Specialty Pharmacy Clinical Policy Bulletins Aetna Non-Medicare Prescription Drug Plan
Subject: IVIG SGM 2042-A P2025
Drug
Intravenous Immune Globulin (IVIG)
Alyglo
Asceniv
Bivigam
Flebogamma DIF
Gammagard Liquid
Gammagard Liquid ERC
Gammagard S/D
Gammaked
Gammaplex
Gamunex-C
Octagam
Panzyga
Privigen
Qivigy
Yimmugo
Policy:
Indications
The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy.
Clinical records describing standard treatments tried and failed
Toxic shock syndrome or toxic necrotizing fasciitis due to group A streptococcus
Documented presence of fasciitis (toxic necrotizing fasciitis due to group A streptococcus only)
Microbiological data (culture or Gram stain)
Coverage Criteria
Primary Immunodeficiency1-12,14-16,65,66,67,70,71
Initial authorization of 6 months may be granted for members with any of the following diagnoses:
Severe combined immunodeficiency (SCID) or congenital agammaglobulinemia (e.g., X-linked or autosomal recessive agammaglobulinemia):30-32,35,44
Diagnosis confirmed by genetic or molecular testing, or
Pretreatment IgG level < 200 mg/dL, or
Absence or very low number of T cells (CD3 T cells < 300/microliter) or the presence of maternal T cells in the circulation (SCID only)
Wiskott-Aldrich syndrome, DiGeorge syndrome, or ataxia-telangiectasia (or other non-SCID combined immunodeficiency):30-32
Diagnosis confirmed by genetic or molecular testing (if applicable), and
History of recurrent bacterial infections (e.g., pneumonia, otitis media, sinusitis, sepsis, gastrointestinal), and
Impaired antibody response to pneumococcal polysaccharide vaccine (see Appendix A)
Common variable immunodeficiency (CVID):30-34
Age 2 years or older, and
Other causes of immune deficiency have been excluded (e.g., drug induced, genetic disorders, infectious diseases such as HIV, malignancy), and
Pretreatment IgG level < 500 mg/dL or ≥ 2 SD below the mean for age, and
History of recurrent bacterial infections, and
Impaired antibody response to pneumococcal polysaccharide vaccine (see Appendix A)
Hypogammaglobulinemia (unspecified), IgG subclass deficiency, selective IgA deficiency, selective IgM deficiency, or specific antibody deficiency:30-35
History of recurrent bacterial infections, and
Impaired antibody response to pneumococcal polysaccharide vaccine (see Appendix A), and
Any of the following pre-treatment laboratory findings:
Hypogammaglobulinemia: IgG < 500 mg/dL or ≥ 2 SD below the mean for age
Selective IgA deficiency: IgA level < 7 mg/dL with normal IgG and IgM levels
Selective IgM deficiency: IgM level < 30 mg/dL with normal IgG and IgA levels
IgG subclass deficiency: IgG1, IgG2, or IgG3 ≥ 2 SD below mean for age assessed on at least 2 occasions; normal IgG (total) and IgM levels, normal/low IgA levels
Specific antibody deficiency: normal IgG, IgA and IgM levels
Other predominant antibody deficiency disorders must meet all of the following:
History of recurrent bacterial infections, and
Impaired antibody response to pneumococcal polysaccharide vaccine (see Appendix A), and
A pre-treatment laboratory finding of hypogammaglobulinemia: IgG < 500 mg/dL or ≥ 2 SD below the mean for age
Other combined immunodeficiency must meet all of the following:
Diagnosis confirmed by genetic or molecular testing (if applicable), and
History of recurrent bacterial infections (e.g., pneumonia, otitis media, sinusitis, sepsis, gastrointestinal), and
Impaired antibody response to pneumococcal polysaccharide vaccine (see Appendix A)
Re-authorization of 12 months may be granted when the following criteria are met:
A reduction in the frequency of bacterial infections has been demonstrated since initiation of IG therapy, AND
IgG trough levels are monitored at least yearly and maintained at or above the lower range of normal for age (when applicable for indication),35 OR
The prescriber will re-evaluate the dose of IG and consider a dose adjustment (when appropriate).
