BlueCross BlueShield of Tennessee Medical Policy Manual

Intravenous Immune Globulin (IVIG) Therapy

NDC CODE(S)

59730-6502-XX - Bivigam 5 g protein

 

59730-6503-XX - Bivigam 10 g protein

 

44206-0416-XX - Carimune NF 3 g protein

 

44206-0417-XX - Carimune 6 g protein

 

44206-0418-XX - Carimune 12 g protein

 

61953-0005-XX - Flebogamma 10% DIF 5, 10, 20 g protein

 

61953-0004-XX - Flebogamma 5% DIF 2.5, 5, 10, 20 g protein

 

13533-0800-XX - Gamunex-C 1, 2.5, 5, 10, 20, 40  g protein

 

00944-2700-XX - Gammagard Liquid - 1, 2.5, 5, 10, 20, 30 g protein

 

00944-2656-XX - Gammagard S/D 5 g protein

 

00944-2658-XX - Gammagard S/D 10 g protein

 

76125-0900-XX - Gammaked 1, 2.5, 5, 10, 20 g protein

 

64208-8234-XX - Gammaplex 2.5, 5, 10, 20 g protein

 

68982-0850-XX - Octagam 10% 2, 5, 10, 20 g protein

 

67467-0843-XX - Octagam 5% 1, 5 g protein

 

44206-0436-XX - Privigen  5 g protein

 

44206-0437-XX - Privigen  10 g protein

 

44206-0438-XX - Privigen  20 g protein 

 

44206-0439-X X- Privigen  40 g protein

DESCRIPTION

Immune globulins or immunoglobulins (Ig) are specialized glycoproteins which function in the body as antibodies in the immune system.  Produced by plasma cells, there are five human isotypes of immunoglobulins, IgA, IgD, IgE, IgG and IgM.  Of these, IgG, IgA and IgM are referred to as natural antibodies as they are produced without deliberate immunization or antigen exposure.  IgD and IgE are generally produced in response to the introduction of foreign antigens to which they bind and deactivate.  Together, all immunoglobulin isotypes are vital components of the body’s immune response.

IgG is the most common of the immunoglobulins.  It has multiple functions including placental antibody transfer, phagocytic cell surface binding and the activation of complement.  Commercial preparations of intravenous immune globulins (IVIGs) are sterile, highly purified IgG products manufactured from large pools of human plasma, typically from 1000 or more healthy blood donors.  They contain more than 95% unmodified IgG but only trace amounts of IgA and/or IgM.  IVIG products are used in the treatment of multiple conditions.

REFER TO DECISION SUPPORT TREE

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

·       Intravenous immune globulin (IVIG) therapyis considered medically appropriate if ALL of the following criteria are met:

§  Ataxic neuropathies associated with anti-GD1b IgM antibodies considered to be chronic disease

§  Autoimmune mucocutaneous blistering diseases if ALL of the following:

·       Diagnosis of ANY ONE of the following:

o   Pemphigus vulgaris

o   Pemphigus foliaceus

o   Bullous pemphigoid

o   Mucous membrane pemphigoid

o   Epidermolysis bullosa acquisita

o   Pemphigus gestationis (Herpes gestationis)

o   Linear IgA dermatosis

·       Disease is ALL of the following

o   Confirmed by biopsy

o   Severe, debilitating and extensive

o   Progressive

o   Refractory to trial of conventional therapy with corticosteroids and concurrent immunosuppressive treatment (e.g.,  azathioprine, cyclophosphamide, mycophenolate mofetil)

§  Bacterial infections associated with neonates, as treatment or prevention, if therapy is adjunctive (i.e., to increase efficacy of primary treatment)

§  Chronic Inflammatory/Immune Demyelinating Polyneuropathy (CIDP)if ALL of the following:

·       Disease course is progressive or relapsing/remitting for 2 months or longer

·       Individual has abnormal or absent deep tendon reflexes in upper or lower limbs

·       Electrodiagnostic testing indicates demyelination by ANY ONE of the following:

o   Partial motor conduction block in at least two motor nerves or in 1 nerve plus one other demyelination criterion listed here in at least 1 other nerve

o   Distal CMAP duration increase in at least 1 nerve plus one other demyelination criterion listed here in at least 1 other nerve

o   Abnormal temporal dispersion conduction must be present in at least 2 motor nerves

o   Reduced conduction velocity in at least 2 motor nerves

o   Prolonged distal motor latency in at least 2 motor nerves

o   Absent F wave in at least two motor nerves plus one other demyelination criterion listed here in at least 1 other nerve

o   Prolonged F wave latency in at least 2 motor nerves

·       Cerebrospinal fluid analysis indicates white cell count of <10 cells/mm3, CSF protein is elevated

·       Individual is intolerant of or refractory to corticosteroids (e.g. prednisolone, prednisone) given in therapeutic doses over at least three months

·       Initial approval will be for 3 months with subsequent authorizations for one year

·       Baseline in strength/weakness has been documented using objective clinical measuring tool (e.g. INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin) and renewals will require current results

