The use of intravenous immune globulin should be reserved for patients with serious defects of antibody function. The goal is to provide immune globulin to those who lack it. Medicare will provide coverage for intravenous immune globulin when it is used in treatment of the following conditions:
- Primary immunodeficiency.
- Immune-mediated Thrombocytopenia (ITP).
- Human Immunodeficiency Virus (HIV) (for pediatric use only).
- Bone marrow transplantation.
- Chronic B-cell lymphocytic leukemia.
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
disease and possibly decrease morbidity in some other conditions. Kawasaki
IVIG is the preferred treatment method for patients who require 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.
IVIG is covered for treatment of the following biopsy-proven conditions:
- Pemphigus vulgaris.
- Pemphigus foliaceus.
- Bullous pemphigoid.
- Mucous membrane pemphigoid (aka, cicatricial pemphigoid), benign mucous membrane pemphigoid, with or without mention of ocular movement.
- Epidermolysis bullosa acquisita.
Patients must meet at least one of the following criteria:
- Failed conventional therapy. Contractors have the discretion to define what constitutes failure of conventional therapy.
- Conventional therapy is contraindicated. Contractors have the discretion to define what constitutes contraindications to conventional therapy.
- Have rapidly progressive disease in which a clinical response could not be affected quickly enough using conventional agents. In these situations, IVIG therapy would be given along with conventional treatment(s) and the IVIG would be used only until conventional therapy could take effect.
Note: In addition, IVIG for the treatment of autoimmune mucocutaneous blistering disease must be used only for short-term therapy and not as a maintenance therapy.
Other preparations of IVIG are available:
- RhoD immune globulin for use in preventing postpartum Rhesus isoimmunization.
- Cytomegalovirus immune globulin for use in treating or preventing cytomegaloviral disease in transplant recipients.
- Hepatitis B immune globulin intravenous for use in treating prevention of hepatitis B recurrence following liver transplantation in hepatitis B surface antigen (HBsAG)-positive liver transplant patients. (FDA approved April 6, 2007.)
Physicians should avoid prescribing IVIG except for patients with severe immune deficiency and who have low antibody levels or for those whom have other well-established indications for therapy with IVIG as described within this LCD.
Bone Marrow Transplantation (BMT):
Per Medicare National Coverage Determinations Manual – Pub. 100-03, Part 2, Section 110.8.1, IVIG will be covered for use in BMT recipients to reduce the incidence of infections and acute graft versus host disease. Both conditions below must be met for coverage:
- The graft recipient must be 21 years of age or older.
- The BMT must be a covered Medicare procedure.
Primary Humoral Immunodeficiencies:
IVIG will be covered for use as replacement therapy in patients with primary immunodeficiencies in whom severe impairment of antibody capacity is present in the following conditions:
- Congenital agammaglobulinemia.
- Common variable immunodeficiency.
- Wiskott-Aldrich syndrome
- X-linked immunodeficiency with hyper-IgM.
- Severe combined immunodeficiencies.
- Deficient qualitative and/or quantitative antibody production.
- Have at least one bacterial infection directly attributable to this deficiency.
Idiopathic Thrombocytopenic Purpura (ITP):
IVIG will be covered for both acute and chronic refractory ITP.
Acute ITP, IVIG is covered for:
- 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 covered for patients meeting all of the following conditions:
- Prior treatment with corticosteroids and splenectomy.
- Duration of illness of greater than six months.
- Age of 10 years or older.
- No concurrent illness/disease explaining thrombocytopenia.
- Platelet counts persistently at or below 20,000/ul.
Chronic Lymphocytic Leukemia (CLL):
IVIG will be covered when used to prevent recurrent bacterial infections in patients with B-cell chronic lymphocytic leukemia meeting all of the following conditions:
- Must have unequivocally documented 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.
Human Immunodeficiency Virus (HIV) Infection:
IVIG will be covered for patients infected with HIV to reduce significant bacterial infection meeting all of the following conditions:
- Age younger than 14 years old.
- Evidence of either qualitative or quantitative humoral immunologic defects.
