Specialty Pharmacy Clinical Policy Bulletins Aetna Non-Medicare Prescription Drug Plan
Subject: SCIG SGM 2043-A P2024_R
Drug
Subcutaneous Immune Globulin (SCIG)
HIZENTRA
HYQVIA
CUTAQUIG
CUVITRU
XEMBIFY
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.
Hizentra is indicated as replacement therapy for primary humoral immunodeficiency in adults and pediatric patients 2 years of age and older.
Hizentra is indicated for the treatment of adult patients with chronic inflammatory demyelinating polyneuropathy (CIDP) as maintenance therapy to prevent relapse of neuromuscular disability and impairment.
Limitations of Use Hizentra maintenance therapy in CIDP has been systematically studied for 6 months and for a further 12 months in a follow-up study. Maintenance therapy beyond these periods should be individualized based upon the patient’s response and need for continued therapy.
HyQvia (Immune Globulin Infusion 10% [Human] with Recombinant Human Hyaluronidase)
HyQvia is indicated for the treatment of primary immunodeficiency in adults and pediatric patients two years of age and older.
HyQvia is indicated for the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) as maintenance therapy to prevent relapse of neuromuscular disability and impairment in adults.
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 Immunodeficiency
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):
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):
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):
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:
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),40 OR
The prescriber will re-evaluate the dose of IG and consider a dose adjustment (when appropriate).
Myasthenia Gravis
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 of 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 Polymyositis
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:
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:
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:
Platelet count < 30,000/mcL, or
Significant bleeding symptoms
ITP in pregnant women: authorization through delivery may be granted to pregnant women with ITP.
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)
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 Patients
Initial authorization of 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)
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)
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)
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)
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 Syndrome
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)
Authorization of 6 months may be granted for severe, refractory anemia associated with bone marrow suppression, with parvovirus B19 viremia.
Stiff-person Syndrome
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 Toxicities
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 nervous system 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 Aplasia
Authorization of 6 months may be granted for acquired red cell aplasia.
Acute Disseminated Encephalomyelitis
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 Disease
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 aquisita) 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 Anemia
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 Neutropenia
Authorization of 6 months may be granted for autoimmune neutropenia where treatment with G-CSF (granulocyte colony stimulating factor) is not appropriate.
Birdshot Retinochoroidopathy
Authorization of 6 months may be granted for birdshot (vitiliginous) retinochoroidopathy that is not responsive to immunosuppressives (e.g., corticosteroids, cyclosporine).
BK Virus Associated Nephropathy
Authorization of 6 months may be granted for BK virus associated nephropathy.
Churg-Strauss Syndrome
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 Meningoencephalitis
Authorization of 6 months may be granted for severe cases of enteroviral meningoencephalitis.
Hematophagocytic Lymphohistiocytosis (HLH) or Macrophage Activation Syndrome (MAS)
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 Newborn
Authorization of 6 months may be granted for isoimmune hemolytic disease in neonates.
HIV-associated Thrombocytopenia
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 has 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 or 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 Syndrome
Authorization of 6 months may be granted to treat severe eczema in hyperimmunoglobulinemia E syndrome.
Hypogammaglobulinemia from CAR-T therapy
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 Myeloma
Authorization of 6 months may be granted for multiple myeloma in members who have recurrent, serious infections despite the use of prophylactic antibiotics.
Neonatal Hemochromatosis
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-myoclonus
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
Post-transfusion Purpura
Authorization of 1 month may be granted for post-transfusion purpura.
Rasmussen Encephalitis
Authorization of 6 months may be granted for Rasmussen encephalitis in members whose symptoms do not improve with anti-epileptic drugs and corticosteroids.
Renal Transplantation
Authorization of 6 months may be granted for a member undergoing renal transplantation from a live donor with ABO incompatibility or positive cross match.
Secondary Immunosuppression Associated with Major Surgery, Hematological Malignancy, Major Burns, and Collagen-Vascular Diseases
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 Transplantation
Authorization of 6 months may be granted for solid organ transplantation for allosensitized members.
Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome
Authorization of 1 month may be granted for severe cases of toxic epidermal necrolysis or Stevens-Johnson syndrome.
Toxic Shock Syndrome
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 Erythematosus
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) Prophylaxis
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 Prophylaxis
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 Prophylaxis
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 Streptococcus
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 coverage criteria.
Appendix
Appendix A: Impaired Antibody Response to Pneumococcal Polysaccharide Vaccine
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)
Undergoing a medical or dental procedure where blood loss is anticipated
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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.
