Successful treatment of adult-onset Still’s disease with tocilizumab monotherapy: two case reports and literature review

Abstract

Adult-onset Still's disease (AOSD) is a systemic inflammatory disease of unknown etiology. Recently, it has been reported that quite a few cases of refractory AOSD were successfully treated with tocilizumab (TCZ) and corticosteroids were withdrawn in some of these patients. We report two AOSD patients who were treated successfully with TCZ monotherapy; thus, avoiding corticosteroid treatment. Because both of the patients refused to take corticosteroids, we planned to treat them with 8 mg/kg of TCZ monotherapy at weeks 0, 2, 6 and subsequently every 4 weeks. The efficacy of TCZ was assessed by patients' clinical symptoms such as fever, arthralgia, skin eruptions, and laboratory markers such as serum levels of CRP, ferritin, and IL-6. We also reviewed 14 previous case reports including 30 cases who had been treated with TCZ for AOSD. Our patients responded rapidly and have been maintained in clinical remission without corticosteroid treatment. In the literature review, concomitant corticosteroid treatment described in 13 cases was successfully tapered in 7 and discontinued in 6 cases. TCZ monotherapy can be a candidate for the first-line therapy for some AOSD patients.

Hemophagocytic Lymphohistiocytosis in Adults

“…Recent studies of patients with adult-onset Still disease complicated by MAS have evaluated the efficacy of the IL-1 receptor antagonist anakinra and the IL-6 receptor tocilizumab. 31,32 Although small case reports have been promising, there is concern that the inhibition of a single cytokine by a biological response modifier may be of limited efficacy for the treatment of the cytokine storm that is present in MAS. Confirmation of the efficacy of these agents awaits larger trials.…”

Review: Is There a Window of Opportunity for Treatment of Systemic Juvenile Idiopathic Arthritis?

“…Importantly, recent studies of the IL‐6 receptor antagonist tocilizumab found impressive efficacy in systemic JIA, both for treatment of systemic symptoms (fever and rash) and for treatment of chronic arthritis (). No data are available concerning the efficacy of tocilizumab as first‐line therapy in systemic JIA, although case reports of patients with AOSD suggest that this approach might also be effective ().…”

THU0576 Therapeutic options for patients with rare rheumatic diseases: a systematic review and meta-analysis

“…Sreenath 1 , S. Cherian 1 , G. Antony 1 , U. Mony 2 , P. Shenoy 1 . 1 Centre for Arthritis and Rheumatism (CARE); 2 Molecular Medicine, AIMS, Cochin, India Background: Palindromic rheumatism (PR) although often considered as a benign disease can be severe and resistant to DMARDs in some patients. In these patients it can result in almost daily attacks, migrating from joint to joint resulting in poor quality of life.…”