Condition

Indications

Autoimmune hemolytic anemia, refractory (D59.1)

IVIG may be considered medically necessary in persons with warm-type autoimmune hemolytic anemia that does not respond to corticosteroids or splenectomy, or those for whom the latter two treatments are contraindicated.

Bacterial infection in HIV-infected children (B20)

IVIG is considered medically necessary in children with HIV-infection who meet ANY of the following criteria:

1. Those with hypogammaglobulinemia, i.e., serum IgG concentration less than 250 mg/dL;
2. Those with recurrent serious bacterial infections, i.e., defined as two or more infections such as bacteremia, meningitis, or pneumonia in a 1-year period;
3. Those who fail to form antibodies to common antigens, such as measles, pneumococcal, and/or Haemophilus influenza type b vaccine;
4. Those living in areas where measles is highly prevalent and who have not developed an antibody response after two doses of measles, mumps, and rubella virus vaccine live;
5. Single dose for HIV-infected children who are exposed to measles;

HIV-infected children with chronic bronchiectasis that is sub optimally responsive to antimicrobial and pulmonary therapy.

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (G61.81), also known as Chronic Relapsing Polyneuropathy, including diabetes mellitus-CIDP and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) variant

Symmetric or focal neurologic deficits with slowly progressive or relapsing course over 2 months or longer with neurophysiological abnormalities.

Persons typically respond to IMMUNE GLOBULIN or plasma exchange within the first several weeks of treatment and may demonstrate sustained improvement for many weeks or months. Relapses may require periodic isolated treatments with a single dose of IMMUNE GLOBULIN or single plasma exchange. If a person responds successfully to infrequent booster treatments of either IMMUNE GLOBULIN or plasma exchange, it is reasonable to maintain this form of treatment rather than adding corticosteroids or other immunosuppressant’s.

Chronic Lymphocytic Leukemia (CLL) (C91.10-C91.12) in patients with hypogammaglobulinemia (D80.1)

IgG level less than 600mg/dL; and:

1. Severe bacterial infection within preceding 6 months or 2 or more bacterial infections in one year; or
2. Evidence of specific antibody deficiency.

Dermatomyositis, Polymyositis (M33.00-M33.99)(includes Juvenile)

     I.    Members presenting at least one item from the 1st criterion and four items from the 2nd through 9th criteria are said to have dermatomyositis. Patients presenting no items from the 1st criterion and at least four items from the 2nd through 9th criteria are said to have Polymyositis.

1)    Skin lesions

a)    Heliotrope rash (red purple edematous erythema on the upper palpebra)

b)    Gottron`s sign (red purple keratotic, atrophic erythema, or macules on the extensor surface of finger joints)

c)    Erythema on the extensor surface of extremity joints: slightly raised red purple erythema over elbows or knees

2)    Proximal muscle weakness (upper or lower extremity and trunk)

3)    Elevated serum CK (creatine kinase) or aldolase level

4)    Muscle pain on grasping or spontaneous pain

5)    Myogenic changes on EMG (short-duration, polyphasic motor unit potentials with spontaneous fibrillation potentials)

6)    Positive anti-Jo-1 (histadyl tRNA synthetase) antibody

7)    Non-destructive arthritis or arthralgias

8)    Systemic inflammatory signs (fever: more than 37° C at axilla, elevated serum CRP level or accelerated ESR of more than 20 mm/h by the Westergren method)

9)    Pathological findings compatible with inflammatory myositis (inflammatory infiltration of skeletal evidence of active regeneration may be seen

 AND

    II.    Member has severe active illness; and

  III.    Member is intolerant or refractory to 1st and 2nd line therapies:

1)    1st line therapy - Corticosteroids (e.g., prednisone);

2)    2nd line therapy - Immuno-suppressants (e.g., methotrexate, azithioprine, cyclophosphamide, and cyclosporine).