§  Chronic lymphocytic lymphoma, for prevention of infections, if individual has ALL of the following:

·       Finding of ANY ONE of the following:

o   Hypogammaglobulinemia with IgG level < 200

o   Individual has a deficiency in producing antibodies in response to vaccination and recurrent infections that include a minimum of one of the following

§  Four or more ear infections within 1 year

§  Two or more serious sinus infections within 1 year

§  Two or more months of antibiotics with little effect

§  Two or more pneumonias within 1 year

§  Recurrent or deep skin abscesses

§  Need for intravenous antibiotics to clear infections

§  Two or more deep-seated infections including septicemia

·       Individual has failed with conventional therapy alone (e.g.,  azathioprine, cyclosporine and/or cyclophosphamide)

·       Authorization length is 1 course (1 month) for initial approval

§  Dermatomyositis/Polymyositis if ALL of the following:

·       Individual has failed corticosteroid therapy or corticosteroid therapy is contraindicated

·       Individual has failed a trial of immunosuppressants, e.g. methotrexate, azathioprine

·       Severe active disease state with skin rash and proximal weakness in upper and/or lower limbs

·       CPK >1,000 with documentation of previously normal CPK

·       Documented baseline per physical exam

·       Initial approval valid for 3 months

·       Renewals will require current CPK lab and physical exam

§  Fetal alloimmune thrombocytopenia (FAIT) for authorization until delivery date if ANY ONE of the following:

·       Individual has a history of a prior FAIT pregnancy

·       There is family history of the disease

·       Screening reveals platelet alloantibodies

§  Guillain-Barré Syndrome (GBS) if ALL of the following:

·       Disease is severe (individual requires assistance to walk

·       Onset of symptoms are recent (less than 1 month)

·       Approval will be granted for a maximum of 2 rounds of therapy within 6 weeks on onset

·       Authorization is valid for 2 months only

§  Immune thrombocytopenia / idiopathic thrombocytic purpura (ITP) for ANY ONE of the following:

·       Acute disease state of ANY ONE of the following with authorization valid for one month only:

o   Rapid rise in platelets is required for acute bleeding due to severe thrombocytopenia (e.g., platelet counts < 30 x 109/L)

o   Increase platelet counts prior to invasive surgical procedure, e.g., splenectomy, platelets < 100 x 109/L)

o   Severe thrombocytopenia (platelet count <20 x 109/L) and considered to be at risk for intracerebral hemorrhage

·       Chronic immune thrombocytopenia (CIT) if ALL of the following:

o   Increased risk for bleeding with platelet count <30 x 109/L

o   History of failure of, contraindication to or intolerance to corticosteroids

o   Duration of illness > 6 months

o   Individual ≥ 2 years of age

§  Kawasaki disease for the prevention of coronary artery aneurysms if ALL of the following:

·       Administered with concomitant aspirin therapy

·       Authorization valid for one month only

§  Lambert-Eaton Myasthenic Syndrome if the individual has failed conventional therapy

§  Measles postexposure for ANY ONE of the following:

·       Pregnant woman without evidence of measles immunity

·       Severely immunocompromised person (e.g., individual with severe primary immunodeficiency, bone marrow or stem cell transplant recipient, receiving treatment for acute lymphocytic leukemia, diagnosed with AIDS or HIV)

§  Multifocal motor neuropathy if ALL of the following:

·       Partial conduction block or abnormal temporal dispersion conduction present in at least 2 nerves

·       Baseline in strength/weakness documented using objective clinical measurement tool (e.g., INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin)with renewal to require current results

·       Initial authorization length of 1 course (1 month) to assess viability of treatment

§  Multiple sclerosis if ALL of the following:

·       Disease is relapsing-remitting

·       As second-line therapy (i.e., after failure of initial treatment of choice)

§  Myasthenia gravis with ALL of the following:

·       Individual in myasthenic crisis (impending respiratory or bulbar compromise)

·       Disease is chronic with failure of conventional therapy alone (azathioprine, cyclosporine and/or cyclophosphamide), steroids

·       Exacerbation of disease such as difficulty swallowing, acute respiratory failure or functional disability leading to discontinuation of physical activity

§  Parvovirus B19 if ALL the following:

·       Disease is chronic

·       Individual with severe anemia secondary to bone marrow suppression

§  Pediatric HIV for prevention of bacterial infection if experiencing serious bacterial infections over a 1 year period (e.g., meningitis, bacteremia, pneumonia)

§  Primary immunodeficiencies (PID)/defective antibody synthesis (i.e., genetic defects),including but not limited to Common variable immunodeficiency (CVID) (i.e., hypogammaglobulinemia with recurrent infections); Congenital agammaglobulinemia (e.g.,  X-linked agammaglobulinemia); Hyperimmunoglobulin E (Hyper-IgE) syndrome; Severe combined immunodeficiencies (SCID); Wiskott-Aldrich syndrome (thrombocytopenia and eczema); X-linked hyperimmunoglobulin M (Hyper-IgM) syndrome if ANY ONE of the following:

·       Hypogammaglobulinemia with IgG level < 200

·       Individual has history of infections with ALL of the following:

o   Deficiency in producing antibodies in response to vaccination with titers drawn before challenging with vaccination and between 4 and 8 weeks of vaccination

o   Recurrent infections that include a minimum of one of the following:

§  Four or more ear infections within 1 year

§  Two or more serious sinus infections within 1 year

§  Two or more months of antibiotics with little effect

§  Two or more pneumonias within 1 year

§  Recurrent or deep skin abscesses

§  Need for intravenous antibiotics to clear infections

§  Two or more deep-seated infections including septicemia

§  Solid organ or bone marrow transplant if ANY ONE of the following:

·       Prior to solid organ transplant or bone marrow if high risk for antibody-mediated rejection (e.g., highly sensitized or receiving an ABO/ blood type incompatible organ)

·       Post solid organ or bone marrow transplant for treatment of an antibody-mediated rejection

·       Prevention of cytomegalovirus (CMV) induced pneumonitis

§  Stem Cell or Allogeneic Bone Marrow transplant if ALL of the following:

·       Used for ANY ONE of the following:

o   Hematopoietic cell transplant (regardless of cell source) with severe hypogammaglobulinemia (i.e., IgG levels less than 400mg/dL)

o   Prevention of graft-versus-host disease (GVHD) associated with interstitial pneumonia or infection (e.g.. cytomegalovirus, varicella-zoster virus, recurrent bacterial infection) as adjunctive treatment

o   Prevention of infection (i.e., cytomegalovirus)

·       Individual’s transplant was less than 100 days ago

·       Authorization is valid for 3 months

§  Stiff person syndrome if ALL of the following:

·       Individual positive for anti-GAD antibodies

·       Severe disability in carrying out daily activities with baseline physical exam documented

·       Failure of a minimum of two of the following therapies:

o   Benzodiazepines (e.g., diazepam, clonazepam)

o   Baclofen

o   Phenytoin

o   Clonidine

o   Tizanidine

§  Tetanus postexposure when tetanus immune globulin (TIG) is unavailable

§  Toxic shock syndrome if ALL of the following:

·       Treatment is adjunctive

·       Individual is severely ill with ANY ONE of the following:

o   Infection refractory to several hours of aggressive therapy

o   Presence of an undrainable focus

o   Persistent oliguria with pulmonary edema

·       Authorization is valid for 1 course (1 month) only

RENEWAL CRITERIA

·       Intravenous immune globulin (IVIG) therapy is considered medically appropriate for renewal therapy if ALL of the following criteria are met:

 

§  Autoimmune mucocutaneous blistering diseases with ALL of the following:

·       Documented improvement over baseline per physical exam

·       Renewals will be approved for 6 months

§  Chronic Immune Thrombocytopenia/ITP with ALL of the following:

·       Disease response as indicated by the achievement and maintenance of a platelet count of at least 50 X 109/L as necessary to reduce the risk for bleeding

·       Renewals will be approved for a year

§  Chronic Inflammatory/Immune Demyelinating Polyneuropathy with ALL of the following:

·       Individual has demonstrated a clinical response to therapy based on an objective clinical measuring tool (e.g. INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin)

·       Renewals will be approved for a year

§  Chronic lymphocytic leukemia with ALL of the following:

·       Disease response as evidenced by one or more of the following:

o   Decrease in the frequency of infection

o   Decrease in the severity of infection

·       Renewals will be approved for 6 months

§  Dermatomyositis/Polymyositis with ALL of the following:

·       CPK (Creatine phosphokinase) levels are lower upon renewal request

·       Improvement above baseline per physical exam

·       Renewals will be approved for 6 months

§  Multifocal Motor Neuropathy with ALL of the following: 

·       Individual has demonstrated a clinical response to therapy based on an objective clinical measuring tool (e.g. INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin)

·       Renewals will be approved for a year

§  Pediatric HIV for bacterial control or prevention if ALL of the following:

·       Patient’s age does not exceed 13 years

·       Renewals will be approved for a year

§  Primary immunodeficiency (PID) if ALL of the following:

·       Disease response as evidenced by one or more of the following:

o   Decrease in the frequency of infection

o   Decrease in the severity of infection

·       Renewals will be approved for a year

§  Solid organ (kidney, liver, lung, heart, pancreas) and bone marrow transplant complications if ALL of the following:

·       Disease response

·       Renewals will be approved for a year

§  Stem Cell or Allogeneic Bone Marrow Transplant if ALL of the following:

·       Patient’s IgG is less than or equal to 400mg/dL

·       Therapy does not exceed 360 days past patient’s allogeneic bone marrow transplantation

·       May only be renewed for coverage up to 360 days post patient’s allogeneic bone marrow transplantation

§  Stiff Person Disease  if ALL of the following:

·       Documented improvement over baseline per physical exam

·       Renewals will be approved for a year

INDICATION(S)*

DOSAGE

Auto-immune blistering diseases

2g/kg divided over 5 days in a 28 day cycle

CLL

400mg/kg every 3 weeks

PID

up to 800mg/kg every 21 days

CIDP

2g/kg divided over 2-4 days X 1, then 1g/kg every 21 days.