Myasthenia Gravis23-25
Authorization of 1 month may be granted to members who are prescribed IG for worsening weakness, acute exacerbation, or in preparation for surgery.
Worsening weakness includes an increase in any of the following symptoms: diplopia, ptosis, blurred vision, difficulty speaking (dysarthria), difficulty swallowing (dysphagia), difficulty chewing, impaired respiratory status, fatigue, and limb weakness. Acute exacerbations include more severe swallowing difficulties and/or respiratory failure
Pre-operative management (e.g., prior to thymectomy)
Authorization of 6 months may be granted to members with refractory myasthenia gravis who have tried and failed 2 or more standard therapies (e.g., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, rituximab).
Initial authorization of 3 months may be granted when the following criteria are met:
Disease course is progressive or relapsing/remitting for 2 months or longer
Moderate to severe functional disability
The diagnosis was confirmed by electrodiagnostic studies
Re-authorization of 6 months may be granted when the following criteria are met:
Significant improvement in disability and maintenance of improvement since initiation of IG therapy
IG is being used at the lowest effective dose and frequency
Dermatomyositis or Polymyositis13,23-25,41
Initial authorization of 3 months may be granted when the following criteria are met:
Member has at least 4 of the following:
Proximal muscle weakness (upper or lower extremity and trunk)
Elevated serum creatine kinase (CK) or aldolase level
Muscle pain on grasping or spontaneous pain
Myogenic changes on EMG (short-duration, polyphasic motor unit potentials with spontaneous fibrillation potentials)
Positive for anti-synthetase antibodies (e.g., anti-Jo-1, also called histadyl tRNA synthetase)
Non-destructive arthritis or arthralgias
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)
Pathological findings compatible with inflammatory myositis (inflammatory infiltration of skeletal evidence of active regeneration may be seen), and
Standard first-line treatments (corticosteroids) and second-line treatments (immunosuppressants) have been tried but were unsuccessful or not tolerated, or
Member is unable to receive standard first-line and second-line therapy because of a contraindication or other clinical reason.
Re-authorization of 6 months may be granted when the following criterion is met:
Significant improvement in disability and maintenance of improvement since initiation of IG therapy
Newly diagnosed ITP (diagnosed within the past 3 months) or initial therapy: authorization of 1 month may be granted when the following criteria are met:42,43
Children (< 18 years of age)
Significant bleeding symptoms (mucosal bleeding or other moderate/severe bleeding) or
High risk for bleeding (see Appendix B), or
Rapid increase in platelets is required (e.g., surgery or procedure)
Adults (≥ 18 years of age)
Platelet count < 30,000/mcL, or
Platelet count < 50,000/mcL and significant bleeding symptoms, high risk for bleeding or rapid increase in platelets is required, and
Corticosteroid therapy is contraindicated and IG will be used alone or IG will be used in combination with corticosteroid therapy
Chronic/persistent ITP (≥ 3 months from diagnosis) or ITP unresponsive to first-line therapy: authorization of 6 months may be granted when the following criteria are met:42,43
Platelet count < 30,000/mcL, or
Platelet count < 50,000/mcL and significant bleeding symptoms, high risk for bleeding or rapid increase in platelets is required, and
Relapse after previous response to IG or inadequate response/intolerance/contraindication to corticosteroid or anti-D therapy
Adults with refractory ITP after splenectomy: authorization of 6 months may be granted when either of the following criteria is met:42
Platelet count < 30,000/mcL, or
Significant bleeding symptoms
ITP in pregnant women: authorization through delivery may be granted to pregnant women with ITP.42
The member’s risk factor(s) for bleeding (see Appendix B) or reason requiring a rapid increase in platelets must be provided.
B-cell Chronic Lymphocytic Leukemia (CLL)37
Initial authorization of 6 months may be granted when all of the following criteria are met:
IG is prescribed for prophylaxis of bacterial infections.
Member has a history of recurrent sinopulmonary infections requiring intravenous antibiotics or hospitalization.
