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The rules say that plans must pay for a medical service or item if regular Medicare would pay for it in this case. 
You can find this rule at 42 Code of Federal Regulations (CFR) Section 422.101. 
 
The rules state that this LCD addresses off-label uses for IVIG. We define off-label as not in Medicare 
approved compendia or in the FDA label. 
 
NGS has approved IVIG for the following off label uses: 
 
    Stiff-person syndrome may be treated with IVIG when/if standard treatment with Diazepam is no longer 
effective. IVIG therapy is considered medically necessary for stiff-person syndrome when the following 
criteria are met: 
        Diagnosis has been confirmed by anti-glutamic acid decarboxylase (GAD) antibody testing; and 
        Member had an inadequate response to first-line treatment (benzodiazepines and/or baclofen). 
    Autoimmune retinopathy (AIR) that is sight threatening and refractory to corticosteroid and 
immunosuppressant therapy 
        Induction Dose - 1.5g/kg in divided dose over 3 days. 
        Maintenance Dose - 0.4 to 1.5 g/kg in single or divided doses monthly. 
        The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each 
patient. 
    Pure red cell aplasia related to human parvovirus B19 infection - IVIG therapy is considered medically 
necessary for severe, refractory anemia associated with bone marrow suppression, with parvovirus B19 
viremia. 
    Hematopoetic Stem Cell Transplantation (HSCT) - IVIG is indicated to prevent the risk of acute graft-
versus-host disease, associated interstitial pneumonia (infectious or idiopathic) and infections (e.g., 
cytomegalovirus infections [CMV], varicella-zoster virus infection, and recurrent bacterial infection) after 
HSCT in patients 20 years of age or older during the first 100 days after transplantation. It is not indicated in 
HSCT patients younger than 20 years of age, nor is it recommended for autologous transplants. The evidence is 
sufficient to conclude that the complications of stem cell transplantation are independent of the source of stem 
cells. 
 
        Dosage guideline is 500 mg/kg body weight given on days 7 and 2 pre-transplantation, then weekly 
through day 90 post-transplantation.     Secondary hypogammaglobulinemia - there is an association between the administration of certain 
pharmaceuticals and the development of hypogammaglobulinemia. 
    Chronic Graft versus Host Disease (GVHD) IVIG is indicated in patient with chronic GVHD who meet all 
of the following criteria: 
 
        Laboratory proven hypogammaglobulinemia with IgG levels <400 mg/dl. 
        At least one acute infection requiring hospitalization and/or parenteral antibiotics 
        Confirmed diagnosis of chronic GVHD 
        At least 100 days post transplant. 
    Hypogammaglobulinemia for Graft vs. Host Disease (GVHD)- 
        There is sufficient evidence to conclude that patients who require ongoing immunosuppression for the 
treatment of chronic GVHD are at increased risk when compared to their peers who do not require such 
treatment. 
        There is sufficient evidence that such patients who are hypogammaglobulinemic (IgG < 400 mg/d) and 
have experienced one serious infection may benefit from treatment of IVIG 
        There is insufficient evidence that prophylactic treatment of HSCT patients with IVIG provides a benefit 
in the absence of hypogammaglobulinemia 
 
    Systemic Lupus Erythematosus - The routine use of IVIG is not usually recommended. IVIG may be used in 
patients with severe active systemic lupus erythematosus for whom other interventions have been unsuccessful, 
have become intolerable or are contraindicated. 
    Scleromyxedema -Systemic therapy is the treatment method of choice for patients with scleromyxedema. 
Intravenous immunoglobulin (IVIG) is a first choice for therapy based upon multiple case reports and case 
series that support its efficacy and the generally well-tolerated nature of this nonimmunosuppressive treatment. 
 
The mechanism through which IVIG improves scleromyxedema is unclear. Suggested mechanisms focus on 
the immunomodulatory effects of IVIG, including neutralization of circulating autoantibodies by anti-idiotype 
antibodies, functional blockade of fragment crystallizable receptors on macrophages, and inhibition of fibrosis 
via modulation of the production of cytokines and cytokine antagonists. 
 
IVIG is usually administered at the dose of 2 g/kg per month divided over two to four consecutive days per 
month according to the preparation and concentration of IVIG. Improvement in skin and extracutaneous 
symptoms, especially rheumatologic symptoms, often is evident after the first one or two cycles of IVIG. 
Almost all patients exhibit at least partial improvement within four to six cycles. Patients with an unsatisfactory 
response to IVIG after six cycles are typically transitioned to other therapies. 
 
Lower doses of IVIG may also be effective. A patient with skin-limited disease who had failed to respond to 
systemic glucocorticoids, extracorporeal photophoresis, and interferon had a reduction in clinical findings 
within two cycles of IVIG given at a dose of 0.5 g/kg given over five days at four-week intervals. 
 
Although remissions persisting for a few months to three years after cessation of IVIG infusions have been 
reported, the response to IVIG is usually transient. Maintenance IVIG cycles every six to eight weeks are 
generally required to maintain remission. IVIG is typically administered over two to four days at a dose of 2 
g/kg of IVIG every six weeks or 1.5 g/kg of IVIG every four weeks. 
 
    Systemic Capillary Leak Syndrome (SCLS) or Clarksons Disease-  Systemic Capillary Leak Syndrome is a 
rare illness of unknown origin which has been reported through registries, case studies and case series. Due to 
the rarity of the illness, large studies are not expected to be generated. Diagnosis in the most recent review and 
registry review is associated with monoclonal gammopathy. Prophylaxis with IVIG given on a routine monthly 
basis has been associated with increased survival. This monthly prophylaxis should be tapered to the lowest 
effective dose. Medicare is expanding coverage for this illness on a trial basis when associated with 
monoclonal gammopathy and used for prophylaxis but can be withdrawn or altered based on subsequent literature. All other claims will have the appeals process for potential coverage where medical documentation 
and submitted literature can be reviewed for individual consideration. 
 
The dose used in most case reports has been 2 grams per kg intravenously per month, although 1 gram per kg 
per month was noted to be effective in another report. 
 
    Immune-mediated Necrotizing Myositis (IMNM) 
 
IVIG plays a critical role in the medication management of this disease. IVIG is also a critical therapeutic 
option for the management of other myositis syndromes, and was FDA-approved 7/15/2021 for the 
management of dermatomyositis. 
 
You can find this information in the Local Coverage Determination (LCD) Off-Label Use of Intravenous 
Immune Globulin (IVIG) L39314. 
 
If you want to read these Medicare rules, you can go to this website www.medicareappeal.com.