Dermatomyositis/Polymyositis

2g/kg divided over 5 days in a 28 day cycle

ITP

2g/kg divided over 5 days in a 28 day cycle

FAIT

1g/kg/week until delivery

Guillain-Barre

2g/kg divided over 5 days x 1 cycle

Hemolytic disease of the newborn

1g/kg x 1 dose, may be repeated once if needed

Kawasaki’s Disease (Pediatric Patients)

2g/kg x 1 single dose

Multifocal Motor Neuropathy

2g/kg divided over 5 days in a 28 day cycle

Myasthenia Gravis

1 g/kg x 1 dose (acute attacks)

Pediatric HIV

400mg/kg every 28 days

Bone Marrow or Stem Cell Transplant

500mg/kg/week x 90 days, then 500 mg/kg/month up to 360 days post-transplant

Complications of transplanted solid organ: (kidney, liver, lung, heart, pancreas) transplant

2g/kg divided over 5 days in a 28 day cycle

Stiff Person

2g/kg divided over 5 days in a 28 day cycle

Toxic shock syndrome

2g/kg divided over 5 days x 1 cycle

*Dosing for IVIG is highly variable depending on numerous patient specific factors, indication(s), and the specific product selected. For specific dosing regimens refer to current prescribing literature.

APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS

BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.

IMPORTANT REMINDER

We develop Medical Policies to provide guidance to Members and Providers.  This Medical Policy relates only to the services or supplies described in it.  The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy.  For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed.  If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.

ADDITIONAL INFORMATION 

For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).

No controlled studies were found in the published literature that validate the use of intravenous immune globulin (IVIG) therapy for the treatment of other conditions or diseases.

SOURCES

Attarian, S., Boucraut, J., Uzenot, D., Delmont, E., Verschueren, A., Franques, J., et al. (2010). Chronic ataxic neuropathies associated with antii-GD1b IgM antibodies: response to IVIG therapy. The Journal of Neurology, Neurosurgery and Psychiatry, 81: 61-64.

Bhatti, A., Gazali, Z. (2015, December). Recent advances and review on treatment of stiff person syndrome in adults and pediatric patients. Cureus, 7(12): e427. DOI 10.7759.

BlueCross BlueShield Association. Medical Policy Reference Manual. (5:2015). Immune globulin therapy (8.01.05). Retrieved June 3, 2016 from BlueWeb.

Lexicomp Online. (2016, March). AHFS DI. Immune Globulin. Retrieved June 3, 2016 from Lexicomp Online with AHFS.

MICROMEDEX Healthcare Series. Drugdex Evaluations. (2016, May). Immune globulin. Retrieved June 3, 2016 from MICROMEDEX Healthcare Series.

National Organization for Rare Disorders (NORD). (2010). Stiff person syndrome. Retrieved June 3, 2016 from http://rarediseases.org/rare-diseases/stiff-person-syndrome/.

U. S. Food and Drug Administration.  (2012, June). Center for Biologics Evaluation and Research. Gammagard® liquid, immune globulin infusion (human), 10% Solution, for intravenous and subcutaneous administration. Retrieved February 12, 2015 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM070010.pdf.

U. S. Food and Drug Administration.  (2013, July). Center for Biologics Evaluation and Research.   Gammagard® S/D immune globulin intravenous (human). Retrieved February 12, 2015 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM197905.pdf.

U. S. Food and Drug Administration. (2007, July). Center for Biologics Evaluation and Research. Privigen®, immune globulin intravenous (human), 10% liquid. Retrieved November 13, 2012 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM303092.pdf.

U. S. Food and Drug Administration. (2009, February). Center for Biologics Evaluation and Research. Gamunex®-C, [Immune Globulin Injection (Human). Retrieved November 13, 2012 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM069968.pdf.

U. S. Food and Drug Administration. (2009, September). Center for Biologics Evaluation and Research. Gammaplex® immune globulin intravenous (human), 5% liquid. Retrieved November 13, 2012 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM182963.pdf.

U. S. Food and Drug Administration. (2012, January). Center for Biologics Evaluation and Research. Flebogamma® 5% DIF (immune globulin intravenous [human]), solution for intravenous administration. Retrieved November 13, 2012 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM172599.pdf.

U. S. Food and Drug Administration. (2012, January). Center for Biologics Evaluation and Research. Flebogamma® 10% DIF [immune globulin intravenous (human)] for intravenous administration, 10% liquid preparation. Retrieved November 13, 2012 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM296138.pdf.

U. S. Food and Drug Administration. (2013, August). Center for Biologics Evaluation and Research. Carimune® NF, nanofiltered: immune globulin intravenous (human). Retrieved February 17, 2015 from http://www.fda.gov/downloads/BiologicsBloodVaccines/UCM152763.pdf.