Member has a pretreatment serum IgG level <500 mg/dL.
Re-authorization of 6 months may be granted when a reduction in the frequency of bacterial infections has been demonstrated since initiation of IG therapy.
Prophylaxis of Bacterial Infections in HIV-Infected Pediatric Patients20
Initial authorization of up to 6 months may be granted to pediatric members with HIV infection when any of the following criteria are met:
IG is prescribed for primary prophylaxis of bacterial infections and pretreatment serum IgG < 400 mg/dL, or
IG is prescribed for secondary prophylaxis of bacterial infections for members with a history of recurrent bacterial infections (> 2 serious bacterial infections in a 1-year period), or
Member has failed to form antibodies to common antigens, such as measles, pneumococcal, and/or Haemophilus influenzae type b vaccine, or
Member lives in an area where measles is highly prevalent and who have not developed an antibody response after two doses of measles, mumps, and rubella virus vaccine live, or
Member has been exposed to measles and request is for a single dose, or
Member has chronic bronchiectasis that is suboptimally responsive to antimicrobial and pulmonary therapy
Re-authorization of 6 months may be granted when a reduction in the frequency of bacterial infections has been demonstrated since initiation of IG therapy.
Bone Marrow Transplant/Hemopoietic Stem Cell Transplant (BMT/HSCT)17,22
Initial authorization of 6 months may be granted to members who are BMT/HSCT recipients when the following criteria are met:
Therapy will be used to prevent the risk of acute graft-versus-host disease, associated interstitial pneumonia (infectious or idiopathic), septicemia, and other infections (e.g., cytomegalovirus infections [CMV], recurrent bacterial infection).
Either of the following:
IG is requested within the first 100 days post-transplant.
Member has a pretreatment serum IgG < 400 mg/dL.
Re-authorization of 6 months may be granted when a reduction in the frequency of bacterial infections has been demonstrated since initiation of IG therapy.
Multifocal Motor Neuropathy (MMN)39,40
Initial authorization of 3 months may be granted when the following criteria are met:
Member experienced progressive, multifocal, asymmetrical weakness without objective sensory loss in 2 or more nerves for at least 1 month
The diagnosis was confirmed by electrodiagnostic studies
Re-authorization of 6 months may be granted when significant improvement in disability and maintenance of improvement have occurred since initiation of IG therapy
Guillain-Barre Syndrome (GBS)23-25
Authorization of 1 month total may be granted for GBS when the following criteria are met:
Member has severe disease with significant weakness (e.g., inability to stand or walk without aid, respiratory weakness)
Onset of neurologic symptoms occurred less than 4 weeks from the anticipated start of therapy
Lambert-Eaton Myasthenic Syndrome (LEMS)23,24,26
Initial authorization of 6 months may be granted for LEMS when the following criteria are met:
Diagnosis has been confirmed by either of the following:
Neurophysiology studies (e.g., electromyography)
A positive anti- P/Q type voltage-gated calcium channel antibody test
Anticholinesterases (e.g., pyridostigmine) and amifampridine (e.g., 3,4-diaminopyridine phosphate, Firdapse) have been tried but were unsuccessful or not tolerated
Weakness is severe or there is difficulty with venous access for plasmapheresis
Re-authorization of 6 months may be granted when member is responding to therapy (i.e., there is stability or improvement in symptoms relative to the natural course of LEMS).
Kawasaki Syndrome7,8
Authorization of 1 month may be granted for pediatric members with Kawasaki syndrome.
Authorization of 6 months may be granted for treatment of F/NAIT.
Parvovirus B19-induced Pure Red Cell Aplasia (PRCA)29
Authorization of 6 months may be granted for severe, refractory anemia associated with bone marrow suppression, with parvovirus B19 viremia.