Fetal Alloimmume Thrombocytopenia (FAIT)

1. Previous pregnancy affected by FAIT and father homozygous for HPA-1a; and
2. At 20 weeks cordocentesis reveals fetal platelets < 100 x 109/L; or Symptomatic neonates with severe thrombocytopenia, who are at high risk of developing intracranial hemorrhage when washed irradiated maternal platelets are not available, have not been successful, have become intolerable, or are contraindicated.

Guillain Barre Syndrome (GBS) (G61.0) - a.k.a. Acute Infective Polyneuritis (includes Miller-Fisher syndrome [MFS], Pan autonomic polyneuropathy

1. Severe GBS with significant weakness such as inability to stand or walk without aid, respiratory or bulbar weakness, or Miller-Fisher syndrome (MFS); and
2. The disorder has been diagnosed during the first 2 weeks of the illness; and
3. IVIG is initiated within one month of symptom onset. Note: Based on the 2003 AAN guidelines, IVIG should usually be initiated within 2 weeks and no longer than 4 weeks of onset of neuropathic symptoms.

Hematopoietic Stem Cell Transplant (HSCT) or Bone Marrow Transplant (BMT) (Either T86.00 or T86.09, AND D80.1)

IMMUNE GLOBULIN is considered medically necessary for treatment of markedly hypogammaglobulinemic (IgG level less than 600 mg/dL) HSCT or BMT recipients with severe infections.

HIV-associated Thrombocytopenia (G69.59+B20)

- Adult

1. Significant bleeding or platelet count less than 20,000/ul; and
2. Failure of RhIG in Rh-positive patients.

HIV-associated Thrombocytopenia (G69.59+B20)

- Pediatric

Infants and children < 13 years of age whose IgG level is < 600 mg/dL; and

1. 2 or more bacterial infections in a 1-year period despite antibiotic chemoprophylaxis with TMP-SMZ or another active agent; or
2. Child has received 2 doses of measles vaccine and lives in a region with a high prevalence of measles; or
3. Member has HIV-associated thrombocytopenia despite antiretroviral therapy; or
4. Member has chronic bronchiectasis that is sub optimally responsive to antimicrobial and pulmonary therapy; or
5. T4 cell count is greater than or equal to 200/mm3

Idiopathic Thrombocytopenic Purpura (ITP) (D69.3)– Adult

Other causes of thrombocytopenia have been ruled out by history and peripheral smear; and

1. Unresponsive to corticosteroid therapy; and
   
   
2. Management of acute bleeding due to severe thrombocytopenia (platelet counts less than 30,000/ul); or
3. To increase platelet counts prior to invasive major surgical procedures (e.g., splenectomy), or To defer or avoid splenectomy; or
4. In members with severe thrombocytopenia (platelet counts less than 20,000/ul) considered to be at risk for intracerebral hemorrhage.

Idiopathic Thrombocytopenic Purpura (ITP) (D69.3)– Pediatric

Acute ITP:

1. IMMUNE GLOBULIN as initial therapy if platelet count < 20,000/ul, especially when member has emergency bleeding or is at risk for severe life-threatening bleeding; or
2. Persons with severe thrombocytopenia (platelet counts less than 20,000/ul) considered to be at risk for intracerebral hemorrhage.

Note: IMMUNE GLOBULIN not indicated if only mild manifestations of bleeding.

Chronic ITP:

In high risk persons when platelet count low or person symptomatic; and

1. Failure of other therapies or
2. Member is a high risk for post-splenectomy sepsis.

Hemolytic Disease of the Newborn (P55.0-P55.9)

Not responding to phototherapy to decrease the need for exchange transfusion. Physician discretion important in deciding.