- Current bacterial infections, despite appropriate antimicrobial prophylaxis.
Chronic Inflammatory Demyelinating Polyneuritis (CIDP):
The diagnosis of this condition must be documented in the medical record and must be consistent with published diagnostic criteria for this condition.
- The patient has unequivocal CIDP as defined by the mandatory clinical and physiologic or pathologic criteria of the
of Neurology (Neurology 41: pp. 617–618, 1991) or from the Medical Advisory Committee of the Neuropathy Association (J Peripheral Nervous Assn., 2003, 8:282–284). American Academy
- The patient has proved refractory to or intolerant of prednisone or azathioprine given in therapeutic doses over at least three months.
- The patient has a neurologic function assessment score of at least three or greater on the Rankin Scale at the time of initial therapy.
IVIG will not be covered as an initial therapy for patients with newly diagnosed CIDP or as maintenance therapy in patients failing to respond to an initial course of IVIG following therapies with other agents. An exception to IVIG as an initial therapy would be in patients with severe CIDP (Rankin scores of 4 or 5) in whom a rapid therapeutic response is deemed medically desirable or in any patient meeting coverage criteria above (bullets one and three) for whom immunosuppressives are contraindicated. Patients responsive to an initial course of IVIG will be eligible for maintenance therapy coverage only if unequivocal neurological deterioration occurs at some future point in time.
The routine use of IVIG is not usually recommended for polymyositis or dermatomyositis. IVIG may be used in patients with severe active illness for whom other interventions have been unsuccessful, have become intolerable or are contraindicated.
Refractory myopathies are, by definition, diseases that are 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 patients responsive to but intolerant of continual high-dose steroids as reflected by severe adverse side effects (e.g., steroids myopathy or severe osteoporosis) in whom trials of other immunosuppressive agents, unless contraindicated, have been unsuccessful in achieving significant long-term steroid dose reductions.
Three other coverage conditions which must all be met, in addition to the above, are:
- Biopsy-proven disease.
- At least a four- to six-month trail of prednisone or prednisone combination therapies.
- Lack of response/poor response to therapies as reflected by persistently elevated serum Creatine Kinase (CK) levels and/or lack of improvement on muscle strength improvement scales.
Inclusion body myositis is generally refractory to all therapies and its rate of progression appears to be unaltered by most therapies. IVIG will not be covered for use in patients with inclusion body myositis.
Immune Modulation prior to Transplantation
IVIG has not been proven safe and effective when used for immune modulation of highly sensitized patients prior to transplantation and is therefore not covered for this indication.
LCD Individual Consideration
Certain unusual uses of IVIG may be covered on an LCD Individual Consideration basis. Such situations are described in the four conditions below. The LCD Individual Consideration procedure is described in the attached Article.
Autoimmune Hemolytic Anemia:
The routine use of IVIG is not usually recommended. IVIG may have a role in patients with warm-type autoimmune hemolytic anemia that does not respond to corticosteroids or splenectomy or those for whom the latter two treatments are contraindicated. Coverage determination will require LCD Individual Consideration.
Multifocal Motor Neuropathy:
The routine use of IVIG is not usually recommended. IVIG may be considered in patients 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. Coverage determination will require LCD Individual Consideration.
Multiple Sclerosis (MS):
The current evidence is inadequate to assess the value of IVIG in the treatment of multiple sclerosis. IVIG may be useful in persons as a second-line therapy in acute relapses of Relapsing Remitting Multiple Sclerosis (MS), but is generally not considered effective for maintenance therapy of MS or in slowing disease progression. LCD Individual Consideration may be given when IVIG is used in the treatment of an acute relapse of Relapsing Remitting MS.
Systemic Lupus Erythematosus:
The routine use of IVIG is not usually recommended. IVIG may be used in patients with severe active systemic lupus erythematosus for whom other interventions have been unsuccessful, have become intolerable or are contraindicated. Coverage determination will require LCD Individual Consideration.