Stiff-person Syndrome18,23-25
Authorization of 6 months may be granted for stiff-person syndrome when the following criteria are met:
Diagnosis has been confirmed by anti-glutamic acid decarboxylase (GAD) antibody testing
Member had an inadequate response to first-line treatment (benzodiazepines and/or baclofen)
Management of Immune Checkpoint Inhibitor-Related Toxicities45
Authorization of 1 month may be granted for management of immune checkpoint-inhibitor toxicities when all of the following criteria are met:
Member has experienced a moderate or severe adverse event to a PD-1 or PD-L1 inhibitor (e.g., pembrolizumab, nivolumab, atezolizumab, avelumab, durvalumab)
The offending medication has been held or discontinued
Member experienced one or more of the following adverse events: myocarditis, bullous dermatitis, Stevens-Johnson syndrome, toxic epidermal necrolysis, pneumonitis, myasthenia gravis, peripheral neuropathy, encephalitis, transverse myelitis, severe inflammatory arthritis, Guillain-Barre syndrome, or steroid-refractory myalgias or myositis
Acquired Red Cell Aplasia29
Authorization of 6 months may be granted for acquired red cell aplasia.
Acute Disseminated Encephalomyelitis17
Authorization of 1 month may be granted for acute disseminated encephalomyelitis in members who have had an insufficient response or a contraindication to intravenous corticosteroid treatment.
Autoimmune Mucocutaneous Blistering Disease47
Authorization of 6 months may be granted for autoimmune mucocutaneous blistering disease (includes pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, and epidermolysis bullosa acquisita) when the following criteria are met:
Diagnosis has been proven by biopsy and confirmed by pathology report, and
Condition is rapidly progressing, extensive or debilitating, and
Member has failed or experienced significant complications (e.g., diabetes, steroid-induced osteoporosis) from standard treatment (corticosteroids, immunosuppressive agents).
Autoimmune Hemolytic Anemia17
Authorization of 6 months may be granted for warm-type autoimmune hemolytic anemia in members who do not respond or have a contraindication to corticosteroids or splenectomy.
Autoimmune Neutropenia29
Authorization of 6 months may be granted for autoimmune neutropenia where treatment with G-CSF (granulocyte colony stimulating factor) is not appropriate.
Birdshot Retinochoroidopathy17
Authorization of 6 months may be granted for birdshot (vitiliginous) retinochoroidopathy that is not responsive to immunosuppressives (e.g., corticosteroids, cyclosporine).
BK Virus Associated Nephropathy48
Authorization of 6 months may be granted for BK virus associated nephropathy.
Churg-Strauss Syndrome49
Authorization of 6 months may be granted for severe, active Churg-Strauss syndrome as adjunctive therapy for members who have experienced failure, intolerance, or are contraindicated to other interventions.
Enteroviral Meningoencephalitis50
Authorization of 6 months may be granted for severe cases of enteroviral meningoencephalitis.
Hematophagocytic Lymphohistiocytosis (HLH) or Macrophage Activation Syndrome (MAS)51
Authorization of 6 months may be granted for treatment of hypogammaglobulinemia in HLH or MAS when total IgG is less than 400 mg/dL or two standard deviations below the mean for age.
Hemolytic Disease of Newborn29
Authorization of 6 months may be granted for isoimmune hemolytic disease in neonates.
HIV-associated Thrombocytopenia29
Authorization of 6 months may be granted for HIV-associated thrombocytopenia when the following criteria are met:
Pediatric members with IgG < 400 mg/dL and one of the following:
2 or more bacterial infections in a 1-year period despite antibiotic chemoprophylaxis with TMP-SMZ or another active agent, or
Received 2 doses of measles vaccine and lives in a region with a high prevalence or measles, or
HIV-associated thrombocytopenia despite anti-retroviral therapy, or
Chronic bronchiectasis that is suboptimally responsive to antimicrobial and pulmonary therapy, or
T4 cell count ≥ 200/mm3
Adult members with significant bleeding, platelet count < 20,000/mcL, and failure of RhIG in Rh-positive patients
Hyperimmunoglobulinemia E Syndrome52
Authorization of 6 months may be granted to treat severe eczema in hyperimmunoglobulinemia E syndrome.
