Local Coverage Determination (LCD)

Intravenous Immunoglobulin (IVIG)

L34580

Expand All | Collapse All

Contractor Information

LCD Information

Document Information

LCD ID
L34580
LCD Title
Intravenous Immunoglobulin (IVIG)
Proposed LCD in Comment Period
N/A
Source Proposed LCD
DL34580
Original Effective Date
For services performed on or after 10/01/2015
Revision Effective Date
For services performed on or after 07/07/2022
Revision Ending Date
N/A
Retirement Date
N/A
Notice Period Start Date
08/20/2020
Notice Period End Date
10/03/2020
AMA CPT / ADA CDT / AHA NUBC Copyright Statement

CPT codes, descriptions and other data only are copyright 2022 American Medical Association. All Rights Reserved. Applicable FARS/HHSARS apply.

Fee schedules, relative value units, conversion factors and/or related components are not assigned by the AMA, are not part of CPT, and the AMA is not recommending their use. The AMA does not directly or indirectly practice medicine or dispense medical services. The AMA assumes no liability for data contained or not contained herein.

Current Dental Terminology © 2022 American Dental Association. All rights reserved.

Copyright © 2022, the American Hospital Association, Chicago, Illinois. Reproduced with permission. No portion of the American Hospital Association (AHA) copyrighted materials contained within this publication may be copied without the express written consent of the AHA. AHA copyrighted materials including the UB‐04 codes and descriptions may not be removed, copied, or utilized within any software, product, service, solution or derivative work without the written consent of the AHA. If an entity wishes to utilize any AHA materials, please contact the AHA at 312‐893‐6816.

Making copies or utilizing the content of the UB‐04 Manual, including the codes and/or descriptions, for internal purposes, resale and/or to be used in any product or publication; creating any modified or derivative work of the UB‐04 Manual and/or codes and descriptions; and/or making any commercial use of UB‐04 Manual or any portion thereof, including the codes and/or descriptions, is only authorized with an express license from the American Hospital Association. The American Hospital Association (the "AHA") has not reviewed, and is not responsible for, the completeness or accuracy of any information contained in this material, nor was the AHA or any of its affiliates, involved in the preparation of this material, or the analysis of information provided in the material. The views and/or positions presented in the material do not necessarily represent the views of the AHA. CMS and its products and services are not endorsed by the AHA or any of its affiliates.

Issue

Issue Description

This LCD outlines limited coverage for this service with specific details under Coverage Indications, Limitations and/or Medical Necessity.

CMS National Coverage Policy

Title XVIII of the Social Security Act §1862(a)(1)(A) allows coverage and payment for only those services that are considered to be reasonable and necessary for the diagnosis or treatment of illness or injury or to improve the functioning of a malformed body member.

42 Code of Federal Register §411.15(a)(k), Particular services excluded from coverage.

CMS Internet-Only Manual, Pub. 100-02, Medicare Benefit Policy Manual, Chapter 6, §20.5.1, Coverage of Outpatient Therapeutic Services Incident to a Physician’s Service Furnished on or After August 1, 2000, and Before January 1, 2010.

CMS Internet-Only Manual, Pub. 100-02, Medicare Benefit Policy Manual, Chapter 8, §50.5, Drugs and Biologicals.

CMS Internet-Only Manual, Pub. 100-02, Medicare Benefit Policy Manual, Chapter 13, §110, Physician Services, and §120, Services and Supplies Furnished “Incident to” Physician’s Services.

CMS Internet-Only Manual, Pub. 100-02, Medicare Benefit Policy Manual, Chapter 15, §§50, 50.1, 50.2, 50.3, 50.4.1, 50.4.2, 50.4.3, 50.4.5, §50.4.6, Drugs and Biologicals, and §200, Nurse Practitioner (NP) Services.

CMS Internet-Only Manual, Pub. 100-03, Medicare National Coverage Determinations Manual, Chapter 1, Part 4, §250.3, Intravenous Immune Globulin for the Treatment of Autoimmune Mucocutaneous Blistering Diseases.