Medicare provides payment for the discarded drug/biological remaining in a single-use drug product after administering what is reasonable and necessary for the patient’s condition. If the physician has made good faith efforts to minimize the unused portion of the drug/biological in how patients are scheduled and how he ordered, accepted, stored and used the drug and made good faith efforts to minimize the unused portion of the drug in how it is supplied, then the program will cover the amount of drug discarded along with the amount administered. Documentation requirements are given below. Coding and billing instructions can be referenced in the attached article. Reference to national policy:Medicare Claims Processing Manual – Pub. 100-04, Chapter 17, Section 40.
Note: The JW modifier is not used on claims for drugs or biologicals provided under the Competitive Acquisition Program (CAP). Reference to national policy: Medicare Claims Processing Manual, Pub. 100-04, Chapter 17, Section 100.2.9.
Notice: This LCD imposes diagnosis limitations that support diagnosis to procedure code automated denials. However, services performed for any given diagnosis must meet all of the indications and limitations stated in this policy, the general requirements for medical necessity as stated in CMS payment policy manuals, any and all existing CMS national coverage determinations, and all Medicare payment rules.
As published in CMS IOM 100-08, Section 13.5.1, to be covered under Medicare, a service shall be reasonable and necessary. When appropriate, contractors shall describe the circumstances under which the proposed LCD for the service is considered reasonable and necessary under Section 1862(a)(1)(A). Contractors shall consider a service to be reasonable and necessary if the contractor determines that the service is:
- Safe and effective.
- Not experimental or investigational (exception: routine costs of qualifying clinical trial services with dates of service on or after September 19, 2000, which meet the requirements of the clinical trials NCD are considered reasonable and necessary).
- Appropriate, including the duration and frequency that is considered appropriate for the service, in terms of whether it is:
- Furnished in accordance with accepted standards of medical practice for the diagnosis or treatment of the patient’s condition or to improve the function of a malformed body member.
- Furnished in a setting appropriate to the patient’s medical needs and condition.
- Ordered and furnished by qualified personnel.
- One that meets, but does not exceed, the patient’s medical need.
- At least as beneficial as an existing and available medically appropriate alternative.
Bill Type Codes
Contractors may specify Bill Types to help providers identify those Bill Types typically used to report this service. Absence of a Bill Type does not guarantee that the policy does not apply to that Bill Type. Complete absence of all Bill Types indicates that coverage is not influenced by Bill Type and the policy should be assumed to apply equally to all claims.
12X, 13X, 21X, 22X, 23X, 71X, 73X, 77X, 83X, 85X
Bill Type Note: Code 73X end-dated for Medicare use March 31, 2010; code 77X effective for dates of service on or after April 1, 2010.
Contractors may specify Revenue Codes to help providers identify those Revenue Codes typically used to report this service. In most instances Revenue Codes are purely advisory; unless specified in the policy services reported under other Revenue Codes are equally subject to this coverage determination. Complete absence of all Revenue Codes indicates that coverage is not influenced by Revenue Code and the policy should be assumed to apply equally to all Revenue Codes.
Providers are reminded to refer to the long descriptors of the CPT codes in their CPT book. The American Medical Association (AMA) and the Centers for Medicare & Medicaid Services (CMS) require the use of short CPT descriptors in policies published on the Web.
