Appeal type: Experimental/Investigational
Decision: Overturned

Diagnosis: Bilateral Chorioretinal Inflammation And Bilateral Panuveitis Of The Eye
Treatment: Intravenous Immunoglobulin (IVIG) Code J1569
The insurer denied the Intravenous Immunoglobulin (IVIG) Code J1569.
The denial is upheld. 

The patient is a female with the diagnosis of autoimmune retinopathy, for whom request is made for coverage of infusion of IVIG (intravenous immunoglobulin) for six months.  

The provider letter states the patient is having loss of peripheral vision is progressive; Provider indicates the patient is having side-effects on corticosteroids, failed Humira, methotrexate, and Cellcept; Azathioprine higher dose is causing side-effects.  Local steroid treatment has been another option, but difficult; other options include Rituximab and Actemra

No, the health plans should not cover the treatment with IVIG (intravenous immunoglobulin) Therapy (J1569).

Autoimmune retinopathies (AIRs) are a group of inflammatory-mediated diseases characterized by the presence of antiretinal antibodies (ARAs), visual field deficits, and photoreceptor dysfunction in the setting of progressive unexplained vision loss. The AIRs (autoimmune retinopathies) encompass the better-studied paraneoplastic syndromes, such as cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), and a larger group of AIRs (autoimmune retinopathies) that have similar clinical and immunologic features but that are not associated with an underlying malignancy. The term non-paraneoplastic autoimmune retinopathy
(npAIR) was coined to describe this latter group, which has previously been called recoverin-associated retinopathy, autoimmune retinopathy in the absence of cancer, or, simply, autoimmune retinopathy.

There are no standard guidelines for the management of AIR (autoimmune retinopathy). The rarity of AIRs (autoimmune retinopathies), and the difficulty in firmly establishing the diagnosis, has limited investigations that evaluate treatment outcomes. A review of the literature shows that treatment results are found primarily as case reports and a few small case series.

According to Grange et al, common approaches to both paraneoplastic and non-paraneoplastic autoimmune retinopathy include systemic or local corticosteroids, intravenous immunoglobulin or plasmapheresis. Additionally, antimetabolites, such as mycophenolate mofetil and azathioprine, and T-cell inhibitors such as cyclosporine have been used. Less frequently, targeted B-cell therapy, such as anti-CD20 (cluster of differentiation 20) monoclonal antibody (rituximab), has also been used. Unfortunately, therapy is not helpful once widespread retinal degeneration has occurred.

Reflecting standard of care, Medicare will provide coverage for intravenous immune globulin when it is used in treatment of the following conditions:
1)	Primary immunodeficiency.
2)	Immune-mediated Thrombocytopenia (ITP).
3)	Kawasaki disease.
4)	Human Immunodeficiency Virus (HIV) (for pediatric use only).
5)	Bone marrow transplantation.
6)	Chronic B-cell lymphocytic leukemia.

The patient's diagnosis does not fall within the standard of care for generally accepted indications for the use of IVIG. The literature involving the use of IVIG in this situation is not robust, and it does not support that it is likely to be more beneficial than other treatment options. Therefore, the use of IVIG in this patient's situation would be considered to be experimental/investigational. As the use of IVIG (intravenous immunoglobulin) is experimental/investigational, its use for the treatment of this patient is not established as medically appropriate.

No, treatment with IVIG (intravenous immunoglobulin) is not likely to be more beneficial than other treatment options, which include Rituximab or Actemra.  

The current available medical literature does not support IVIG (intravenous immunoglobulin) as being superior to other immunomodulatory therapy.