Appeal type: Experimental/Investigational
Decision: Overturned

Diagnosis: Neuropathy, biopsy proven Sjogren's Syndrome, 
POTS, Rheumatoid Arthritis and Mast Cell Activation Syndrome
Treatment: Octagam Injection (J1568) Immune Globulin (IVIG)
The insurer denied: Octagam Injection (J1568) Immune Globulin 
(IVIG)
The denial is overturned.

This patient is an adult female with history Sjogren's disease with small fiber neuropathy. She also has positional orthostatic tachycardia syndrome (POTS), rheumatoid arthritis and mast cell activation syndrome. The patient has previously tried and failed biologic disease modifying anti-rheumatic drugs (DMARDs) Humira and Orencia along with conventional disease modifying anti-rheumatic drug hydroxychloroquine. The request for Octagam was initially denied due to policy considering it experimental, investigational or unproven for prevention of 
infections or for R68.89 other general symptoms. The reason the provider is appealing the determination is because clinical studies do support the use of intravenous immunoglobulin (IVIG) in the treatment of small fiber neuropathy related to Sjogren's.

The health plan should cover the proposed treatment.

Primary Sjogren's syndrome (pSS) is an autoimmune connective tissue disease affecting the exocrine glands, leading to damage of their structure and impairment of their function. In the course of Primary Sjogren's syndrome the internal organs may be involved, and the symptoms may concern any system. Neurological disorders are one of the most common extraglandular manifestations of Primary Sjogren's syndrome. Available literature data estimate the prevalence of neurological symptoms as about 8.570% of patients diagnosed with Primary Sjogren's syndrome. The most common neurological complication of Primary Sjogren's syndrome is peripheral neuropathy, and in particular sensory polyneuropathy. The reported neuropathies in Primary Sjogren's syndrome included distal sensory polyneuropathy, axonal sensorimotor polyneuropathy, chronic inflammatory demyelinating polyneuropathy (CIDP), multiple mononeuropathy, sensory neuronopathy and small fiber neuropathy. One of the main mechanisms for development of neuropathies in Primary Sjogren's syndrome is inflammation of the vessel supplying blood to the nerves, in some cases of necrotic nature, with perivascular inflammatory infiltration. An exception is sensory ganglionopathy related mainly to lymphocytic infiltration around the dorsal root ganglia. The role of antibodies in development of neuropathy has not been proven. 

Based on the patient's clinical symptoms, testing and severity, the health plan should cover the proposed treatment.

Treatment of small-fiber neuropathy is aimed initially at symptomatic relief of the associated pain. First-line therapeutic agents include gabapentin and pregabalin. Serotonin-norepinephrine reuptake inhibitors (SNRIs) such as duloxetine or venlafaxine may be substituted or added to first-line therapy if pain control is inadequate. Tri-cyclic antidepressants (TCA) may also be effective for treatment of neuropathic pain. A trial of immunomodulatory or immunosuppressive therapy is appropriate in patients in whom sensory deficits are rapidly progressive or severe or in those who have failed symptomatic therapy. Intravenous immunoglobulin (IVIG), in a dose of 0.4 grams per kilograms for 5 days, is generally recommended, based on beneficial results in anecdotal case reports and several small series, although duration of therapy is not clear. Small fiber neuropathies may not respond to corticosteroid therapy; however, this observation is limited by the coexistence of multiple forms of peripheral neuropathy in the studied populations. Small studies evaluating rituximab in painful sensory neuropathy have had disappointing results.