Injection, immune globulin (Gammaplex®), intravenous, non-lyophilized (e.g., liquid), 500 mg
Injection, cytomegaolvirus immune globulin intravenous, human, per vial
Injection, immune globulin (Privigen™), intravenous, non-lyophilized (e.g., liquid), 500 mg
Injection, immune globulin (Gamunex), intravenous, non-lyophilized (e.g. liquid), 500 mg
Injection, immune globulin, intravenous, lyophilized (e.g. powder), not otherwise specified, 500 mg
Injection, immune globulin (Octagam), intravenous, non-lyophilized (e.g. liquid), 500 mg
Injection, immune globulin (Gammagard Liquid), intravenous, non-lyophilized (e.g. liquid), 500 mg
Injection, immune globulin (Flebogamma/Flebogamma dif), intravenous, non-lyophilized (e.g. liquid), 500 mg
Injection, hepatitis B immune globulin (Hepagam B), intravenous, 0.5 ml
Injection, RhoD immune globulin, human, minidose, 50 mcg (250 IU)
Injection, RhoD immune globulin, human, full dose, 300 mcg (1500 IU)
Injection, RhoD immune globulin, intravenous, human, solvent detergent, 100 IU
ICD-10 Codes that Support Medical Necessity
B20* Human immunodeficiency virus [HIV] disease
C91.10 Chronic lymphocytic leukemia of B-cell type not having achieved remission
C91.11 Chronic lymphocytic leukemia of B-cell type in remission
C91.12 Chronic lymphocytic leukemia of B-cell type in relapse
D59.0 Drug-induced autoimmune hemolytic anemia
D59.1 Other autoimmune hemolytic anemias
D69.3 Immune thrombocytopenic purpura
D69.41 Evans syndrome
D69.6* Thrombocytopenia, unspecified
D70.8 Other neutropenia
D80.0 Hereditary hypogammaglobulinemia
D80.1* Nonfamilial hypogammaglobulinemia
D80.5 Immunodeficiency with increased immunoglobulin M [IgM]
D81.0 Severe combined immunodeficiency [SCID] with reticular dysgenesis
D81.1 Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
D81.2 Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
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
D83.0 Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells
D83.8 Other common variable immunodeficiencies
D83.9 Common variable immunodeficiency, unspecified
G25.82 Stiff-man syndrome
G35 Multiple sclerosis
G61.0 Guillain-Barre syndrome
G61.81 Chronic inflammatory demyelinating polyneuritis
G61.82 Multifocal motor neuropathy
G61.89 Other inflammatory polyneuropathies
G62.89 Other specified polyneuropathies
G64 Other disorders of peripheral nervous system
G70.00 Myasthenia gravis without (acute) exacerbation
G70.01 Myasthenia gravis with (acute) exacerbation
H46.8 Other optic neuritis
L10.0 Pemphigus vulgaris
L10.1 Pemphigus vegetans
L10.2 Pemphigus foliaceous
L10.3 Brazilian pemphigus [fogo selvagem]
L10.4 Pemphigus erythematosus
L10.5 Drug-induced pemphigus
L10.81 Paraneoplastic pemphigus
L10.89 Other pemphigus
L10.9 Pemphigus, unspecified
L12.0 Bullous pemphigoid
L12.1 Cicatricial pemphigoid
L12.8 Other pemphigoid
L12.9 Pemphigoid, unspecified
L13.8 Other specified bullous disorders
L14 Bullous disorders in diseases classified elsewhere
L40.1 Generalized pustular psoriasis
M30.3 Mucocutaneous lymph node syndrome [Kawasaki]
M33.00 Juvenile dermatopolymyositis, organ involvement unspecified
M33.01 Juvenile dermatopolymyositis with respiratory involvement
M33.02 Juvenile dermatopolymyositis with myopathy
M33.09 Juvenile dermatopolymyositis with other organ involvement
M33.10 Other dermatopolymyositis, organ involvement unspecified
M33.11 Other dermatopolymyositis with respiratory involvement
M33.12 Other dermatopolymyositis with myopathy
M33.19 Other dermatopolymyositis with other organ involvement
M33.20 Polymyositis, organ involvement unspecified
M33.21 Polymyositis with respiratory involvement
M33.22 Polymyositis with myopathy
M33.29 Polymyositis with other organ involvement
M33.90 Dermatopolymyositis, unspecified, organ involvement unspecified
M33.91 Dermatopolymyositis, unspecified with respiratory involvement
M33.92 Dermatopolymyositis, unspecified with myopathy
M33.99 Dermatopolymyositis, unspecified with other organ involvement
M36.0 Dermato(poly)myositis in neoplastic disease
T86.00 Unspecified complication of bone marrow transplant
T86.01 Bone marrow transplant rejection
T86.02 Bone marrow transplant failure
T86.03 Bone marrow transplant infection
T86.09 Other complications of bone marrow transplant
Z78.9* Other specified health status
Z91.89* Other specified personal risk factors, not elsewhere classified
Z92.21* Personal history of antineoplastic chemotherapy
ICD-9-CM Codes That Support Medical Necessity
The CPT/HCPCS codes included in this LCD will be subjected to “procedure to diagnosis” editing. The following lists include only those diagnoses for which the identified CPT/HCPCS procedures are covered. If a covered diagnosis is not on the claim, the edit will automatically deny the service as not medically necessary.
