDA Web site]. Available at: http://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm127589.htm . Accessed February 11, 2019.

US Food and Drug Administration (FDA). Vaccines, Blood & Biologics. Immune globulin intravenous (IGIV) indications. 03/08/2018. [FDA Web site]. Available at: http://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm133691.htm . Accessed February 11, 2019.


Coding

Inclusion of a code in this table does not imply reimbursement. Eligibility, benefits, limitations, exclusions, precertification/referral requirements, provider contracts, and Company policies apply.

The codes listed below are updated on a regular basis, in accordance with nationally accepted coding guidelines. Therefore, this policy applies to any and all future applicable coding changes, revisions, or updates.

In order to ensure optimal reimbursement, all health care services, devices, and pharmaceuticals should be reported using the billing codes and modifiers that most accurately represent the services rendered, unless otherwise directed by the Company.

The Coding Table lists any CPT, ICD-9, ICD-10, and HCPCS billing codes related only to the specific policy in which they appear.

CPT Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD - 10 Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD -10 Diagnosis Code Number(s)

See Attachment A


HCPCS Level II Code Number(s)



MEDICALLY NECESSARY

SUBCUTANEOUS IMMUNE GLOBULIN (SCIG)

J1555 Injection, immune globulin (cuvitru), 100 mg

J1559 Injection, immune globulin (Hizentra), 100 mg

J1561 Injection, immune globulin, (Gamunex/Gamunex-C/Gammaked), nonlyophilized (e.g., liquid), 500 mg

J1569 Injection, immune globulin, (Gammagard liquid), nonlyophilized, (e.g., liquid), 500 mg

The following code is used to represent HyQvia:
J1575 Injection, immune globulin/hyaluronidase, 100 mg immuneglobulin

The following codes are used to represent Cutaquig, Xembify:
C9399 Unclassified drugs or biologicals

J3590 Unclassified biologics


INTRAVENOUS IMMUNE GLOBULIN (IVIG)

J1459 Injection, immune globulin (Privigen), intravenous, nonlyophilized (e.g., liquid), 500 mg

J1556 Injection, immune globulin (Bivigam), 500 mg

J1557 Injection, immune globulin, (Gammaplex), intravenous, nonlyophilized (e.g., liquid), 500 mg

J1561 Injection, immune globulin, (Gamunex/Gamunex-C/Gammaked), nonlyophilized (e.g., liquid), 500 mg

J1568 Injection, immune globulin, (Octagam), intravenous, nonlyophilized (e.g., liquid), 500 mg

J1569 Injection, immune globulin, (Gammagard liquid), nonlyophilized, (e.g., liquid), 500 mg

J1572 Injection, immune globulin, (Flebogamma/Flebogamma Dif), intravenous, nonlyophilized (e.g., liquid), 500 mg

S9338 Home infusion therapy, immunotherapy, administrative services, professional pharmacy services, care coordination, and all necessary supplies and equipment (drugs and nursing visits coded separately), per diem

The following code is used to represent CARIMUNE NF AND GAMMAGARD S/D:
J1566 Injection, immune globulin, intravenous, lyophilized (e.g., powder), not otherwise specified, 500 mg

The following code is used to represent AscenivTM and Panzyga® :
J1599 Injection, immune globulin, intravenous, nonlyophilized (e.g., liquid), not otherwise specified, 500mg

NOT ELIGIBLE FOR REIMBURSEMENT

THE FOLLOWING CODE IS USED TO REPRESENT VIVAGLOBIN AND IS NO LONGER MANUFACTURED AND HAS BEEN WITHDRAWN FROM THE MARKET:
J1562 Injection, immune globulin (Vivaglobin), 100 mg



Revenue Code Number(s)

N/A


Misc Code

N/A:

N/A


Coding and Billing Requirements


Cross References

Attachment A: Immune Globulin: Intravenous (IVIG), Subcutaneous (SCIG)
Description: ICD-10 DIAGNOSIS CODES

Attachment B: Immune Globulin: Intravenous (IVIG), Subcutaneous (SCIG)
Description: Dosage and Frequency Requirements






Policy History

Revisions from MA08.009h
10/21/2019This policy was updated to communicate the Company's coverage criteria, including Dosing and Frequency, of the new SCIG product, Xembify.

Per Novitas Solutions, Inc. "MM11295 – Update to Coverage of Intravenous Immune Globulin for Treatment of Primary Immune Deficiency Diseases in the Home", additional primary immune deficiency diseases and codes were added to this policy for IVIG and SCIG.

Revisions from MA08.009g
06/17/2019This Policy was revised with the following changes:
  • The following new SCIG product was added: Cutaquig
  • The following new IVIG products were added: AscenivTM, Panzyga®.
  • New FDA-approved indication for SCIG (e.g., Hizentra®) for CIDP was added.
  • New indication for IVIG, per Novitas L35093, for Systemic Capillary Leak Syndrome (SCLS) or Clarkson's Disease: IVIG is considered medically necessary and, therefore, covered in individuals with Systemic Capillary Leak Syndrome (SCLS) or Clarkson's Disease when associated with monoclonal gammopathy and used for prophylaxis to increase survival
  • Lambert-Eaton: plasma exchange was added an as example of prior therapies.

