The interleukin-1 (IL-1) pathway is the target of multiple biologic medications used in autoimmune and autoinflammatory diseases. The three available IL-1 antagonists include anakinra (IL-1 receptor antagonist), rilonacept (soluble IL-1 receptor decoy), and C (long-acting, fully human IgG1 anti-Il-1β (beta) monoclonal antibody). Due to their markedly different half-lives, anakinra is given as a daily subcutaneous injection, whereas rilonacept is given via weekly subcutaneous injection and canakinumab is given subcutaneously every 8 weeks. Anakinra has been an effective treatment for patients with RA, SJIA, and polyarticular JIA [13, 14]. In patients with periodic fever syndromes, anakinra has been used in patients with FMF who are nonresponders to colchicine as well as patients with TRAPS, the deficiency of IL-1 receptor antagonist (DIRA), and hyper-IgD syndrome (HIDS) and those with one of the cryopyrinopathies [familial cold autoinflammatory syndrome (FCAS), Muckle–Wells syndrome (MWS), and neonatal onset multisystem autoinflammatory disease/chronic infantile neurologic cutaneous and articular syndrome (NOMID/CINCA)] [15–22]. The longer acting rilonacept and canakinumab have been approved for use in patients with FCAS and MWS and are also being used in NOMID patients [21–26]. Regarding patients with AA amyloidosis, the various IL-1 inhibitors have been successful at slowing the progression of and, in some cases, treatment results in regression of amyloid-associated proteinuria [15, 26–30].

A newer therapy for the treatment of RA and SJIA is tocilizumab, the humanized anti-human IL-6 receptor antibody. Preliminary evidence has shown efficacy in treating both RA- and SJIA-associated AA amyloidosis [31, 32]. Currently ongoing is an international trial using the molecule, eprodisate disodium, in patients with AA amyloidosis-associated nephropathy. By binding the amyloidogenic precursor proteins, eprodisate disodium attempts to prevent the deposition of amyloid in organs, hence preserving renal function. For more information regarding ongoing clinical trials involving these medications for the diseases discussed above, the website: www.​clinicaltrials.​gov maintains a database of federally and privately supported clinical trials conducted worldwide.