Appeal type: Experimental/Investigational
Decision: Overturned Decision of Health Plan

Nature of Statutory Criteria/Case Summary: An enrollee has requested authorization and coverage for Gamunex-C immune globulin injection (IVIG). Dysautonomia is an umbrella term referring to any disorder associated with dysfunction of the autonomic nervous system. There are many different mechanisms by which dysautonomia may arise, but there has been increasing evidence and awareness that it may be immune-mediated in some patients. Autoimmune autonomic dysfunction may be peripheral due to an immune-mediated small fiber polyneuropathy (SFPN) and may manifest clinically as postural tachycardia syndrome, orthostatic intolerance, orthostatic hypotension, inappropriate sinus tachycardia, gastrointestinal dysmotility, complex regional pain syndrome, and/or neurogenic bladder. Schofield and Chemali note that IVIG is a first-line treatment for immune-mediated neuropathies such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. In an evaluation of the efficacy of immune modulatory therapy in children with SFPN, Oaklander and Klein reported that out of 15 patients given a trial of immune modulatory therapy with IVIG and/or steroids, 12 patients, or 80%, demonstrated improvement. In a retrospective analysis on the efficacy and safety of IVIG in patients with disabling, refractory autoimmune dysautonomias, including patients with postural tachycardia syndrome and gastrointestinal dysmotility, Schofield and Chemali reported that 83.5% improved on IVIG as defined by at least a twenty percent improvement in the composite autonomic symptom scale 31 and/or functional ability score. The authors concluded, There is increasing evidence that IVIG is safe and effective in a subset of patients with autonomic disorders and evidence for autoimmunity. A four-month IVIG trial should be considered in severely affected patients who are refractory to lifestyle and pharmacological therapies. In this case, the patient has an autoimmune autonomic nervous system dysfunction and has tried and failed multiple treatments. Based on current medical literature, patients with dysautonomia may improve clinically with IVIG treatment. Therefore, Gamunex-C IVIG is likely to be more beneficial for treatment of the patients condition than any available standard therapy.Findings:  Three of the three physician reviewers found the requested Gamunex-C IVIG is likely to be more beneficial for treatment of the patients medical condition than any available standard therapy.Final Result: The reviewers determined that the requested service is likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. Therefore, the Health Plans denial should be overturned. Credentials/Qualifications: Physician reviewer 1 is board certified in neurology and is actively practicing. Physician reviewers 2 and 3 are board certified in neurology with sub-specialty certification in vascular neurology and are actively practicing. The reviewers are experts in the treatment of the enrollees medical condition and knowledgeable about the proposed treatment through recent or current actual clinical experience treating those with the same or a similar medical condition.