Rilonacept

ARCALYST (rilonacept) is an interleukin-1 blocker indicated for: Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome (FCAS), and Muckle-Wells Syndrome (MWS) in adults and children 12 years and older. Maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric patients weighing 10 kg or more. Treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in adults and children 12 years and older

Rilonacept is an interleukin-1 alpha (IL-1α) and interleukin-1 beta (IL-1β) cytokine trap. Rilonacept blocks IL-1 signaling by acting as a soluble decoy receptor that binds both IL-1α and IL-1β and prevents its interaction with cell surface receptors. Rilonacept also binds interleukin-1 receptor antagonist (IL-1ra). The equilibrium dissociation constants for rilonacept binding to IL-1α, IL-1β, and IL-1ra were 1.4 pM, 0.5 pM, and 6.1 pM, respectively. CAPS refers to rare genetic syndromes generally caused by mutations in the NLRP-3 [nucleotide-binding domain, leucine rich family (NLR), pyrin domain containing 3] gene (also known as Cold-Induced Autoinflammatory Syndrome-1 [ CIAS1 ]). CAPS disorders are inherited in an autosomal dominant pattern with male and female offspring equally affected.

Administer by subcutaneous injection. CAPS, FCAS, MWS, and RP: Adults: – Loading dose: 320 mg, delivered as two 160 mg (2 mL) injections. – Maintenance dose: 160 mg (2 mL) injection once weekly. Pediatric patients 12 years to 17 years: – Loading dose: 4.4 mg/kg, up to a maximum of 320 mg, delivered as 1 or 2 injections (not to exceed 2 mL/injection). – Maintenance dose: 2.2 mg/kg, up to a maximum of 160 mg (2 mL) injection, once weekly. DIRA: Adults and pediatric patients weighing 10 kg or more: – 4.4 mg/kg up to a maximum of 320 mg, delivered as 1 or 2 injections (2 mL/injection) once weekly.

None. None.

Serious Infections: Serious, life-threatening infections have been reported in patients taking ARCALYST. Do not initiate treatment with ARCALYST in patients with active or chronic infections. Discontinue treatment if a patient develops a serious infection. Hypersensitivity Reactions: If a hypersensitivity reaction occurs, discontinue administration of ARCALYST and initiate appropriate therapy. Immunizations: Avoid live vaccines. Update recommended vaccinations prior to initiation of therapy per current guidelines.

The following clinically significant adverse reactions are described elsewhere in the labeling. Serious Infections Risk of Malignancy Hypersensitivity Reactions Lipid Profile Changes The most common adverse reactions reported by patients with CAPS and RP treated with ARCALYST are injection-site reactions and upper respiratory tract infections.

TNF-Blocking Agent and IL-1 Blocking Agent Specific drug interaction studies have not been conducted with ARCALYST. Concomitant administration of another drug that blocks IL-1 with a TNF-blocking agent in another patient population has been associated with an increased risk of serious infections and an increased risk of neutropenia. The concomitant administration of ARCALYST with TNF-blocking agents may also result in similar toxicities and is not recommended. The concomitant administration of ARCALYST with other drugs that block IL-1 has not been studied. Based upon the potential for pharmacologic interactions between rilonacept and a recombinant IL-1ra, concomitant administration of ARCALYST and other agents that block IL-1 or its receptors is not recommended.

Store refrigerated at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light. After reconstitution, ARCALYST may be kept at room temperature, protected from light.