U. S. Food and Drug Administration. (2013, June). Center for Biologics Evaluation and Research. Immune globulin intravenous (human), 10% liquid, Bivigam®. Retrieved March 5, 2015 from http://www.fda.gov/downloads/biologicsbloodvaccines/bloodbloodproducts/approvedproducts/licensedproductsblas/fractionatedplasmaproducts/ucm334609.pdf.

U. S. Food and Drug Administration. (2014, July). Center for Biologics Evaluation and Research. Octagam® 10% [immune globulin intravenous (human)] liquid solution for intravenous administration. Retrieved February 12, 2015 from http://www.fda.gov/downloads/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/UCM064946.pdf.

ORIGINAL EFFECTIVE DATE:  12/4/1997

MOST RECENT REVIEW DATE:  12/1/2016

ID_MRx

Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.

Pharmaceutical Decision Support Tree

Intravenous Immune Globulin (IVIG) Therapy (Bivigam®, Carimune®, Carimune® NF, Flebogamma® 5% DIF, Flebogamma® 10% DIF, Gamunex®-C, Gammagard® Liquid, Gammagard® S/D, Gammaked®, Gammaplex®, Octagam® 5%, Octagam® 10% and Privigen®)

 

1.     Is this the initial request for IVIG therapy?

 

If yes, go to question #2

If no, go to questions #51

 

2.     Is there a baseline BUN (blood urea nitrogen) and serum creatinine value that has been obtained within 30 days?

 

If yes, go to question #3

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

3.     Does the individual have a diagnosis of ataxic neuropathies associated with anti-GD1b IgM antibodies considered to be chronic disease?

 

If yes, go to question #4

If no, go to question #5

 

4.     Is the request for 460 billable units or less per 28 days (1 billable unit = 500 mg) for a period of 1 month for review with specific dosage regimen per current prescribing literature?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

5.     Does the individual have a diagnosis ofautoimmune mucocutaneous blistering diseases with a diagnosis of ANY ONE of the following?

 

·       Pemphigus vulgaris

·       Pemphigus foliaceus

·       Bullous pemphigoid

·       Mucous membrane pemphigoid

·       Epidermolysis bullosa acquisita

·       Pemphigus gestationis (Herpes gestationis)

·       Linear IgA dermatosis

       And the disease is ALL of the following:

·       Confirmed by biopsy

·       Progressive, severe, debilitating and extensive

·       Refractory to trial of conventional therapy with corticosteroids and concurrent immunosuppressive treatment (e.g.,  azathioprine, cyclophosphamide, mycophenolate mofetil)

 

If yes, go to question #6

If no, go to question #7


 

6.     Is the request for 460 billable units or less per 28 days (1 billable unit = 500 mg) for a period of 6 months with dosing at 2g/kg divided over 5 days in a 28 day cycle?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

7.     Does the individual have a diagnosis of bacterial infections associated with neonates, as treatment or prevention, if therapy is adjunctive (i.e., to increase efficacy of primary treatment)?

 

If yes, go to question #8

If no, go to question #9

 

8.     Is specific dosage determined by current prescribing literature with authorization valid for 1 course (1 month) only?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

9.     Does the individual have a diagnosis of chronic inflammatory/immune demyelinating polyneuropathy (CIDP) if ALL of the following?

 

·       Disease course is progressive or relapsing/remitting for 2 months or longer

·       Individual has abnormal or absent deep tendon reflexes in upper or lower limbs

·       Electrodiagnostic testing indicates demyelination by ANY ONE of the following:

o   Partial motor conduction block in at least two motor nerves or in 1 nerve plus one other demyelination criterion listed here in at least 1 other nerve

o   Distal CMAP duration increase in at least 1 nerve plus one other demyelination criterion listed here in at least 1 other nerve

o   Abnormal temporal dispersion conduction must be present in at least 2 motor nerves

o   Reduced conduction velocity in at least 2 motor nerves

o   Prolonged distal motor latency in at least 2 motor nerves

o   Absent F wave in at least two motor nerves plus one other demyelination criterion listed here in at least 1 other nerve

o   Prolonged F wave latency in at least 2 motor nerves

·       Cerebrospinal fluid analysis indicates white cell count of <10 cells/mm3 and CSF protein is elevated

·       Individual is intolerant of or refractory to corticosteroids (e.g. prednisolone, prednisone) given in therapeutic doses over at least three months

·       Initial approval will be for 3 months with subsequent authorizations for one year

·       Baseline in strength/weakness has been documented using objective clinical measuring tool (e.g. INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin) and renewals will require current results

 

If yes, go to question #10

If no, go to question #11

 

10.  Is the request for 460 billable units per 4 days or less for loading dose and 230 billable units per 21 days for maintenance with a dosage of 2g/kg divided over 2-4 days X 1, then 1g/kg every 21 days?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

11.  Does the individual have a diagnosis of chronic lymphocytic lymphoma, for prevention of infections, if there is a finding of ANY ONE of the following?

