Diagnosis: Chronic autonomic dysfunction.
Treatment: Immunoglobulin (codes J1599 - Panzyga x19 visits).

The insurer denied the Immunoglobulin (codes J1599 - Panzyga x19 visits).
The denial is upheld.

The patient is a female with chronic autonomic dysfunction, with symptoms of severe peripheral neuropathy as well as headaches, and weakness in her arms, legs, and feet. The patient had some improvement with steroid treatment but she could no longer take steroids due to side effects of severe vomiting and headaches. She had also tried and failed several pain medications. The patient was recommended her provider to undergo intravenous immunoglobulin (IVIG) therapy (Panzyga). This was denied as not being medically necessary.

According to an office note by the neurologist, the patient has small fiber neuropathy and migraine headaches, with severe pain throughout her body and difficulty walking as well as fatigue and memory difficulties. The patient recently had an episode of severe headache and left-sided weakness. Imaging was unremarkable and she was given medication which caused her to see spots in her vision. An electroencephalogram (EEG) was ordered to rule out seizure and brain magnetic resonance image (MRI) was ordered to rule out a mass or vascular lesion. A repeat small fiber nerve biopsy was recommended, and intravenous immunoglobulin (IVIG) therapy was to be considered.

A sweat gland nerve fiber density study was consistent with small fiber neuropathy.

The provider noted in his letter that studies have shown if all other medical options have been exhausted, intravenous immunoglobulin (IVIG) is the next step in treatment.

No, the proposed treatment with intravenous immunoglobulin is not medically necessary.
Medically necessary means healthcare services provided for the prevention, evaluation, diagnosing, or treating an illness or injury and its symptoms. The services/treatments must be provided in accordance with generally accepted standards of medical practice and clinically appropriate, meaning credible scientific evidence published in peer-reviewed medical literature that is generally recognized by the relevant medical community.

There is no evidence that the patient's small fiber neuropathy is an inflammatory condition. There are no tests (ie, blood tests, spinal fluid tests) to support inflammation.

According to reference 4, "Small fiber neuropathy (SFN) is a prevalent neurologic syndrome. Testing methods have emerged in recent years to better diagnose it, including autonomic tests and skin punch biopsy. SFN (Small fiber neuropathy) can present in a non-length-dependent fashion and can be mistaken for syndromes such as fibromyalgia and complex regional pain syndrome. SFN (Small fiber neuropathy) is caused by a variety of metabolic, infectious, genetic, and inflammatory diseases. Recently treatments have emerged for TTR (transthyretin ) amyloid neuropathy and Fabry disease, and novel biomarkers have been found both in genetic and inflammatory SFN (Small fiber neuropathy) syndromes. Ongoing trials attempt to establish the efficacy of intravenous immunoglobulin in inflammatory SFN (Small fiber neuropathy) syndromes."

Since the patient's small fiber neuropathy (SFN) is not inflammatory, intravenous immunoglobulin (IVIG) is not in accordance with generally accepted standards of medical care and, as such, is not medically necessary.