Treatment of refractory adult-onset still’s disease with tocilizumab: report of two cases and review of the literature

Abstract

Adult-onset Still's disease (AOSD) is empirically treated with nonsteroidal anti-inflammatory drugs, corticosteroids, conventional disease-modifying antirheumatic drugs, tumor necrosis factor-blocking agents or anakinra. The monoclonal anti-interleukin (IL)-6 antibody tocilicumab (TOC) has recently been approved for the treatment of rheumatoid arthritis and may be an attractive therapeutic option for AOSD as well. We report two AOSD patients treated with TOC and review of the current data on the use of TOC in AOSD. TOC was applied to the first patient after failure of cloroquin, methotrexate, adalimumab and etanercept. The second patient received TOC because of inefficacious methotrexate treatment. TOC was well tolerated by both the patients, and no clinically significant side effects occurred. Including these two cases, a total of seven AOSD patients have been successfully treated with TOC so far. TOC may be a promising treatment option for AOSD patients refractory to conventional disease-modifying antirheumatic drugs anakinra and tumor necrosis factor-[Formula: see text].

Adult onset Still's disease: experience from a tertiary care rheumatology unit

“…[15][16][17][18][19] There are several recent reports of the successful use of various biologics like anakinra, etanercept, infliximab, adalimumab and tocilizumab in refractory cases of AOSD. [19][20][21] However, these are expensive, thus we used tocilizumab in one patient refractory to steroids and methotrexate with good response.…”

Anti-CD-20 Therapy in Refractory Adult Still’s Disease

“…Despite that the exact role in disease modification with anti-CD-20 therapy hasn't been surveyed in these patients yet evidences have shown that following B cell depletion with rituximab treatment, a decrease was observed for IL-1b, IL-1RA, IL-2R, IL-4, IL-5, IL12, IL-15, IL-17, TNF-α, GM-CSF for 24 weeks with partial recovery over a period of 24 -48 weeks which suggests that B cell depletion might down regulate the release of T cell mediated pro-inflammatory cytokine release [9][10][11][12][13]. Considering that the exact pathogenesis of ASD remains poorly understood, therefore encouraging trials addressing the use of different lines of biologic disease modifying drugs in patients with AoSD might provide further insight into the pathogenesis of this complex disease.…”

“…The goal of treatment in patients with ASD is not solely focused on treating joint symptoms but also targets the systemic manifestations of the disease. With the new era of biologic therapy (Biologic disease modifying anti-rheumatic drugs), anti-tumor necrosis factor alpha therapy, anti-IL-1 therapy and recently anti-IL-6 are highly recommended in patients with chronic systemic activity who are considered refractory to traditional DMARDs [7][8][9][10][11][12][13]. Trials studying the use of anti-CD-20 (rituximab) in conventional DMARD non-responders with systemic onset rheumatoid arthritis (ASD) are lacking with only two case reports displaying efficacy of the drug in refractory cases who failed anti-TNF therapy.…”

Steroid-sparing Effects of Etanercept in a Patient with Steroid-dependent Adult-onset Still's Disease

“…We did not observe any correlations between the response to ETN and age or gender. Only two of the 12 patients who switched from other TNF inhibitors, such as infliximab or adalimumab, responded to ETN (3,13,16,18,19,23,24,28,29,31,32), suggesting that physicians should avoid the use of ETN in patients with an inadequate response to other TNF inhibitors. Three patients requiring discontinuation of ETN due to infection have been reported (3,12,30).…”