Hyperimmunoglobulin E Syndrome (Jobs syndrome; Hyper IgE syndrome) (D82.4)

Recurrent staphylococcal abscesses and markedly elevated serum IgE with normal IgG, IgA, and IgM concentrations

Idiopathic Thrombocytopenic Purpura (ITP) (D69.3), Chronic Refractory

1. Age of 10 years or older; and
2. Duration of illness of greater than six months; and
3. No concurrent illness/disease explaining thrombocytopenia; and
4. Prior treatment with corticosteroids and splenectomy has failed or
5. Member is at high risk for post-splenectomy sepsis.

Immune Thrombocytopenic Purpura (ITP) in Pregnancy (D69.3+O99.111-O99.13)

1. Refractory to steroids with platelet counts < 10,000/ul in the third trimester; or
2. Platelet counts < 30,000/ul associated with bleeding before vaginal delivery or
3. C-section; or Pregnant women who have previously delivered infants with autoimmune thrombocytopenia; or
4. Pregnant women who have platelet counts less than 50,000/ul during the current pregnancy; or
5. Pregnant women with past history of splenectomy

Immunosuppressed Patients (D80.1, D80.8)

To prevent or modify recurrent bacterial or viral infections (e.g., CMV) in members with iatrogenic ally induced, or disease associated immunosuppression (IgG < 600 mg/dL) with one of the following:

1. Solid organ transplants or extensive surgery with immunosuppression (Note: In particular, IMMUNE GLOBULIN may be medically necessary in persons undergoing multiple courses of plasm apheresis as a treatment for allograft reception or for other indications; these persons may receive IMMUNE GLOBULIN at the completion of therapy if their IgG level is less than 600 mg/dL); or
2. Hematological malignancy; or
3. Extensive burns; or
4. Collagen-vascular disease.

Kawasaki disease (M30.3) (Mucocutaneous Lymph Node Syndrome [MCLS])

Diagnosis must be established - no specific lab test - diagnosis is established by meeting the following criteria:

1. Fever present for at least five days; and
2. Four of the following five conditions are met:

·         Mucous membrane changes such as a red tongue and dry fissured lips;

·         Swelling of the hands and feet;

·         Enlarged lymph nodes in the neck;

·         Diffuse red rash covering most of the body;

·         Redness of the eyes.

Lambert-Eaton Myasthenic Syndrome (LEMS) (G70.80, G70.81, G73.1)

No response to anticholinesterases and Diaminopyridine); and

1. Used as an alternative to plasma exchange if weakness is severe; or
2. When there is difficulty with venous access for plasmapheresis.

Myasthenia Gravis (G70.00-G70.01)

1. Treatment of acute myasthenic crisis with decompensation (respiratory failure, or disabling weakness requiring hospital admission); and
2. Other treatments have been unsuccessful or are contraindicated (e.g., azathioprine, cyclosporine, and cyclophosphamide).

Note: For management of myasthenic crises, IMMUNE GLOBULIN is administered over 2 to 5 days. Use of IMMUNE GLOBULIN as maintenance therapy is considered experimental and investigational.

Multifocal Motor Neuropathy with Conduction Block (G60.3, G60.8, G60.9, G61.89, G62.89)

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 IMMUNE GLOBULIN treatment).

Multiple Myeloma (MM) (C90.00-C90.02)

1. “Plateau Phase” MM (> 3 months since diagnosis); and
2. IgG level < 600mg/dL; and
3. 2 or more significant infections in last year or
4. a single life-threatening infection; or
5. Evidence of specific antibody deficiency.

Multiple Sclerosis (MS) (G35) - Relapsing-remitting
(not primary or secondary progressive MS)

1. Severe manifestations of relapsing-remitting MS (not primary or secondary progressive MS); and
2. Standard approaches (i.e., interferons - Betaseron, Avonex, and Rebif) have failed, become intolerable, or are contraindicated.

Parvovirus B19 Infection, Chronic, with Severe Anemia (Pure Red Cell Aplasia) (B34.3+D60.0)

Severe, refractory anemia with documented parvovirus B19 viremia.