Coverage Guidance

Coverage Indications, Limitations, and/or Medical Necessity

Intravenous Immune Globulin (IVIG) is a solution of human immunoglobulins specifically prepared for intravenous infusion. Immunoglobulins contain a broad range of antibodies that act specifically against bacterial and viral antigens.

INDICATIONS:

  • The treatment of primary immunodeficiency syndromes associated with defects in humoral immunity to replace or boost immunoglobulin G (IgG)
  • The treatment of idiopathic thrombocytopenic purpura (ITP) when a rapid rise in the platelet count is required such as prior to surgery, to control excessive bleeding, or to defer or avoid splenectomy
  • Treatment of Kawasaki disease, in conjunction with aspirin
  • Prevention of recurrent bacterial infections in patients with hypogammaglobulinemia associated with B-cell chronic lymphocytic leukemia (CLL)
  • Decreasing risk of acute graft-vs.-host disease, associated interstitial pneumonia, and infections after bone marrow transplant in the first 100 days after transplantation
  • Reducing the risk of severe bacterial infections in human immunodeficiency virus (HIV) infected children with a CD4 count of greater than 200 to 400
  • Second-line treatment of certain autoimmune myopathies
  • The treatment of adults with Guillain-Barré syndrome diagnosed within the first 2 weeks of illness
  • Treatment of hyperimmunoglobulinemia E syndrome and Lambert-Eaton myasthenic syndrome (LEMS)
  • Treatment of multifocal motor neuropathy (MMN)
  • Treatment of relapsing-remitting multiple sclerosis
  • Treatment of chronic parvovirus B19 infection and severe anemia associated with bone marrow suppression and pure red cell aplasia
  • Treatment of progressive pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, and epidermolysis bullosa acquisita in patients that have failed conventional treatment and patients in whom conventional therapy is otherwise contraindicated
  • For coverage criteria of Autoimmune Mucocutaneous Blistering Diseases, please see CMS Internet-Only Manual, Pub. 100-03, Medicare National Coverage Determinations Manual, Chapter 1, Part 4, §250.3
  • Treatment of stiff person syndrome
  • Treatment of Myasthenia Gravis (MG) in patients who have profound, rapidly progressive and/or potentially life-threatening muscular weakness and are refractory to, or intolerant of cholinesterase inhibitors, corticosteroids and azathioprine
  • The prevention and/or treatment of organ rejection in patients sensitized to living or cadaveric organ donors
  • Schonlein-Henoch
  • Treatment of Paraneoplastic Visual Loss
  • Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) in patients meeting the necessary criteria
  • Treatment of Multiple Myeloma for the prevention of life-threatening infections due to reduced gamma globulins
Summary of Evidence

IVIG has been used for many years in the treatment of immunodeficiency disease. As the knowledge of these diseases has improved and expanded, the use of IVIG has proportionally grown. With this, the body of literature supporting the use of IVIG in these situations has equally grown. The literature supports the use of IVIG in acute situations such as prevention of infections in patients with solid organ and bone marrow transplants, myeloma, leukemia, and HIV infections as well as in the acquired and genetic immunodeficiency syndromes such as CIDP. Additionally, the use in blocking damage by toxic antibodies in Guillain-Barré syndrome, pemphigus and its variants has been shown to be effective.

Analysis of Evidence (Rationale for Determination)

IVIG is an important adjunct or primary treatment in multiple situations where immunoglobulin abnormalities cause complications because of decreased or toxic antibodies. As shown in the Bibliography, there is a significant literature base to support its use in these situations. This contractor will continue to monitor the literature for additional uses of IVIG as they develop.

General Information

Associated Information

Documentation Requirements

Documentation supporting the medical necessity should be legible, maintained in the patient's medical record, and must be made available to the A/B MAC upon request.

Utilization Guidelines

Paraneoplastic Visual Loss is an autoimmune disorder believed to be caused by the remote effects of cancer on the retina (cancer associated retinopathy (CARI) or optic nerve). Research has shown that the use of intravenous immunoglobulins for Paraneoplastic Visual Loss would be another treatment option.