Hypogammaglobulinemia from CAR-T therapy45,53,54,64
Authorization of 6 months may be granted for members with IgG < 400 mg/dL receiving treatment with CAR-T therapy (including but not limited to idecabtagene vicleucel [Abecma], tisagenlecleucel [Kymriah], or axicabtagene ciloleucel [Yescarta]).
Multiple Myeloma17,29,55
Authorization of 6 months may be granted for multiple myeloma in members who have recurrent, serious infections despite the use of prophylactic antibiotics.
Neonatal Hemochromatosis56
Authorization of 6 months may be granted for prophylaxis in members who are pregnant with a history of pregnancy ending in documented neonatal hemochromatosis.
Opsoclonus-myoclonus57,58
Authorization of 6 months may be granted for treatment of either of the following:
Paraneoplastic opsoclonus-myoclonus-ataxia associated with neuroblastoma
Refractory opsoclonus-myoclonus, as last-resort treatment
Pediatric Acute-onset Neuropsychiatric Syndrome (PANS)/Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infections (PANDAS) (See Appendix C)69
Initial Authorization of 6 months may be granted when the following criteria are met:
Child meets PANS Research Consortium Diagnostic Criteria for PANS/PANDAS:
Documentation of abrupt, dramatic onset (within less than one month) of obsessive-compulsive disorder or severely restricted food intake; and
Documentation of concurrent presence of additional neuropsychiatric symptoms, with similarly severe and acute onset, from at least two of the following seven categories:
Deterioration in school performance (related to attention deficit/hyperactivity disorder (ADHD)-like symptoms, memory deficits, cognitive changes)
Sensory or motor abnormalities
Somatic signs and symptoms, including sleep disturbances, enuresis, or urinary frequency; and
Onset of symptoms occurs between 3 years of age and puberty; and
Documentation that other causes of symptoms have been ruled out.
Child has tried and failed treatment with systemic corticosteroids.
Documented objective assessment of baseline symptoms has been submitted (e.g., Children's Yale-Brown Obsessive-Compulsive Scale [CY-BOCS], Clinical Global Impression of Severity [CGIS], Parent-Rated Pediatric Acute Neuropsychiatric Symptom Scale [PANS Scale]).
Re-authorization of 6 months maybe granted when documentation of objective clinical response to therapy has been submitted (e.g., Children's Yale-Brown Obsessive-Compulsive Scale [CY-BOCS], Clinical Global Impression of Severity [CGIS], Parent-Rated Pediatric Acute Neuropsychiatric Symptom Scale [PANS Scale]).
Post-transfusion Purpura29
Authorization of 1 month may be granted for post-transfusion purpura
Rasmussen Encephalitis17
Authorization of 6 months may be granted for Rasmussen encephalitis in members whose symptoms do not improve with anti-epileptic drugs and corticosteroids.
Renal Transplantation59
Authorization of 6 months may be granted for a member undergoing renal transplantation from a live donor with ABO incompatibility or positive cross match.
Retinocochleocerebral vasculopathy, Central nervous system-predominant68
Authorization of 6 months may be granted for treatment of central nervous system (CNS)-predominant Retinocochleocerebral vasculopathy (Susac syndrome (SuS)) when used as an adjunctive therapy to corticosteroid and other treatment options.
Secondary Immunosuppression Associated with Major Surgery, Hematological Malignancy, Major Burns, and Collagen-Vascular Diseases17,55
Authorization of 6 months may be granted to prevent or modify recurrent bacterial or viral infections in members with secondary immunosuppression (IgG < 400 mg/dL) associated with major surgery, hematological malignancy, extensive burns, or collagen-vascular disease.
Solid Organ Transplantation17,60,61
Authorization of 6 months may be granted for solid organ transplantation for allosensitized members.
Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome17
Authorization of 1 month may be granted for severe cases of toxic epidermal necrolysis or Stevens-Johnson syndrome.
Toxic Shock Syndrome17,62
Authorization of 1 month may be granted for staphylococcal or streptococcal toxic shock syndrome when the infection is refractory to several hours of aggressive therapy, an undrainable focus is present, or the member has persistent oliguria with pulmonary edema.
