Neighborhood Health Plan RI SCIG Coverage Update

Coverage Criteria for SCIG

Covered Indications (FDA-approved and Compendial)

Covered when ALL of the following are met:

Base coverage condition: Indication is an FDA-approved indication or a listed compendial use and all applicable approval criteria specific to that indication are satisfied; member has no exclusions to the prescribed therapy.

See indication-specific criteria in subsequent sections for detail

Primary Immunodeficiency - Initial Authorization Criteria

Initial authorization of 6 months may be granted for members meeting ANY one of the diagnostic-specific criteria sets below.

PI Initial diagnoses

1. Severe combined immunodeficiency (SCID) or congenital agammaglobulinemia: Diagnosis confirmed by genetic or molecular testing OR pretreatment IgG < 200 mg/dL OR absence/very low T cells (CD3 < 300/µL) or presence of maternal T cells (SCID only)
2. Wiskott-Aldrich syndrome, DiGeorge syndrome, ataxia-telangiectasia (or other non-SCID combined immunodeficiency): Diagnosis confirmed by genetic/molecular testing (if applicable) AND history of recurrent bacterial infections AND impaired antibody response to pneumococcal polysaccharide vaccine (see Appendix A)
See Appendix A for vaccine response details
3. Common variable immunodeficiency (CVID): Age ≥2 years AND other causes of immune deficiency excluded AND pretreatment IgG < 500 mg/dL or ≥2 SD below mean for age AND history of recurrent bacterial infections AND impaired antibody response to pneumococcal polysaccharide vaccine (see Appendix A)
4. Hypogammaglobulinemia, IgG subclass deficiency, selective IgA or IgM deficiency, or specific antibody deficiency: History of recurrent bacterial infections AND impaired pneumococcal vaccine response (see Appendix A) AND one or more specified laboratory findings (e.g., IgG <500 mg/dL; IgA <7 mg/dL with normal IgG/IgM; IgM <30 mg/dL with normal IgG/IgA; IgG subclass ≥2 SD below mean on ≥2 occasions; or specific antibody deficiency with normal total Ig levels)
Laboratory abnormalities must meet specified numeric thresholds
5. Other predominant antibody deficiency disorders: Meets criteria a, b, and c.i from section 4 above (history of recurrent infections, impaired pneumococcal vaccine response, and relevant hypogammaglobulinemia laboratory finding)
6. Other combined immunodeficiency: Meets criteria for non-SCID combined immunodeficiency as described under section 2 above (diagnosis confirmation, recurrent infections, impaired vaccine response)

Primary Immunodeficiency - Re-authorization Criteria

Re-authorization of 12 months may be granted when ALL of the following are met:

PI Re-authorization: 1) A reduction in the frequency of bacterial infections since initiation of IG therapy; AND 2) IgG trough levels are monitored at least yearly and maintained at or above the lower range of normal for age (when applicable), OR 3) Prescriber will re-evaluate IG dose and consider adjustment when appropriate.

Either condition 2 or 3 suffices in combination with 1

Myasthenia Gravis

Authorization may be granted for either short-term use or longer-term refractory disease.

MG short-term: Authorization of 1 month for members prescribed IG for worsening weakness, acute exacerbation, or pre-operative management (e.g., prior to thymectomy).

Worsening weakness includes increase in diplopia, ptosis, blurred vision, dysarthria, dysphagia, difficulty chewing, impaired respiratory status, fatigue, or limb weakness; acute exacerbations include severe swallowing difficulties or respiratory failure

MG refractory: Authorization of 6 months for refractory myasthenia gravis when patient has tried and failed 2 or more standard therapies (e.g., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, rituximab).

CIDP

CIDP initial and re-authorization criteria.

CIDP initial: Initial authorization of 3 months when: disease course is progressive or relapsing/remitting for ≥2 months; there is moderate to severe functional disability; and diagnosis is confirmed by electrodiagnostic studies.

CIDP re-authorization: Re-authorization of 6 months when there is significant improvement in disability and maintenance of improvement since IG initiation AND IG is being used at the lowest effective dose and frequency.

Dermatomyositis/Polymyositis

Initial and re-authorization criteria for inflammatory myopathies.