 

·       Hypogammaglobulinemia with IgG level < 200

·       Individual has a deficiency in producing antibodies in response to vaccination and recurrent infections that include a minimum of one of the following

o   Four or more ear infections within 1 year

o   Two or more serious sinus infections within 1 year

o   Two or more months of antibiotics with little effect

o   Two or more pneumonias within 1 year

o   Recurrent or deep skin abscesses

o   Need for intravenous antibiotics to clear infections

o   Two or more deep-seated infections including septicemia

 

If yes, go to question #12

If no, go to question #14

 

12.  Has the individual failed with conventional therapy alone (e.g., azathioprine, cyclosporine and/or cyclophosphamide)?

 

If yes, go to question #13

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

13.  Is the request for 92 billable units or less for a dosage of 400mg/kg every 3 weeks for an initial approval authorization of 1 course (1 month) for review?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

14.  Does the individual have a diagnosis of dermatomyositis/polymyositis if ALL of the following?

 

·       Individual has failed corticosteroid therapy or corticosteroid therapy is contraindicated

·       Individual has failed a trial of immunosuppressants, e.g. methotrexate, azathioprine

·       Severe active disease state with skin rash and proximal weakness in upper and/or lower limbs

·       CPK >1,000 with documentation of previously normal CPK

·       Documented baseline per physical exam

·       Renewals will require current CPK lab and physical exam

 

If yes, go to question #15

If no, go to question #16

 

15.  Is the request for 460 billable units per 28 days or less for a dosage of 2g/kg divided over 5 days in a 28 day cycle for an initial approval valid for 3 months?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria


 

16.  Does the individual have a diagnosis of fetal alloimmune thrombocytopenia (FAIT) for authorization until delivery date if ANY ONE of the following?

 

·       Individual has a history of a prior FAIT pregnancy

·       There is family history of the disease

·       Screening reveals platelet alloantibodies

 

If yes, go to question #17

If no, go to question #18

 

17.  Is the request for 200 billable units or less per 7 days for a dosage of 1g/kg/week until delivery?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

18.  Does the individual have a diagnosis of Guillain-Barré Syndrome (GBS) if ALL of the following?

 

·       Disease is severe (individual requires assistance to walk

·       Onset of symptoms are recent (less than 1 month)

·       Approval will be granted for a maximum of 2 rounds of therapy within 6 weeks on onset

 

If yes, go to question #19

If no, go to question #20

 

19.  Is the request for 460 billable units or less per 5 days x 1 cycle for a dosage of 2g/kg divided over 5 days x 1 cycle with and authorization is valid for 2 months only?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

20.  Does the individual have a diagnosis of immune thrombocytopenia / idiopathic thrombocytic purpura (ITP) for the acute disease state of ANY ONE of the following with authorization valid for one month only?

 

·       Rapid rise in platelets is required for acute bleeding due to severe thrombocytopenia (e.g., platelet counts < 30 x 109/L)

·       Increase platelet counts prior to invasive surgical procedure, e.g., splenectomy, platelets < 100 x 109/L)

·       Severe thrombocytopenia (platelet count <20 x 109/L) and considered to be at risk for intracerebral hemorrhage

 

If yes, go to question #22

If no, go to question #21


 

21.  Does the individual have a diagnosis of immune thrombocytopenia / idiopathic thrombocytic purpura (ITP) for Chronic immune thrombocytopenia (CIT) if ALL of the following?

 

·       Increased risk for bleeding with platelet count <30 x 109/L

·       History of failure of, contraindication to or intolerance to corticosteroids

·       Duration of illness > 6 months

·       Individual ≥ 2 years of age

 

If yes, go to question #22

If no, go to question #23

 

22.  Is the request for 460 billable units or less per 28 days for dosage of 2g/kg divided over 5 days in a 28 day cycle?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

23.  Does the individual have a diagnosis of Kawasaki disease for the prevention of coronary artery aneurysms if ALL of the following?

 

·       Administered with concomitant aspirin therapy

·       Authorization valid for one month only

 

If yes, go to question #24

If no, go to question #25

 

24.  Is the request for 232 billable units x 1 dose for dosage of 2g/kg x 1 single dose?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

25.  Does the individual have a diagnosis of Lambert-Eaton Myasthenic Syndrome if the individual has failed conventional therapy?

 

If yes, go to question #26

If no, go to question #27

 

26.  Is the request for 460 billable units or less per 28 days for specific dosage determined by current prescribing literature with authorization valid for 1 course (1 month) for review?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

27.  Does the individual have a diagnosis of measles postexposure for ANY ONE of the following?

 

·       Pregnant woman without evidence of measles immunity

·       Severely immunocompromised person (e.g., individual with severe primary immunodeficiency, bone marrow or stem cell transplant recipient, receiving treatment for acute lymphocytic leukemia, diagnosed with AIDS or HIV)

 

If yes, go to question #28

If no, go to question #29

 

28.  Is the request for specific dosage determined by current prescribing literature with authorization valid for 1 course (1 month) for review?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

29.  Does the individual have a diagnosis of multifocal motor neuropathy if ALL of the following?

 