Systemic Lupus Erythematosus17,63
Authorization of 6 months may be granted for severe, active SLE in members who have experienced inadequate response, intolerance or have a contraindication to first and second line therapies (e.g., hydroxychloroquine, glucocorticoids, anifrolumab, rituximab).
Measles (Rubeola) Prophylaxis18
Authorization of 1 month may be granted for postexposure prophylaxis to prevent or modify symptoms of measles (rubeola) in susceptible members exposed to the disease less than 6 days previously.
Tetanus Treatment and Prophylaxis18
Authorization of 1 month may be granted for treatment or postexposure prophylaxis of tetanus as an alternative when tetanus immune globulin (TIG) is unavailable.
Varicella Prophylaxis18
Authorization of 1 month may be granted for postexposure prophylaxis of varicella in susceptible individuals when varicella-zoster immune globulin (VZIG) is unavailable.
Toxic Necrotizing Fasciitis Due to Group A Streptococcus17
Authorization of 1 month may be granted for members with fasciitis due to invasive streptococcal infection.
Continuation of Therapy
Authorization may be granted for continuation of therapy when either the following criteria is met:
For conditions with reauthorization criteria listed under the coverage criteria section: Members who are currently receiving IG therapy must meet the applicable reauthorization criteria for the member’s condition.
For all other conditions, all members (including new members) must meet the requirements in the coverage criteria.
Appendix
Appendix A: Impaired Antibody Response to Pneumococcal Polysaccharide Vaccine31,32
Age 2 years and older: impaired antibody response demonstrated to vaccination with a pneumococcal polysaccharide vaccine
Not established for children less than 2 years of age
Excludes the therapy initiated in the hospital setting
Appendix B: Examples of Risk Factors for Bleeding (not all inclusive)42,43
Undergoing a medical or dental procedure where blood loss is anticipated
Profession or lifestyle predisposes patient to trauma (e.g., construction worker, fireman, professional athlete)
Appendix C69
Members suspected of having PANS/PANDAS should be evaluated with the following tests, as indicated:
Complete blood cell counts with manual differential
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
If there are elevated inflammatory markers, fatigue, rashes, or joint pain, antinuclear antibody (ANA) or fluorescent antinuclear antibody (FANA) should be obtained (if ANA is elevated, the member should be evaluated for lupus)
Comprehensive metabolic panel
If liver function tests are abnormal or Kayser–Fleisher rings are present, the member should be evaluated for Wilson’s disease with ceruloplasmin and 24 urine copper tests.
Throat culture, anti-streptolysin O (ASO) and anti-DNAse B
Urinalysis (to assess hydration) and to rule out inflammation for children with urinary complaints
If urinalysis reveals pyuria, clean-catch urine culture
Antiphospholipid antibody work up if the member has chorea, petechiae, migraines, stroke, thrombosis, thrombocytopenia, or levido rash. Workup should include:
anticardiolipin antibody,
dilute Russell’s viper venom time (dRVVT),
b 2-glycoprotein I antibodies.
Members should be evaluated for other causes of symptoms, including:
Autism/Autistic Spectrum Disorder
Developmental Delay/Mental Retardation
Cerebral palsy
Genetic conditions and syndromes (such as Down Syndrome, Fragile X, Rett Syndrome, etc.)
Cerebral malformation syndromes
Inborn errors of metabolism
CNS infections
Toxic cerebral insults (such as kernicterus, chemo-radiation, etc.)
Obsessive compulsive disorder
Anorexia nervosa
Avoidant/restrictive food intake disorder (ARFID)
Tourette syndrome
Transient tic disorder
Bipolar disorder
Sydenham chorea
Autoimmune encephalitis
Systemic autoimmune disease
Wilson’s disease
Place of Service:
Outpatient
The above policy is based on the following references:
Asceniv [package insert]. Boca Raton, FL: ADMA Biologics; April 2019.
Bivigam [package insert]. Boca Raton, FL: ADMA Biologics; March 2024.
Gammagard S/D IgA less than 1 mcg/mL [package insert]. Westlake Village, CA: Baxalta US Inc.; September 2016.