Myositis initial: Initial authorization of 3 months when member has at least 4 of the listed clinical, laboratory, or pathologic features (e.g., proximal muscle weakness; elevated CK or aldolase; muscle pain; myogenic EMG changes; anti-synthetase antibodies; non-destructive arthritis/arthralgias; systemic inflammatory signs; compatible pathology) AND standard first-line (corticosteroids) and second-line immunosuppressants have been tried and were unsuccessful or not tolerated, or are contraindicated.

Myositis re-authorization: Re-authorization of 6 months when there is significant improvement in disability and maintenance of improvement since initiation of IG therapy.

Idiopathic Thrombocytopenic Purpura (ITP)

Authorization per disease phase and age.

ITP initial (children): Authorization of 1 month for children (<18 years) with significant bleeding (e.g., mucosal bleeding), high risk for bleeding (see Appendix B), or when a rapid increase in platelets is required (e.g., for a procedure).

Provide member's bleeding risk factors or reason for rapid platelet increase (Appendix B)

ITP initial (adults): Authorization of 1 month for adults (≥18 years) with platelet count <30,000/mcL, or platelet count <50,000/mcL with significant bleeding/high bleeding risk or need for rapid platelet increase, when corticosteroid therapy is contraindicated and IG will be used alone or with corticosteroids.platelets <50,000/mcL when bleeding/high risk

ITP chronic/persistent: Authorization of 6 months for chronic/persistent ITP (≥3 months) when platelet count <30,000/mcL or platelets <50,000/mcL with significant bleeding/high risk or need for rapid platelet increase, AND relapse after previous response to IG or inadequate response/intolerance/contraindication to corticosteroid or anti-D therapy.

B-cell Chronic Lymphocytic Leukemia (CLL)

Prophylactic use criteria.

CLL initial: Initial authorization of 6 months when IG is prescribed for prophylaxis of bacterial infections AND member has history of recurrent sinopulmonary infections requiring IV antibiotics or hospitalization AND pretreatment serum IgG <500 mg/dL.pretreatment IgG <500 mg/dL

CLL re-authorization: Re-authorization of 6 months when a reduction in the frequency of bacterial infections has been demonstrated since initiation of IG therapy.

Pediatric HIV prophylaxis

Criteria for IG prophylaxis in pediatric HIV-infected patients.

Pediatric HIV initial: Initial authorization of 6 months when any of the following apply: pretreatment IgG <400 mg/dL for primary prophylaxis; history of >2 serious bacterial infections in 1 year; failure to form antibodies to common antigens (e.g., measles, pneumococcal, Hib); lives in high measles-prevalence area and failed to develop measles antibody after two MMR doses or exposed and requesting single dose; chronic bronchiectasis suboptimally responsive to therapy.pretreatment IgG <400 mg/dL

Pediatric HIV re-authorization: Re-authorization of 6 months when a reduction in the frequency of bacterial infections has been demonstrated since initiation of IG therapy.

BMT/HSCT

Prophylactic or early post-transplant use.

BMT/HSCT initial: Initial authorization of 6 months when IG is used to prevent graft-versus-host disease, interstitial pneumonia, septicemia, CMV and recurrent bacterial infections AND either IG is requested within the first 100 days post-transplant OR pretreatment serum IgG <400 mg/dL.pretreatment IgG <400 mg/dL or request within 100 days

BMT/HSCT re-authorization: Re-authorization of 6 months when a reduction in the frequency of bacterial infections has been demonstrated since initiation of IG therapy.

Multifocal Motor Neuropathy (MMN)

Initial and re-authorization criteria for MMN.

MMN initial: Initial authorization of 3 months when progressive multifocal asymmetrical weakness without objective sensory loss in ≥2 nerves for ≥1 month AND diagnosis confirmed by electrodiagnostic studies.

MMN re-authorization: Re-authorization of 6 months when there is significant improvement in disability and maintenance of improvement since initiation of IG therapy.

Guillain-Barré Syndrome (GBS)

Acute authorization criteria.

GBS authorization: Authorization of 1 month total when member has severe disease with significant weakness (e.g., inability to stand or walk without aid, respiratory weakness) AND onset of neurologic symptoms occurred less than 4 weeks from the anticipated start of therapy.onset <4 weeks

Lambert-Eaton Myasthenic Syndrome (LEMS)

Initial and re-authorization criteria for LEMS.

LEMS initial: Initial authorization of 6 months when diagnosis is confirmed by neurophysiology studies OR a positive anti-P/Q type VGCC antibody test AND anticholinesterases and amifampridine have been tried and were unsuccessful or not tolerated AND weakness is severe or venous access for plasmapheresis is difficult.