Pediatric acute-onset neuropsychiatric syndrome (PANS) and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS)

MUST MEET ALL

1.    Pediatric patient with a diagnosis of PANS or PANDAS AND

2.    Patients PCP, in consultation with an AR licensed pediatric psychiatrist and an AR licensed physician who practices in at least one (1) pediatric subspecialty (including neurology, rheumatology or infectious disease) who has treated the patient all determine and agree that the treatment is necessary and follow a patient-specific treatment plan. PCP may consult with the childhood post-infectious autoimmne encephalopathy center of excellence for treatment plan

3.    Member has had treatment with two or more less-intensive therapies (e.g.limited course of non-steroidal anti-inflammatory drugs, corticosteroids, selective serotonin reuptake inhibitors, behavioral therapy, short course antibiotic therapy), and these therapies were not effective;

4.    Up to 3 monthly immunomodulatory courses of IVIG therapy may be recommended for treatment of PANDAS and PANS. Reevaluation for additional treatment at 3 months by the pediatric sub-specialist will be required for continued therapy. Reevaluation must include objective clinical testing by a specialist trained in structured and/or semi-structured interview assessments such as a neuropsychologist which must be performed both pre-treatment and post-treatment to demonstrate significant clinical improvement.

Pemphigus Vulgaris (Autoimmune Mucocutaneous Blistering Diseases) - includes Pemphigus Foliaceus, Bullous Pemphigoid, Mucous Membrane Pemphigoid (a.k.a. Cicatricial Pemphigoid), and Epidermolysis bullosa aquisita (L10.0-L10.9, L12.0-L12.9)

1. The diagnosis has been proven by biopsy and confirmed by pathology report; and
2. The condition is rapidly progressing, extensive or debilitating; and
3. Corticosteroids, immuno-suppressive agents have failed or
4. The member has experienced significant complications from standard treatment, such as diabetes or steroid-induced osteoporosis.

Post-transfusion purpura (PTP) (D69.51)

1. Decreased platelets (usually < 10,000/ul); and
2. 2 - 14 days post transfusion with bleeding

Primary Humoral Immunodeficiencies

1. Selective IgA Immunodeficiency (D80.2)
2. Selective IgM Immunodeficiency (D80.4)
3. Congenital hypogammaglobulinemia (D80.0)
4. Immunodeficiency with near/normal IgM (absent IgG, IgA) - a.k.a. Hyper IgM syndrome (D80.5, D80.6)
5. Other deficiency of humoral immunity (D80.8, D80.9)

Severe combined immunodeficiency disorders (D81.0-D81.2) (e.g., X-SCID, jak3, ZAP70, ADA, PNP, RAG defects, Ataxia Telangiectasia, DiGeorge syndrome, common variable immunodeficiency

1. Hypogammaglobulinemia (Total IgG < 400 mg/dL). AND
2. 2 or more bacterial infections per year due to persistent and significant reduction in total IgG or IgG subclasses; OR
3. dL).
4. Documented lack of antibody function as demonstrated by lack of response to immunizations (e.g. pneumovax)

Note: The use of IMMUNE GLOBULIN may not be beneficial in secondary immuno-deficiency states - Correction of the underlying condition is the preferred approach.

Selective IgG Subclass Deficiency (D80.3)

1. Member has unexplained recurrent or persistent severe bacterial infections; and
2. Infections fail to respond adequately to conservative measures, including meticulous hygiene and prophylactic antibiotics; and
3. Member has demonstrated an inability to mount an adequate response to protein and polysaccharide antigens, as determined by the following criteria;
   1.  Member has documented inability to mount an antibody response to protein antigens: Serum antibody titers to tetanus and/or diphtheria should be obtained prior to immunization with diphtheria and/or tetanus vaccine and two to four weeks after immunization. An inadequate response is defined as less than a fourfold rise in antibody titer; and
   2. Member has documented inability to mount an adequate antibody response to polysaccharide antigens. Serum antibody titers to pneumococcus should be measured prior to immunization and three to six weeks after immunization with polyvalent pneumococcal polysaccharide vaccine (e.g., Pneumovax). An inadequate response is defined as less than a 2-fold rise in titer over baseline in at least one serotype tested.
      Note: Response to polysaccharide antigens is not reliable in children less than 2 years of age.
4. IMMUNE GLOBULIN should be discontinued and the medical necessity of IMMUNE GLOBULIN should be reevaluated 1 year after initiating therapy and every two years thereafter by reassessing immune response to protein and polysaccharide antigens.