·       Partial conduction block or abnormal temporal dispersion conduction present in at least 2 nerves

·       Baseline in strength/weakness documented using objective clinical measurement tool (e.g., INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin) with renewal to require current results

 

If yes, go to question #30

If no, go to question #31

 

30.  Is the request for 460 billable units or less per 28 days for dosage of 2g/kg divided over 5 days in a 28 day cycle for 1 month to assess viability of treatment?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

31.  Does the individual have a diagnosis of multiple sclerosis if ALL of the following?

 

·       Disease is relapsing-remitting

·       As second-line therapy (i.e., after failure of initial treatment of choice)

 

If yes, go to question #32

If no, go to question #33

 

32.  Is the request for specific dosage determined by current prescribing literature with authorization valid for 1 course (1 month) for review?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

33.  Does the individual have a diagnosis of myasthenia gravis if ALL of the following?

 

·       Individual in myasthenic crisis (impending respiratory or bulbar compromise)

·       Disease is chronic with failure of conventional therapy alone (azathioprine, cyclosporine and/or cyclophosphamide), steroids

·       Exacerbation of disease such as difficulty swallowing, acute respiratory failure or functional disability leading to discontinuation of physical activity

 

If yes, go to question #34

If no, go to question #35

 

34.  Is the request for 230 billable units X 1 dose for dosage of 1 g/kg x 1 dose (acute attacks)?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria


 

35.  Does the individual have a diagnosis of parvovirus B19 if ALL the following?

 

·       Disease is chronic

·       Individual with severe anemia secondary to bone marrow suppression

 

If yes, go to question #36

If no, go to question #37

 

36.  Is the request for specific dosage determined by current prescribing literature with authorization valid for 1 course (1 month) for review?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

37.  Does the individual have a diagnosis of pediatric HIV for prevention of bacterial infection if experiencing serious bacterial infections over a 1 year period (e.g., meningitis, bacteremia, pneumonia)?

 

If yes, go to question #38

If no, go to question #39

 

38.  Is the request for 47 billable units or less per 28 days for dosage of 400mg/kg every 28 days?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

39.  Does the individual have a diagnosis of primary immunodeficiencies (PID)/defective antibody synthesis (i.e., genetic defects),including but not limited to Common variable immunodeficiency (CVID) (i.e., hypogammaglobulinemia with recurrent infections); Congenital agammaglobulinemia (e.g.,  X-linked agammaglobulinemia); Hyperimmunoglobulin E (Hyper-IgE) syndrome; Severe combined immunodeficiencies (SCID); Wiskott-Aldrich syndrome (thrombocytopenia and eczema); X-linked hyperimmunoglobulin M (Hyper-IgM) syndrome if ANY ONE of the following?

 

·       Hypogammaglobulinemia with IgG level < 200

·       Individual has history of infections with ALL of the following:

o   Deficiency in producing antibodies in response to vaccination with titers drawn before challenging with vaccination and between 4 and 8 weeks of vaccination

o   Recurrent infections that include a minimum of one of the following:

§  Four or more ear infections within 1 year

§  Two or more serious sinus infections within 1 year

§  Two or more months of antibiotics with little effect

§  Two or more pneumonias within 1 year

§  Recurrent or deep skin abscesses

§  Need for intravenous antibiotics to clear infections

§  Two or more deep-seated infections including septicemia

 

If yes, go to question #40

If no, go to question #41

 

40.  Is the request for 184 billable units or less per 21 days for dosage up to 800mg/kg every 21 days?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

41.  Does the individual have a diagnosis of a solid organ or bone marrowtransplant if ANY ONE of the following?

 

·       Prior to solid organ transplant or bone marrow if high risk for antibody-mediated rejection (e.g., highly sensitized or receiving an ABO/ blood type incompatible organ)

·       Post solid organ or bone marrow transplant for treatment of an antibody-mediated rejection

·       Prevention of cytomegalovirus (CMV) induced pneumonitis

 

If yes, go to question #42

If no, go to question #43

 

42.  Is the request for 460 billable units or less per 28 days for dosage of 2g/kg divided over 5 days in a 28 day cycle?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

43.  Does the individual have a diagnosis of a stem cell or allogeneic bone marrow transplant if ALL of the following?

 

·       Used for ANY ONE of the following:

o   Hematopoietic cell transplant (regardless of cell source) with severe hypogammaglobulinemia (i.e., IgG levels less than 400mg/dL)

o   Prevention of graft-versus-host disease (GVHD) associated with interstitial pneumonia or infection (e.g.. cytomegalovirus, varicella-zoster virus, recurrent bacterial infection) as adjunctive treatment

o   Prevention of infection (i.e., cytomegalovirus)

·       Individual’s transplant was less than 100 days ago

·       Authorization is valid for 3 months

 

If yes, go to question #44

If no, go to question #45

 

44.  Is the request for 115 billable units per 7 days for dosage of 500mg/kg/week x 90 days, then 500 mg/kg/month up to 360 days post-transplant?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