Gammaked [package insert]. Research Triangle Park, NC: Grifols Therapeutics LLC; January 2020.
Gammaplex 5% [package insert]. Borehamwood, United Kingdom: Bio Products Laboratory; May 2024.
Gammaplex 10% [package insert]. Borehamwood, United Kingdom: Bio Products Laboratory; May 2024.
Gamunex-C [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc.; January 2020.
Octagam 10% [package insert]. New York, NY: Pfizer Labs; January 2024.
Octagam 5% [package insert]. New York, NY: Pfizer Labs; January 2024.
Panzyga [package insert]. New York, NY: Pfizer Labs; April 2025.
Privigen [package insert]. Kankakee, IL: CSL Behring LLC; March 2022.
DRUGDEX® System (electronic version). Truven Health Analytics, Ann Arbor, MI. Available at http://www.micromedexsolutions.com [available with subscription]. Accessed May 6, 2025.
AHFS Drug Information. http://online.lexi.com/lco. Accessed May 6, 2025.
Perez EE, Orange JS, Bonilla F, et al. Update on the use of immunoglobulin in human disease: a review of evidence by Work Group Report of the American Academy of Allergy, Asthma, and Immunology. J Allergy Clin Immunol. 2017;139:S1-46.
Clinical Consult. CVS Caremark Clinical Programs Review: Focus on immunology programs. October 2007.
Tomblyn M, Chiller T, Einsele H, et al. Guidelines for preventing infectious complications among hematopoietic cell transplant recipients: a global perspective. Biol Blood Marrow Transplant. 2009;15(10):1143-1238.
Feasby T, Banwell B, Bernstead T, et al. Guidelines on the use of intravenous immune globulin for neurologic conditions. Transfus Med Rev. 2007;21(2):S57-S107.
Donofrio PD, Berger A, Brannagan TH 3rd, et al. Consensus statement: the use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the AANEM ad hoc committee. Muscle Nerve. 2009;40(5):890-900.
Elovaara I, Apostolski S, van Doorn P, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol. 2008;15(9):893-908.
Patwa HS, Chaudhry V, Katzberg H, et al. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2012;78(13);1009-1015.
Clinical Consult. CVS Caremark Clinical Programs Review: Focus on immunology programs. November 2009.
Clinical Consult. CVS Caremark Clinical Programs Review: Focus on immunology programs. October 2008.
Anderson D, Kaiser A, Blanchette V, et al. Guidelines on the use of intravenous immune globulin for hematologic conditions. Transfus Med Rev. 2007;21(2):S9-S56.
Picard C, Al-Herz W, Bousfiha A, et al. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. J Clin Immunol. 2015; 35(8):696-726.
Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186-205.e1-78.
Orange JS, Ballow M, Stiehm ER, et al. Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest section of the American Academy of Allergy, Asthma and Immunology. J Allergy Clin Immunol. 2012;130:S1-S24.
Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R. New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin. Clin Exp Immunol. 2013;174(2):203-11.
European Society for Immunodeficiencies. Diagnostic criteria for PID. http://esid.org/Working-Parties/Clinical/Resources/Diagnostic-criteria-for-PID2. Accessed May 7, 2025.
Van den Bergh PY, Hadden RD, van Doorn PA, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - second revision. Eur J Neurol. 2021;28(11):3556-3583.
Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Societies guideline on management of multifocal motor neuropathy. J Peripher Nerv Syst. 2010;15:295-301.
Olney RK, Lewis RA, Putnam TD, Campellone JV. Consensus criteria for the diagnosis of multifocal motor neuropathy. Muscle Nerve. 2003;27:117-121.
Dalakas M. Inflammatory muscle diseases. N Engl J Med. 2015;372(18):1734-1747.
Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019;3(23):3829-3866.
Provan D, Arnold DM, Bussel JB, et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Adv2019;3(22): 3780–3817.
Shearer WT, Dunn E, Notarangelo LD, et al. Establishing diagnostic criteria for severe combined immunodeficiency disease (SCID), leaky SCID, and Omenn syndrome: the Primary Immune Deficiency Treatment Consortium experience. J Allergy Clin Immunol. 2014;133(4):1092.
