Hizentra denials in California external review

What the reviewers wrote

Where the denial was overturned

Nature of Statutory Criteria/Case Summary: The patient has requested reimbursement and prospective authorization and coverage for Hizentra home infusions (13 grams administered by a nurse), (12 grams administered by a nurse), and (25 grams self-administered), and future for Hizentra 25 gram self-administered home infusions every 14 days for one year. Dysautonomia refers 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).

Findings: The physician reviewer found that Nature of Statutory Criteria/Case Summary: The patient requested authorization and coverage for Hizentra, for subcutaneous immunoglobulin (SCIG) treatment.Immunoglobulin therapy has had a major impact on the successful treatment of previously untreatable or poorly controlled autoimmune neurological disorders. The U.S. Food and Drug Administration has approved Hizentra for the treatment of primary immunodeficiency and chronic inflammatory demyelinating polyneuropathy. Immunoglobulin therapy has been used increasingly with significant efficacy in the treatment of patients with disabling autoimmune forms of dysautonomia, which are most often small fiber autonomic and/or sensory polyneuropathies. There is substantial medical literature documenting the benefit of SCIG for the treatment of AAN, SFN, and POTS.

Where the denial was upheld

Nature of Statutory Criteria/Case Summary: The patient has requested authorization and coverage for subcutaneous immunoglobulin (IVIG) therapy with Hizentra. Findings: The physician reviewer found that In a recent review of patients with small fiber neuropathy, Basantsova and colleagues noted that small fiber neuropathy is a disease highlighted by severe burning pain, sometimes induced by skin stimulation, and/or sensory symptoms of numbness, allodynia, and burning pain in the limbs. On review of the records provided for review, this patient does have these reported clinical symptoms. Novak and colleagues reported that mast cell activation syndrome patients can have small fiber neuropathy symptoms on biopsy and testing. Moreover, treatment with IVIG remains controversial in this patient’s clinical setting.

Findings: The physician reviewer found that Nature of Statutory Criteria/Case Summary: The patient has requested authorization and coverage for Hizentra subcutaneous immunoglobulin injection. The records reflect that this patient has been diagnosed with immunoglobulin G subclass deficiency (IgGSD). Few controlled studies have addressed immunoglobulin replacement therapy in cases of isolated subclass deficiency. One study found that immunoglobulin replacement therapy is indicated for patients with significantly impaired antibody production. The records indicate that this patient does not have impaired antibody production. Furthermore, the medical literature recommends the use of prophylactic antibiotics for the management of IgGSD. The records provided do not show that this patient has been treated with prophylactic antibiotics.

These outcomes describe eligible cases completed through California DMHC’s Independent Medical Review program. They do not estimate the chance that an internal appeal, an external review in another jurisdiction, or your individual case will succeed. Use the record to identify evidence patterns involving Hizentra, then check the rights and deadlines that apply to your plan.