Sources of Information

N/A

Bibliography
  1. Anderson D, Ali K, Blanchette V, et al. Guidelines on the use of intravenous immune globulin for hematologic conditions. Transfusion Medicine Reviews. 2007;21(2 Suppl 1):S9-S56.
  2. Bain PG, Motomura M, Newsom-Davis J, et al. Effects of intravenous immunoglobulin on muscle weakness and calcium-channel autoantibodies in the lambert-eaton myasthenic syndrome. Neurology. 1996;47:678-683.
  3. Balasubramanian SK, Sadaps M, Thota S, et al. Rational management approach to pure red cell aplasia. Haematologica. 2018;103(2):221-230.
  4. Chapel HM and Lee M. The use of intravenous immune globulin in multiple myeloma. Clin Exp Immunol. 1994;97(Suppl 1):21–24.
  5. Crabol Y, Terrier B, Rozenberg F, et al. Intravenous immunoglobulin therapy for pure red cell aplasia related to human parvovirus B19 infection: A retrospective study of 10 patients and review of the literature. Clinical Infectious Diseases. 2013;56(7):968-977.
  6. Donofrio PD, Berger A, Brannagan TH, et al. Consensus statement: The use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the AANEM ad hoc committee. Muscle Nerve. 2009;40(5):890-900.
  7. Elovaara I, Apostolski S, van Doorn P, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur Jour of Neurol. 2008;15(9):893-908.
  8. Gammaked™. Grifols Therapeutics; 2017. Accessed 5/25/22.
  9. Gamunex®-C. Grifols Therapeutics; 2003. Accessed 5/25/22.
  10. Guy J, Aptsiauri N. Treatment of paraneoplastic visual loss with intravenous immunoglobulin: Report of 3 cases. Arch Ophthalmol. 1999;117(4):471-477.
  11. John R, Lietz K, Burke E, et al. Intravenous immunoglobulin reduces anti-HLA alloreactivity and shortens waiting time to cardiac transplantation in highly sensitized left ventricular assist device recipients. Circulation. 1999;100(19 Suppl):II229-35.
  12. Jordan SC, Toyoda M, Kahwaji J, Vo AA. Clinical aspects of intravenous immunoglobulin use in solid organ transplant recipients. Am J Trans. 2011;11(2):196-202.
  13. Khalafallah A, Maiwald M, Cox A, et al. Effect of immunoglobulin therapy on the rate of infections in multiple myeloma patients undergoing autologous stem cell transplantation or treated with immunomodulatory agents. Medit J Hemat Infect Dis. 2010;2(1):Open Journal System.
  14. Kim IK, Vo A, Jordan SC. Transplantation in highly HLA sensitized patients: challenges and solutions. Transplantation Research and Risk Management. 2014;6:99-106.
  15. Latov N. Diagnosis of CIDP. Neurol. 2002;59(12 Suppl 6):S2-6.
  16. Leech SH, Lopez-Cepero M, LeFor WM, et al. Management of the sensitized cardiac recipient: The use of plasmapheresis and intravenous immunoglobulin. Clin Transplant. 2006;20(4):476-484.
  17. National Institute for Health and Clinical Excellence. Multiple sclerosis: Management of multiple sclerosis in primary and secondary care. NICE; 2014.
  18. National Comprehensive Cancer Network, NCCN Guidelines. Version 1. 2020.
  19. Orange JS, Hossny EM, Weiler CR, et al. Use of intravenous immunoglobulin in human disease: A review of evidence by members of the primary immunodeficiency committee of the American academy of allergy, asthma and immunology. J Allergy Clin Immunol. 2006;117(4):S525-53.
  20. Patwa HS, Chaudhry V, Katzberg H, Rae-Grant AD, So YT. Evidence-based guideline: Intravenous immunoglobulin in the treatment of neuromuscular disorders: Report of the therapeutics and technology assessment subcommittee of the American academy of neurology. Neurol. 2012;78(13):1009-1015.
  21. Pisani BA, Mullen GM, Malinowska K, et al. Plasmapheresis with intravenous immunoglobulin G is effective in patients with elevated panel reactive antibody prior to cardiac transplantation. J Heart Lung Transplant. 1999;18(7):701-706.
  22. Rajkumar SV, Kyle RA. Treatment of multiple myeloma: A comprehensive review. Clin Lymphoma Myeloma. 2009;9(4):278–288.
  23. Ross MA. Intravenous immunoglobulin therapy for neuromuscular disorders. Seminars in Neurol. 2007;27(4):340-346.
  24. Ruts L, Drenthen J, Jacobs BC, van Doorn PA. Distinguishing acute-onset CIDP from fluctuating guillain-barré syndrome: A prospective study. Neurol. 2010;74(21):1680-1686.
  25. Sander HW, Latov N. Research criteria for defining patients with CIDP. Neuro. 2003;60(8 Suppl 3):S8-15.
  26. The United States Pharmacopeia Drug Information (USPDI): Drug Information for the Health Care Professional. 26th ed. Greenwood Village, CO: Thomson Micromedex. 2006:1714-1720.
  27. U.S. Food and Drug Administration (FDA). Immune Globulin Intravenous (IGIV) Indications. Accessed 5/25/22.
  28. Van den Bergh PY, Hadden RD, Bouche P, et al. European federation of neurological societies/peripheral nerve society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force of the European federation of neurological societies and the peripheral nerve society-first revision. Eur J Neurol. 2010;17(3):356-363.
  29. Van Schaik IN, Winer JB, De Haan R, Vermeulen M. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: A systematic review. Lancet Neurology. 2002;1(8):491-498.
  30. Vita GK, Shin JO, Dimachkie MM, Barohn RJ. Lambert-eaton myasthenic syndrome. Neurol Clin. 2018;36(2):379-394.