[HIVPeds]Working Group on Antiretroviral Therapy of the National Pediatric HIV Resource Center. Antiretroviral therapy and medical management of pediatric HIV infection. Pediatrics. 1998;102;1005-1063.
Center for Medicare and Medicaid Services (CMS). Intravenous immune globulin for autoimmune mucocutaneous blistering diseases. Decision Memorandum. CPG-00109N. Baltimore, MD: CMS; January 22, 2002.
Sawinski D, Goral S. BK virus infection: An update on diagnosis and treatment. Nephrol Dial Transplant. 2015;30(2):209-217.
Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss) (EGPA)Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015;26(7):545-553.
McKinney RE, Katz SL, Wilfert CM. Chronic enteroviral meningoencephalitis in agammaglobulinemic patients. Rev Infect Dis. 1987;9(2):334-56.
Sen, E.S., Clarke, SL, Ramanan, A.V. Macrophage Activation Syndrome. Indian J Pediatr. 2016;83(3): 248-53.
Kimata H. High-dose intravenous gammaglobulin treatment of hyperimmunoglobulinemia E syndrome. J Allergy Clin Immunol. 1995;95:771-774.
Yescarta [package insert]. Santa Monica, CA: Kite Pharma; June 2024.
Lexi-Drugs. Lexicomp. Wolters Kluwer Health, Inc. Riverwoods, IL. Available at https://www.online.lexi.com [available with subscription]. Accessed May 7, 2025.
Lopriore E., Mearin M.L., Oepkes D., Devlieger R., Whitington P.F. Neonatal hemochromatosis: Management, outcome, and prevention. Prenat. Diagn. 2013;33:1221–1225.
Tate ED, Pranzatelli MR, Verhulst SJ, et al. Active comparator-controlled rater-blinded study of corticotropin-based immunotherapies for opsoclonus-myoclonus syndrome. J Child Neurol. 2012; 27:875-884.
Mutch LS, Johnston DL. Late presentation of opsoclonus-myoclonus-ataxia syndrome in a child with stage 4S neuroblastoma. J Pediatr Hematol Oncol. 2005;27(6):341-343.
Sonnenday CJ, Ratner LE, Zachary AA, et al. Preemptive therapy with plasmapheresis/intravenous immunoglobulin allows successful live donor renal transplantation in patients with a positive cross-match. Transplant Proc. 2002;34(5):1614-1616.
Razonable RR, Humar A. Cytomegalovirus in Solid Organ Transplantation. Am J Transplant. 2013;13:93-106.
Jordan SC, Toyoda M, Kahwaji J, et al. Clinical Aspects of Intravenous Immunoglobulin Use in Solid Organ Transplant Recipients. Am J Transplant. 2011;11:196-202
Kimberlin DW, Brady MT, Jackson MA, et al. Staphylococcus aureus. American Academy of Pediatrics. Red Book: 2018 Report of the Committee on Infectious Diseases. 2018:733-746
Gordon C, Amissah-Arthur MB, Gayed M, et al. British Society for Rheumatology guideline on management of systemic lupus erythematosus in adults. Rheumatology (Oxford). 2018;57:e1-45.
Abecma [package insert]. Summit, NJ: Celgene Corporation; March 2025.
Alyglo [package insert]. Teaneck, NJ: GC Biopharma USA, Inc.; December 2023.
Yimmugo [package insert]. Dreieich, Germany: Biotest AG; July 2024.
Rennebohm RM, Asdaghi N, Srivastava S, et al: Guidelines for treatment of Susac syndrome - an update. Int J Stroke 2020; 15(5):484-494.
Chang K, Frankovich J, Cooperstock M, et al. Clinical evaluation of youth with pediatric acute-onset neuropsychiatric syndrome (PANS): recommendations from the 2013 PANS Consensus Conference. J Child Adolesc Psychopharmacol. 2015;25(1):3-13.
Qivigy [package insert]. Fort Lee, NJ: Kedrion Biopharma Inc.; September 2025.
Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.