45.  Does the individual have a diagnosis of Stiff person syndrome with ALL of the following?

 

·       Individual positive for anti-GAD antibodies

·       Severe disability in carrying out daily activities with baseline physical exam documented

·       Failure of a minimum of two of the following therapies:

o   Benzodiazepines (e.g., diazepam, clonazepam)

o   Baclofen

o   Phenytoin

o   Clonidine

o   Tizanidine

 

If yes, go to question #46

If no, go to question #47

 

46.  Is the request for 460 billable units or less per 28 days for dosage of 2g/kg divided over 5 days in a 28 day cycle?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

47.  Does the individual have a diagnosis of tetanus postexposure when tetanus immune globulin (TIG) is unavailable?

 

If yes, go to question #48

If no, go to question #49

 

48.  Is the request for 460 billable units or less per 28 days (1 billable unit = 500 mg) for a period of 1 month for review with specific dosage regimen per current prescribing literature?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

49.  Does the individual have a diagnosis of toxic shock syndrome with ALL of the following?

 

·       Treatment is adjunctive

·       Individual is severely ill with ANY ONE of the following:

o   Infection refractory to several hours of aggressive therapy

o   Presence of an undrainable focus

o   Persistent oliguria with pulmonary edema

·       Authorization is valid for 1 course (1 month) only

 

If yes, go to question #50

If no, go to question #51

 

50.  Is the request for 460 billable units or less per 5 days x 1 cycle for dosage of 2g/kg divided over 5 days x 1 cycle?

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

51.  Was the infusion completed with absence of unacceptable toxicity?

 

If yes, go to question #52

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

52.  Does the individual continue to meet the initial approval criteria for the particular indication as required in questions 3 through 50?  

 

If yes, go to question #53

If no, this does not meet medical necessity and/or medical appropriateness criteria

 

53.  Was a BUN level and serum creatinine obtained within the last 6 months with the concentration and rate of infusion adjusted accordingly?

 

If yes, go to question #54

If no, this does not meet medical necessity and/or medical appropriateness criteria


 

54.  Is the request for diagnosis of autoimmune mucocutaneous blistering diseases with ALL of the following?

 

·       Documented improvement over baseline per physical exam

·       Renewals will be approved for 6 months

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #55

 

55.  Is the request for a diagnosis of chronic immune thrombocytopenia/ITP with ALL of the following?

 

·       Disease response as indicated by the achievement and maintenance of a platelet count of at least 50 X 109/L as necessary to reduce the risk for bleeding

·       Renewals will be approved for a year

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #56

 

56.  Is the request for a diagnosis of Chronic Inflammatory/Immune Demyelinating Polyneuropathy with ALL of the following?

 

·       Individual has demonstrated a clinical response to therapy based on an objective clinical measuring tool (e.g. INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin)

·       Renewals will be approved for a year

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #57

 

57.  Is the request for chronic lymphocytic leukemia with ALL of the following?

 

·       Disease response as evidenced by decrease in the frequency of infection and/or decrease in the severity of infection

·       Renewals will be approved for 6 months

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #58

 

58.  Is the request for Dermatomyositis/Polymyositis with ALL of the following?

 

·       CPK (Creatine phosphokinase) levels are lower upon renewal request

·       Improvement above baseline per physical exam

·       Renewals will be approved for 6 months

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #59


 

59.  Is the request for Multifocal Motor Neuropathy with ALL of the following?

 

·       Individual has demonstrated a clinical response to therapy based on an objective clinical measuring tool (e.g. INCAT, Medical Research Council (MRC) muscle strength,6 MWT, Rankin, Modified Rankin)

·       Renewals will be approved for a year

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #60

 

60.  Is the request for Pediatric HIV for bacterial control or prevention with ALL of the following?

 

·       Patient’s age does not exceed 13 years

·       Renewals will be approved for a year

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #61

 

61.  Is the request for primary immunodeficiency (PID) with ALL of the following?

 

·       Disease response as evidenced by decrease in the frequency of infection and/or decrease in the severity of infection

·       Renewals will be approved for a year

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #62

 

62.  Is the request for solid organ (kidney, liver, lung, heart, pancreas) and bone marrow transplant complications if ALL of the following?

 

·       Disease response is shown

·       Renewals will be approved for a year

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #63

 

63.  Is the request for Stem Cell or Allogeneic Bone Marrow Transplant if ALL of the following?

 

·       Patient’s IgG is less than or equal to 400mg/dL

·       Therapy does not exceed 360 days past patient’s allogeneic bone marrow transplantation

·       May only be renewed for coverage up to 360 days post patient’s allogeneic bone marrow transplantation

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #64

 

64.  Is the request for Stiff Person Disease if ALL of the following?

 

·       Documented improvement over baseline per physical exam

·       Renewals will be approved for a year

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, go to question #65

 

65.  Is the request for renewal for an additional indication on review?

 

·       May be considered with submission of clinical information indicating disease improvement

·       Renewal for a period not to exceed 6 months

 

If yes, this satisfies medical necessity and medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

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