The following CPT codes were removed from the policy: 90283, 90284

Attachment B: Dosage and Frequency
    SCIG Section:
    PIDD Induction dosing was expanded from 100-150 mg/kg/weekly to 100-200 mg/kg/weekly.
    PIDD: Cutaquig dosing was added.
    CIDP: New Indication. Hizentra dosing was added.

    IVIG Section: Dosing and Frequency were added/adjusted for the following IVIG indications:
    Scleromyxedema
    Systemic Capillary Leak Syndrome (SCLS) or Clarkson's Disease
    Antibody-Mediated Rejection (AMR)
    Guillain-Barre syndrome
    Myasthenia gravis syndrome

Revisions from MA08.009f
10/22/2018This version of the policy will become effective 10/22/2018.

This policy has been updated to include coverage changes, per Novitas Solutions, Inc., to:
  • Include the following subtypes of Primary Humoral Immunodeficiencies: Nonfamilial hypogammaglobulinemia, Selective deficiency of IgG subclasses, and Transient hypogammaglobulinemia of infancy
  • Add coverage criteria for antibody-mediated rejection (AMR), multiple myeloma, and Rh and ABO incompatibility
  • Clarify types of solid organs included in transplant coverage.


Revisions from MA08.009e
01/01/2018This policy has been identified for the HCPCS code update, effective 01/01/2018.

The following NOC code has been removed from this policy and is replaced by the following HCPCS code:
REMOVED: J3590 Unclassified biologics
REPLACED WITH: J1555 Injection, immune globulin (cuvitru), 100 mg


MA08.009d
10/01/2017This policy has been identified for the ICD-10 CM code update, effective 10/01/2017.

The following ICD-10 CM codes have been added to this policy for IVIG only:

M33.03 Juvenile dermatomyositis without myopathy
M33.13 Other dermatomyositis without myopathy
M33.93 Dermatopolymyositis, unspecified without myopathy


The following ICD-10 CM narratives have been revised in this policy for IVIG only:

M33.00
FROM: Juvenile dermatopolymyositis, organ involvement unspecified
TO: Juvenile dermatomyositis, organ involvement unspecified

M33.01
FROM: Juvenile dermatopolymyositis with respiratory involvement
TO: Juvenile dermatomyositis with respiratory involvement

M33.02
FROM: Juvenile dermatopolymyositis with myopathy
TO: Juvenile dermatomyositis with myopathy

M33.09
FROM: Juvenile dermatopolymyositis with other organ involvement
TO: Juvenile dermatomyositis with other organ involvement

M33.10
FROM: Other dermatopolymyositis, organ involvement unspecified
TO: Other dermatomyositis, organ involvement unspecified

M33.11
FROM: Other dermatopolymyositis with respiratory involvement
TO: Other dermatomyositis with respiratory involvement

M33.12
FROM: Other dermatopolymyositis with myopathy
TO: Other dermatomyositis with myopathy

M33.19
FROM: Other dermatopolymyositis with other organ involvement
TO: Other dermatomyositis with other organ involvement

MA08.009c
12/28/2016This policy was updated to reflect current Medicare Policies. The new SCIG product, Cuvitru, was added to the Policy and to the Dosing and Frequency Attachment. In the Dosing and Frequency Attachment, Kawasaki disease was added to this attachment and Scleromyxedema was revised. In addition, extensive coding changes were made to reflect the Policy's covered indications; as such, the following codes have been removed from this policy: D80.3, T86.19, T86.298, T86.39, T86.49, T86.818, T86.898.

MA08.009b
03/01/2016This policy was updated to communicate the following changes:

New indications with Policy and Dosing criteria were added, per Novitas LCD L32712: Erythema multiforme major (Stevens-Johnson syndrome and toxic epidermal necrolysis), Scleromyxedema, and Systemic Lupus Erythematosus (SLE).

Product and Dosing criteria were added/updated per FDA labeling or peer-reviewed literature: Hizentra (SCIG), HyQvia (SCIG), Multiple sclerosis, CLL, and pediatric individuals with dermatomyositis or polymyositis.

Noncovered positions were added for the following indications, per Novitas LCD L35093: Immune Modulation of highly sensitized individuals prior to transplantation and the Treatment of inclusion body myositis.

Policy criteria were updated for the following indications, per Novitas LCD L32712: Multifocal Motor Neuropathy, Dermatomyositis and Polymyositis.

Policy and dosing criteria were updated for the following indications, per Novitas LCD L32712: Chronic refractory ITP and Myasthenia Gravis Syndrome. Antibody-mediated rejection (AMR) was removed as a covered indication.

Policy was updated for Vivaglobin, a SCIG product, that is no longer available; therefore, so longer eligible for reimbursement.

The coding table has been updated to reflect above criteria. ICD-9 codes were removed from this policy since as of 10/01/2015, ICD-10 codes are effective.

SCIG information was added to the Description Section.

The criteria for required documentation that must be kept regarding DME and Home Infusion related supplies was updated.

MA08.009a
01/01/2016This policy has been identified for the HCPCS code update, effective 01/01/2016.
The following HCPCS code has been added to this policy, as Medically Necessary:
    J1575 Injection, immune globulin/hyaluronidase, (hyqvia), 100 mg immunoglobulin


MA08.009
01/01/2015This is a new policy.




Version Effective Date: 10/21/2019
Version Issued Date: 10/21/2019
Version Reissued Date: N/A