LEMS re-authorization: Re-authorization of 6 months when member is responding to therapy (stability or improvement in symptoms relative to natural course).

Guillain-Barre Syndrome (GBS) — 1 month authorization specifics

Authorization of 1 month may be granted for Guillain-Barre Syndrome when ALL of the following are met:

GBS criteria: 1. Member has severe disease with significant weakness (e.g., inability to stand or walk without aid, respiratory weakness); 2. Onset of neurologic symptoms occurred less than 4 weeks from the anticipated start of therapy.

Lambert-Eaton Myasthenic Syndrome (LEMS) — initial/re-authorization details

Initial authorization of 6 months may be granted for Lambert-Eaton Myasthenic Syndrome when ALL of the following are met; re-authorization when responding:

LEMS initial: a. Diagnosis confirmed by neurophysiology studies or positive anti-P/Q VGCC antibody test; b. Anticholinesterases and amifampridine have been tried but were unsuccessful or not tolerated; c. Weakness is severe or there is difficulty with venous access for plasmapheresis.

Re-authorization of 6 months when member is responding to therapy (stability or improvement).

Kawasaki Syndrome

Authorization of 1 month may be granted for:

Kawasaki Syndrome: Pediatric members with Kawasaki syndrome.

authorization 1 month

F/NAIT

Authorization of 6 months may be granted for:

Fetal/Neonatal Alloimmune Thrombocytopenia (F/NAIT): Treatment of fetal/neonatal alloimmune thrombocytopenia (F/NAIT).

authorization 6 months

Parvovirus B19-induced Pure Red Cell Aplasia (PRCA)

Authorization of 6 months may be granted for:

Parvovirus B19-induced Pure Red Cell Aplasia (PRCA): Severe, refractory anemia associated with bone marrow suppression and parvovirus B19 viremia.

authorization 6 months

Stiff-person Syndrome

Authorization of 6 months may be granted for stiff-person syndrome when ALL of the following are met:

Stiff-person syndrome: 1. Diagnosis confirmed by anti-GAD antibody testing; AND 2. Inadequate response to first-line treatment (benzodiazepines and/or baclofen).

authorization 6 months

Immune checkpoint inhibitor-related toxicities

Authorization of 1 month may be granted for management of immune checkpoint inhibitor-related toxicities when ALL of the following are met:

ICI toxicities: 1. Member experienced a moderate or severe adverse event to a PD-1 or PD-L1 inhibitor; AND 2. The offending medication has been held or discontinued; AND 3. Member experienced one or more specified nervous system adverse events (e.g., 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/myositis).

authorization 1 month

Acute Disseminated Encephalomyelitis

Authorization of 1 month may be granted for:

Acute disseminated encephalomyelitis (ADEM): Members who have had an insufficient response or a contraindication to intravenous corticosteroid treatment.

authorization 1 month

Autoimmune Mucocutaneous Blistering Disease

Authorization of 6 months may be granted for autoimmune mucocutaneous blistering disease when ALL of the following are met:

Autoimmune mucocutaneous blistering disease: 1. Diagnosis proven by biopsy and confirmed by pathology report; AND 2. Condition is rapidly progressing, extensive, or debilitating; AND 3. Member failed or experienced significant complications from standard treatment (corticosteroids, immunosuppressive agents).

Includes pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita

Autoimmune Hemolytic Anemia

Authorization of 6 months may be granted for:

Autoimmune Hemolytic Anemia (AIHA): Warm-type autoimmune hemolytic anemia in members who do not respond or have a contraindication to corticosteroids or splenectomy.

authorization 6 months

Autoimmune Neutropenia

Authorization of 6 months may be granted for:

Autoimmune Neutropenia: Autoimmune neutropenia where treatment with G-CSF is not appropriate.

authorization 6 months

Birdshot Retinochoroidopathy

Authorization of 6 months may be granted for:

Birdshot Retinochoroidopathy: Birdshot (vitiliginous) retinochoroidopathy not responsive to immunosuppressives (e.g., corticosteroids, cyclosporine).

authorization 6 months

BK Virus Associated Nephropathy

Authorization of 6 months may be granted for:

BK Virus Associated Nephropathy: BK virus associated nephropathy.

authorization 6 months

Churg-Strauss Syndrome

Authorization of 6 months may be granted for:

Churg-Strauss Syndrome: Severe, active Churg-Strauss syndrome as adjunctive therapy for members who have experienced failure, intolerance, or contraindication to other interventions.

authorization 6 months

Enteroviral Meningoencephalitis

Authorization of 6 months may be granted for:

Enteroviral Meningoencephalitis: Severe cases of enteroviral meningoencephalitis.

authorization 6 months

HLH / Macrophage Activation Syndrome (MAS)

Authorization of 6 months may be granted when ALL of the following are met:

HLH/MAS hypogammaglobulinemia: Treatment of hypogammaglobulinemia in HLH or MAS when total IgG is less than 400 mg/dL or ≥2 standard deviations below the mean for age.IgG < 400 mg/dL or >=2 SD below mean

authorization 6 months

Hemolytic Disease of Newborn

Authorization of 6 months may be granted for:

Hemolytic Disease of the Newborn: Isoimmune hemolytic disease in neonates.

authorization 6 months

HIV-associated Thrombocytopenia

Authorization of 6 months may be granted for HIV-associated thrombocytopenia when criteria below are met:

Pediatric HIV-associated thrombocytopenia: Pediatric members with IgG < 400 mg/dL and one of: ≥2 bacterial infections in 1 year despite prophylaxis; received 2 doses of measles vaccine and live in high-prevalence region; HIV-associated thrombocytopenia despite ART; chronic bronchiectasis suboptimally responsive to therapy; or T4 cell count ≥200/mm^3.IgG < 400 mg/dL

authorization 6 months

Adult HIV-associated thrombocytopenia: Adult members with significant bleeding, platelet count <20,000/mcL, and failure of RhIG in Rh-positive patients.platelets <20,000/mcL

authorization 6 months

Hyperimmunoglobulinemia E Syndrome

Authorization of 6 months may be granted for:

Hyperimmunoglobulinemia E Syndrome: Treatment of severe eczema in hyperimmunoglobulinemia E (Job) syndrome.

authorization 6 months

HIV-associated Thrombocytopenia — authorization details

HIV-associated Thrombocytopenia — Authorization of 6 months may be granted when the following are met:

Pediatric criteria: Pediatric members with IgG < 400 mg/dL and one of: (a) ≥2 bacterial infections in 1 year despite antibiotic prophylaxis; (b) received 2 doses of measles vaccine and live in high-prevalence region; (c) HIV-associated thrombocytopenia despite ART; (d) chronic bronchiectasis suboptimally responsive to therapy; (e) T4 cell count ≥200/mm^3.IgG < 400 mg/dL

all subcriteria are alternatives

Adult criteria: Adult members with significant bleeding, platelet count <20,000/mcL, and failure of RhIG in Rh-positive patients.platelets <20,000/mcL

all conditions required

Hyperimmunoglobulinemia E Syndrome — severe eczema authorization

Hyperimmunoglobulinemia E Syndrome — Authorization of 6 months may be granted to treat severe eczema.

HIES eczema: Severe eczema in hyperimmunoglobulinemia E syndrome.

authorization 6 months

Hypogammaglobulinemia from CAR-T therapy

Authorization of 6 months may be granted when the following are met:

CAR-T hypogammaglobulinemia: Member receiving CAR-T therapy and IgG < 400 mg/dL (includes but not limited to idecabtagene vicleucel, tisagenlecleucel, axicabtagene ciloleucel).IgG < 400 mg/dL

authorization 6 months

Multiple Myeloma

Multiple Myeloma — Authorization of 6 months may be granted when:

Multiple myeloma recurrent infections: Recurrent, serious infections despite prophylactic antibiotics.

authorization 6 months

Neonatal Hemochromatosis

Authorization of 6 months may be granted for prophylaxis when:

Neonatal hemochromatosis prophylaxis: Member is pregnant with history of a prior pregnancy ending in documented neonatal hemochromatosis.

authorization 6 months

Opsoclonus-myoclonus

Authorization of 6 months may be granted for either paraneoplastic-associated or refractory disease:

Opsoclonus-myoclonus indications: (1) Paraneoplastic opsoclonus-myoclonus-ataxia associated with neuroblastoma OR (2) Refractory opsoclonus-myoclonus as last-resort treatment.

authorization 6 months

Post-transfusion Purpura

Authorization of 1 month may be granted.