Medicare is establishing the following limited coverage for CPT/HCPCS code J0850:
Organ or tissue replaced by transplant
Organ or tissue replaced by transplant, other specified organ or tissue
Organ or tissue replaced by transplant, other specified organ or tissue
Medicare is establishing the following limited coverage for CPT/HCPCS codes C9270, J1459, J1561, J1566, J1568, J1569 and J1572:
Human Immunodeficiency Virus (HIV) disease
Note: Use 042* only for patients younger than 14 years of age.
Lymphoid leukemia, chronic
Deficiency of humoral immunity
Other deficiency of humoral immunity
Deficiency of cell mediated immunity, Wiskott Aldrich syndrome
Combined immunity deficiency
Autoimmune hemolytic anemias
Acq hemolytic anemia nos
Constitutional (pure) red blood cell aplasia
Idiopathic peripheral neuropathy
Inflammatory and toxic neuropathy, acute infective polyneuritis
Inflammatory and toxic neuropathy
Myoneural disorders, myasthenia gravis
Acute febrile Mucocutaneous Lymph Node Syndrome (MCLS)
Rhesus isoimmunization, antepartum condition or complication
Bullous dermatoses, other specified
Diffuse diseases of connective tissue
Complications of transplanted organ, bone marrow
Kidney replaced by transplant
Heart replaced by transplant
Lung replaced by transplant
Liver replaced by transplant
Bone marrow replaced by transplant
Trspl sts-perip stm cell
Medicare is establishing the following dual diagnosis limited coverage for CPT/HCPCS code J1573:
Covered for primary diagnosis:
Liver replaced by transplant
The following are secondary (dual) diagnoses to be used with V42.7 to meet limited coverage for CPT/HCPCS codeJ1573:
Viral hepatitis B with hepatic coma
Viral hepatitis B without mention of hepatic coma.
Medicare is establishing the following limited coverage for CPT/HCPCS codes J2788, J2790 and J2792:
Immune thrombocytopenic purpura
Rhesus isoimmunization, antepartum condition or complication
Note: Providers should continue to submit ICD-9-CM diagnosis codes without decimals on their claim forms and electronic claims.
Diagnoses That Support Medical Necessity
ICD-9-CM Codes That DO NOT Support Medical Necessity
Diagnoses That DO NOT Support Medical Necessity
All diagnoses not listed in the “ICD-9-CM Codes That Support Medical Necessity” section of this LCD.
- Documentation supporting medical necessity should be legible, maintained in the patient’s medical record and made available to Medicare upon request.
- The information contained in the medical record should include all relevant diagnostic laboratory studies, prior history of bleeding, infection, disease progression, prior medical/surgical therapies and any other information essential in establishing that the patient meets the coverage indicators as set forth in this LCD.
- Indications for administration of IVIG therapy must be fully documented in the patient’s medical record.
- Physicians or other providers filing Medicare claims for administration of IVIG therapy at the request of another provider assume full responsibility as to the medical necessity for IVIG under terms and conditions of this LCD. These providers must also be able to meet documentation requirements given above, either directly through their own medical records or indirectly through records obtained from the referring physician.
Drug Wastage Documentation Requirements
Any amount wasted must be clearly documented in the medical record, regardless of whether the JW modifier will be used in billing for the drug/biological, with:
- Date and time.
- Amount of medication wasted.
- Reason for the wastage.
Medicare would expect to see IVIG used only for the indications listed within this LCD.
Notice: This LCD imposes utilization guideline limitations. Despite Medicare’s allowing up to these maximums, each patient’s condition and response to treatment must medically warrant the number of services reported for payment. Medicare requires the medical necessity for each service reported to be clearly demonstrated in the patient’s medical record. Medicare expects that patients will not routinely require the maximum allowable number of services.