Systemic Lupus Erythematosus (M32.1-M32.9)

Members with severe active SLE for whom first- and second-line therapies have been unsuccessful, have become intolerable, or are contraindicated.

Note: Standard first-line therapy of active SLE include non-steroidal anti-inflammatory drugs, followed by low-dose corticosteroids and antimalarial compounds. Second-line therapeutic alternatives are the cytotoxic agent’s methotrexate, azathioprine, or cyclophosphamide.

Toxic shock syndrome (A48.3) or toxic necrotizing fasciitis due to group A streptococcus (M72.6+B95.0)

IMMUNE GLOBULIN is considered medically necessary in persons who are sufficiently ill to require critical care unit support and have documented presence of fasciitis and microbiological data consistent with invasive streptococcal infection (culture or Gram stain).

90281

Immune globulin, human, for intramuscular use

90283

Immune globulin, human, for IV use

96360

Intravenous infusion, hydration; initial, 31 min to 1 hr.

96361

Intravenous infusion, hydration; ea. addt’l hr.

96365

Intravenous infusion, for therapy, prophylaxis, or diagnosis; initial, up to 1 hr.

96366

Intravenous infusion, for therapy, prophylaxis, or diagnosis; ea. addt’l  hr.

96367

Intravenous infusion, for therapy, prophylaxis, or diagnosis; addt’l sequential infusion of a new drug/substance, up to 1 hr.

96368

Intravenous infusion, for therapy, prophylaxis, or diagnosis; concurrent infusion

96413

Chemotherapy administration, IV infusion technique; up to 1 hr., single or initial substance/drug

96415

Chemotherapy administration, IV infusion technique; ea. addt’l hr.

96416

Chemotherapy administration, IV infusion technique; initiation of prolonged chemo infusion (more than 8 hrs.), requiring use of a portable or implantable pump

96417

Chemotherapy administration, IV infusion technique; ea. addt’l sequential infusion (diff substance/drug), up to 1 hr.

J1459

J1551

J1555

Injection, immune globulin (Privigen), IV, nonlyophilized, 500 mg

Injection, immune globulin (Cutaquig), 100mg

Injection, immune globulin (Cuvitru), 100mg

J1554   

Injection, immune globulin (Asceniv), 500 mg (new code eff 04/01/2021)

J1556

Injection, immune globulin (Bivigam), 500 mg

J1557

Injection, immune globulin (Gammaplex), IV, nonlyophilized, 500 mg

J1559

Injection, immune globulin (Hizentra), 100 mg

J1561

Injection, immune globulin (Gamunex/Gamunex-C/Gamma ked), nonlyophilized, 500 mg

J1562

Injection, immune globulin (Vivaglobin), 100 mg

J1566

Injection, immune globulin, IV, lyophilized, NOS, 500 mg

J1568

Injection, immune globulin, (Octagam), IV, nonlyophilized, 500 mg

J1569

Injection, immune globulin, (Gamma Gard liquid), nonlyophilized, 500 mg

J1575

Injection, immune globulin/hyaluronidase, 100mg immune globulin (Hyqvia)

J1599

Injection, immune globulin, IV, nonlyophilized, NOS, 500 mg

J1558

Injection, immune globulin (xembify), 100mg

C9072

Injection, immune globulin (Asceniv), 500 mg (new code eff 01/01/2021) (deleted and replaced by code J1554 eff 04/01/2021)