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.

The patient is an adult female with biopsy-proven Sjogren's syndrome (SS), rheumatoid arthritis (RA), bilateral lower extremity complex regional pain syndrome, and biopsy-proven small fiber neuropathy with worsening neuropathic symptoms. Small fiber neuropathy was diagnosed via skin biopsy 6 years ago, and electromyogram (EMG)/nerve conduction studies 2 years ago. She has painful paresthesia in her lower extremities, lost reflexes in her left leg, and developed bilateral foot drop 4 years ago. She has an unstable gait and occasional uses a wheelchair. For rheumatoid arthritis (RA), she failed treatment with hydroxychloroquine, and developed pneumonia with sepsis when treated with Humira. Humira was stopped earlier this year, and she began treatment with Orencia 2 months ago. Medications for her neuropathic symptoms include Lyrica 150 milligrams (mg) three times daily, tramadol, and medical cannabis. Per medication review, she has also failed treatment with Cymbalta and steroids. The attending provider (AP) asserts that the patient has an inflammatory small fiber neuropathy related to Sjogren's syndrome (SS) and wishes to pursue treatment with Octagam Injection (J1568) Immune Globulin (IVIG). The attending provider (AP) wishes to avoid additional immunosuppressive therapy due to her history of sepsis with Humira. Octagam was denied for this patient because it is deemed experimental and investigational for the treatment of small fiber neuropathy as per policy. The appeal is for Octagam intravenous immunoglobulin (IVIG) for the treatment of inflammatory small fiber neuropathy associated with Sjogren's syndrome (SS).

The health plan should cover Octagam intravenous immunoglobulin for the treatment of small fiber neuropathy related to Sjogren's syndrome (SS) for this patient.

This patient with biopsy-proven Sjogren's syndrome (SS), rheumatoid arthritis (RA), bilateral lower extremity complex regional pain syndrome, and biopsy-proven small fiber neuropathy with worsening neuropathic symptoms has failed multiple treatments. Her neuropathy has not responded to immunomodulatory treatment for rheumatoid arthritis (RA), including hydroxychloroquine, Humira and Orencia. The attending physician (AP) wishes to avoid additional immunosuppression due to the patient's history of pneumonia and sepsis on Humira. The patient has additionally failed treatment with Lyrica, tramadol, Cymbalta, steroids and 
medical cannabis. Per a recent UpToDate review, "IVIG has been reported to be effective for severe or treatment-refractory cases of small fiber neuropathy associated with SS in small series and reports." Prednisone is generally ineffective. "Small studies evaluating rituximab in painful sensory neuropathy have had disappointing results". To the contrary, a 2021 double-blind 
randomized controlled trial evaluating the efficacy and safety of Immune Globulin (IVIG) versus placebo in 60 Dutch patients with idiopathic small fiber neuropathy showed that Immune Globulin (IVIG) treatment had no significant effect on pain in these patients. However, these patients did not have Sjogren's syndrome (SS). Given the successful use of Immune Globulin 
(IVIG) for other inflammatory polyneuropathies, the encouraging data for small fiber neuropathy associated with Sjogren's syndrome (SS), and this patient's lack of response to other standard therapies, the health plan should cover a trial of Octagam Immune Globulin (IVIG) for this patient for six months.