Revision History Information

Revision History DateRevision History NumberRevision History ExplanationReasons for Change
07/07/2022 R20

Under CMS National Coverage Policy updated section headings for regulations. Under Coverage Indications, Limitations and/or Medical Necessity – Indications revised seventh bullet to read “Second-line treatment of certain autoimmune myopathies”.

  • Provider Education/Guidance
10/04/2020 R19

This LCD is being presented for notice. No comments were received during the comment period; therefore, no changes have been made.

At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination; and, therefore not all the fields included on the LCD are applicable as noted in this policy.

  • Provider Education/Guidance
10/10/2019 R18

This LCD is being revised in order to adhere to CMS requirements per chapter 13, section 13.5.1 of the Program Integrity Manual, to remove all coding from LCDs. There has been no change in coverage with this LCD revision. Regulations regarding billing and coding were removed from the CMS National Coverage Policy section of this LCD and placed in the related Billing and Coding: Intravenous Immunoglobulin (IVIG) A56718 article.

At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination; and, therefore not all the fields included on the LCD are applicable as noted in this policy.

  • Provider Education/Guidance
07/25/2019 R17

All coding located in the Coding Information section and all verbiage regarding billing and coding under the Associated Information section has been removed and is included in the related Billing and Coding: Intravenous Immunoglobulin (IVIG) A56718 article.

At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination; and, therefore not all the fields included on the LCD are applicable as noted in this policy.

  • Provider Education/Guidance
07/04/2019 R16

Under Bibliography changes were made to citations to reflect AMA citation guidelines. Formatting, punctuation and typographical errors were corrected throughout the LCD. Acronyms were inserted where appropriate throughout the LCD.

At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination; and, therefore not all the fields included on the LCD are applicable as noted in this policy.

  • Provider Education/Guidance
07/26/2018 R15

Under Coverage Indications, Limitations and/or Medical Necessity Initial Treatment revised the first sentence to now read “Diagnosis of chronic inflammatory demyelinating polyneuropathy as confirmed by all of the following (a through c):”  Under Coverage Indications, Limitations and/or Medical Necessity Initial Treatment “d” the verbiage was changed from “BOTH of the following findings on lumbar puncture18 i.White blood cell count less than 10/mm3” to now read “The following findings on lumbar puncture are not absolutely required but may support the diagnosis:18”. This revision is due to a reconsideration request.