Post-transfusion purpura.

authorization 1 month

Rasmussen Encephalitis

Authorization of 6 months may be granted when the following are met:

Rasmussen encephalitis: Symptoms have not improved with antiepileptic drugs and corticosteroids.

authorization 6 months

Renal Transplantation

Authorization of 6 months may be granted for:

Renal transplant indications: Renal transplantation from live donor with ABO incompatibility OR positive crossmatch.

authorization 6 months

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 infections when ALL are met:

Secondary immunosuppression with IgG < 400 mg/dL associated with major surgery, hematological malignancy, extensive burns, or collagen-vascular disease.IgG < 400 mg/dL

authorization 6 months

Solid Organ Transplantation

Authorization of 6 months may be granted for:

Allosensitized solid organ transplant: Allosensitized member undergoing solid organ transplantation.

authorization 6 months

Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome

Authorization of 1 month may be granted for severe cases.

Toxic epidermal necrolysis or Stevens-Johnson syndrome: Severe toxic epidermal necrolysis or Stevens-Johnson syndrome.

authorization 1 month

Toxic Shock Syndrome

Authorization of 1 month may be granted when any of the following are present:

Toxic shock criteria: Infection refractory to several hours of aggressive therapy OR an undrainable focus is present OR persistent oliguria with pulmonary edema.

authorization 1 month

Systemic Lupus Erythematosus

Authorization of 6 months may be granted for severe, active disease when ALL of the following are met:

Severe, active SLE: Severe, active systemic lupus erythematosus with inadequate response, intolerance, or contraindication to first- and second-line therapies (examples listed in policy).

authorization 6 months

Measles (Rubeola) prophylaxis

Authorization of 1 month may be granted for postexposure prophylaxis when ALL of the following are met:

Measles postexposure prophylaxis: Postexposure prophylaxis to prevent or modify measles (rubeola) in susceptible members exposed less than 6 days previously.exposure <6 days

authorization 1 month

Tetanus treatment and prophylaxis

Authorization of 1 month may be granted as an alternative when TIG is unavailable.

Tetanus prophylaxis/treatment: Treatment or postexposure prophylaxis of tetanus as alternative when tetanus immune globulin (TIG) is unavailable.

authorization 1 month

Varicella prophylaxis

Authorization of 1 month may be granted when VZIG is unavailable for postexposure prophylaxis in susceptible individuals.

Varicella postexposure: Postexposure prophylaxis of varicella in susceptible individuals when varicella-zoster immune globulin (VZIG) is unavailable.

authorization 1 month

Toxic Necrotizing Fasciitis Due To Group A Streptococcus

Authorization of 1 month may be granted for members with invasive streptococcal fasciitis when ALL of the following are met:

Toxic necrotizing fasciitis due to group A streptococcus: Fasciitis due to invasive group A streptococcal infection.

authorization 1 month

Continuation of Therapy

Authorization may be granted for continuation of therapy when either the following criteria is met:

Continuation criteria

A: Member is currently receiving IG therapy for a condition with reauthorization criteria in section III and meets those reauthorization criteria.
B: For all other conditions (including new members), member meets the policy's initial authorization criteria.

All indications not explicitly listed as FDA-approved or compendial uses in this policy are considered experimental/investigational and not medically necessary. Coverage is allowed only for the indications enumerated in the policy when the specific approval criteria for that indication are satisfied and the member has no exclusions to the prescribed therapy.

For members evaluated for SCIG due to concern for an impaired antibody response to pneumococcal polysaccharide vaccine, Appendix A applies. Appendix A defines the condition as documented impaired antibody response to a pneumococcal polysaccharide vaccine in persons age ≥2 years, notes that the criterion is not established for children <2 years, and explicitly excludes therapy that is initiated in the hospital setting.

Summary of Appendix A criteria for an impaired pneumococcal vaccine response: the member must be age 2 years or older with demonstrated impaired antibody response to pneumococcal polysaccharide vaccination; documentation should include post‑vaccination Streptococcus pneumoniae antibody titers consistent with inadequate response. The Appendix specifies that these criteria are not established for children under 2 years and that requests for SCIG based on this indication will not be approved if therapy was started in the hospital setting.

OpenPayer

Subcutaneous Immune Globulin (SCIG) therapy coverage

Coverage Criteria for SCIG

Provider Requirements and Prior Authorization

Thresholds, Diagnostic and Coding-Related Values

Product and Clinical Definitions

Background and Clinical Context

Policy Revision History