Appeal type: Formulary Exception
Decision: Overturned

Diagnosis: Recurrent Pericarditis. 
Treatment: Formulary exception for Arcalyst. 

The insurer denied: Formulary exception for Arcalyst. 
The denial is overturned. 

This patient is an adult female with history of recurrent pericarditis, Behcet's disease and inflammatory polyarthropathy.  The patient has been receiving Arcalyst once weekly with partial response. Per the health plan, the request for Arcalyst for continuation of therapy for recurrent pericarditis is denied. 

The health plan should cover the proposed treatment. 

The term recurrent pericarditis refers to a syndrome in which acute pericarditis recurs after the agent inciting the original acute attack has disappeared or has ceased to be active. Cases with persistent symptoms are considered chronic pericarditis.  Most cases are considered to be autoimmune. 

The 2015 European Society of Cardiology guidelines recommend considering azathioprine or human intravenous immunoglobulin (IVIG) or anakinra.  Emerging evidence supports the use of anakinra especially in corticosteroid-dependent and colchicine resistant patients, in whom clinicians are unable to withdraw corticosteroids and/or colchicine without a recurrence.  In the AIRTRIP trial, 21 patients with at least three episodes of recurrent pericarditis (who also had elevated C-reactive protein levels, colchicine resistance, and glucocorticoid dependence) were treated with anakinra for two months. After two months and following resolution of pericarditis symptoms, patients were randomized in a double-blind fashion to continue anakinra or switch to placebo for six months or until symptoms recurred. After a median follow-up of 14 months, patients in the anakinra group had significantly fewer recurrences (18 versus 90 percent) and greater symptom-free survival compared with patients receiving placebo. However, 20 of 21 patients receiving anakinra experienced a side effect compared with none of the patients receiving placebo. Patients treated with anakinra were able to wean off of corticosteroids. 

Anakinra is an interleukin (IL) receptor antagonist that works by blocking the biological activity of interleukin-one (IL-1) by competitively inhibiting binding of interleukin-one (IL-1) to the type one interleukin receptor. Interleukin-one (IL-1) production is induced in response to inflammatory stimuli and mediates various physiological mechanisms, including inflammation and immunological reactions.  Arcalyst an interleukin-one (IL-1) trap cytokine inhibitor, has been used effectively in the treatment of many diseases. Arcalyst utilizes cytokine trap technology, which allows for effective inhibition due to multicomponent "traps" containing binding sites for the cytokine as well as their accessory proteins. Cytokine traps have been very effective in autoinflammatory diseases because they bind to their respective cytokines with high affinity. The drug has a half-life of 8.6 days, which makes weekly injections an improvement over daily injection of anakinra. 

This patient uses colchicine as needed. While clinical notes do not clearly indicate that the patient has previously failed colchicine and non-steroidal antiinflammaotry drugs (NSAIDs), the patient was already started on Arcalyst and has had partial clinical improvement according to notes. These medications are unlikely to be clinically effective at this time given the patient has had to use Arcalyst. The provider is wanting to increase Arcalyst twice weekly to better control the patient's symptoms. While clinical notes do not indicate how the patient has clinically improved with Arcalyst, it does indicate there has been some clinical response to treatment.  Inability to control her chronic pericarditis could result in constrictive pericarditis and need for cardiac surgery or hospitalizations and possible death. Therefore, the health plan should cover Arcalyst for continuation of therapy.