At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination; and, therefore not all the fields included on the LCD are applicable as noted in this policy.

 

  • Reconsideration Request
05/31/2018 R14

Under CMS National Coverage Policy changed verbiage to now read “describes payment for services that may be furnished to a practitioner. Requests for payment, or bills submitted shall include the appropriate diagnosis code(s)” in the third regulation. The verbiage was changed from “must be reasonable and necessary” to now read “excluded from coverage” in the fourth regulation. The last two regulations were deleted from this section. Under Coverage Indications, Limitations and/or Medical Necessity - A. Initial treatment added the verbiage “American Academy of Neurology” in front of the acronym “AAN” and placed parentheses around the acronym. Under Bibliography changes were made to citations to reflect AMA citation guidelines. The initials of the third author was corrected to now read “van Doorn P” in the third reference. The reference date was changed from 2008 to 2014 in the fifth reference. The link was deleted and the title has changed to now read “Multiple sclerosis: management of multiple sclerosis in primary and secondary care. NICE; 2014” in the ninth reference. The access date was changed to May 21, 2018 in the sixteenth reference. Punctuation and typographical errors were corrected throughout the policy.

At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination; and, therefore not all the fields included on the LCD are applicable as noted in this policy.

  • Provider Education/Guidance
  • Typographical Error
02/26/2018 R13 The Jurisdiction "J" Part B Contracts for Alabama (10112), Georgia (10212) and Tennessee (10312) are now being serviced by Palmetto GBA. The notice period for this LCD begins on 12/14/17 and ends on 02/25/18. Effective 02/26/18, these three contract numbers are being added to this LCD. No coverage, coding or other substantive changes (beyond the addition of the 3 Part B contract numbers) have been completed in this revision.
  • Change in Affiliated Contract Numbers
01/29/2018 R12 The Jurisdiction "J" Part A Contracts for Alabama (10111), Georgia (10211) and Tennessee (10311) are now being serviced by Palmetto GBA. The notice period for this LCD begins on 12/14/17 and ends on 01/28/18. Effective 01/29/18, these three contract numbers are being added to this LCD. No coverage, coding or other substantive changes (beyond the addition of the 3 Part A contract numbers) have been completed in this revision.
  • Change in Affiliated Contract Numbers
12/14/2017 R11

Under CMS National Coverage Policy added CMS Internet-Only Manual, Pub 100-02, Medicare Benefit Policy Manual, Chapter 15, §50.4.6; Less Than Effective Drug.

  • Provider Education/Guidance
10/01/2017 R10

Under ICD-10 Codes That Support Medical Necessity added ICD-10 codes M33.03, M33.13 and M33.93. The code description was revised for M33.00, M33.01, M33.02, M33.09, M33.10, M33.11, M33.12 and M33.19. This revision is due to the 2017 Annual ICD-10 Updates.

At this time 21st Century Cures Act will apply to new and revised LCDs that restrict coverage which requires comment and notice. This revision is not a restriction to the coverage determination; and, therefore not all the fields included on the LCD are applicable as noted in this policy.

  • Provider Education/Guidance
  • Revisions Due To ICD-10-CM Code Changes
04/03/2017 R9 Under ICD-10 Codes That Support Medical Necessity Group 1: Codes added G61.82.
  • Provider Education/Guidance
  • Reconsideration Request
07/07/2016 R8 This LCD is being made an A/B MAC LCD. No coverage changes were made.
  • Provider Education/Guidance
  • Creation of Uniform LCDs Within a MAC Jurisdiction
01/04/2016 R7 The description changed for HCPCS codes J1459, J1557, J1561, J1566, J1568, J1569, J1572 and J1599 under the CPT/HCPCS Codes section.
  • Revisions Due To CPT/HCPCS Code Changes
01/04/2016 R6 Under Coverage Indications, Limitations and/or Medical Necessity added verbiage "for coverage criteria of Autoimmune Mucocutaneous Blistering Diseases, please see NCD 250.3".
Under ICD-10 Codes that Support Medical Necessity added L10.0, L10.1, L10.2, L10.3, L10.4, L10.5, L10.81, L10.89, L10.9, L12.0, L12.1, L12.8, L12.9, L13.8 for reference.
Under ICD-10 Codes that DO NOT Support Medical Necessity removed NOTE: as per CR 9252 Transmittal 1547 the bullous conditions are no longer covered effective January 4, 2016 as coverage for these codes is under NCD 250.3.
  • Provider Education/Guidance
  • NCD Supplementation
  • Typographical Error
01/04/2016 R5 Under CMS National Coverage Policy added reference to Change Request 9252 Transmittal 1547.
Under Coverage Indications, Limitations and/or Medical Necessity created a new section titled Off-label Indication, and added off-label coverage for Livedoid Vasculitis and Livedoid Vasculopathy for patients who are refractory to or have failed conventional therapies.
Under ICD-10 Codes that Support Medical Necessity removed L10.0-L13.8 as per CR 9252 Transmittal 1547 NCD 250.3 (these codes are no longer valid for processing Medicare claims) and added off-label coverage for L95.0 and L95.9.
Under ICD-10 Codes that DO NOT Support Medical Necessity added L10.0-L13.8 as per CR 9252 Transmittal 1547 the bullous conditions are no longer covered.
  • Provider Education/Guidance
  • Public Education/Guidance
  • Request for Coverage by a Provider (Part A)
  • Reconsideration Request
  • Other (Change Request 9252 updated NCD 250.3)
10/29/2015 R4 Under Coverage Indications, Limitations and/or Medical Necessity formatting changes were made. Under Associated Information, sub-heading Utilization Guidelines 2nd paragraph added a space between (CARI) and the word "or". Under Sources of Information and Basis for Decision added the assess dates the web-sited were reviewed.
  • Provider Education/Guidance
10/01/2015 R3 Under Coverage Indications, Limitations and/or Medical Necessity added updated coverage criteria for Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Under Revenue Codes removed the statement "Contractors may specify Revenue Codes to help providers identify those Revenue Codes typically used to report this service. In most instances Revenue Codes are purely advisory; unless specified in the policy services reported under other Revenue Codes are equally subject to this coverage determination. Complete absence of all Revenue Codes indicates that coverage is not influenced by Revenue Code and the policy should be assumed to apply equally to all Revenue Codes" as all revenue codes have been removed from policies. Under ICD-10 Codes that Support Medical Necessity removed L14 per NCD 250.3. Under Sources of Information and Basis for Decision added sources for CIDP coverage.
  • Provider Education/Guidance
  • Reconsideration Request
  • Other (Per CR 9252 NCD 250.3 removed L14. )
10/01/2015 R2 In Coverage Indications, Limitations and/or Medical Necessity made punctuation corrections. In Associated Information corrected Paraneoplastic spelling, removed “This is very rare” and added the sentence “Research has shown that the use of intravenous immunoglobulins for Paraneoplastic Visual Loss would be another treatment option.”
  • Provider Education/Guidance
  • Other (Annual Validation)
10/01/2015 R1 Under CMS National Coverage Policy removed a section from Publication 100-02, Chapter 15, section 50.2 as this reference was specific to Self-Administered Drugs and IVIG would never be self-administered.
  • Provider Education/Guidance

Associated Documents

Attachments
N/A
Related National Coverage Documents
N/A
Public Versions
Updated On Effective Dates Status
06/27/2022 07/07/2022 - N/A Currently in Effect You are here
08/14/2020 10/04/2020 - 07/06/2022 Superseded View
Some older versions have been archived. Please visit the MCD Archive Site to retrieve them.

Keywords

  • IVIG
  • Intravenous Immunoglobulin

Read the LCD Disclaimer