Refractory Adult-Onset Still Disease Successfully Treated With Abatacept

Abstract

Adult-onset Still disease (AOSD) is an inflammatory condition of unknown etiology that responds to glucocorticosteroids and disease-modifying antirheumatic drugs, particularly methotrexate. However, disease refractory to conventional treatment has led to the reported use of biologic therapy including tumor necrosis factor α inhibitors (infliximab, etanercept, and adalimumab), anakinra, rituximab, and tocilizumab. We report the successful use of abatacept in the treatment of a patient with AOSD manifested by polyarthritis, rash, fevers, elevated liver function tests, and ferritin levels refractory to treatment with methotrexate and hydroxychloroquine. Remission has been maintained for 35 months with the addition of abatacept administered once monthly. There is evidence that T-cell activity plays an important role in the autoimmune activity of AOSD, and modulation of CD28 costimulation of T cells by abatacept has specific immunosuppressive actions that make it an appealing alternative therapeutic option for refractory AOSD.

Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy

“…Canakinumab showed some inconsistent results in AOSD [37][38][39] but is currently being investigated for its efficacy on AOSD's joint involvement (NCT02204293). No patient in this study received abatacept or rituximab; the data are very limited but some authors reported efficacy in patients who fail to respond to TNF, IL-1, or IL-6 inhibition [40][41][42][43][44][45].…”

Adult-Onset Still’s Disease: From Pathophysiology to Targeted Therapies

“…Abatacept (CTLA4IgFc), a fusion protein which consists of the extracellular domain of the cytotoxic T-lymphocyte antigen 4 (CTLA-4) and the Fc portion of immunoglobulin G1 (IgG1), inhibits T-cell activation by binding to CD80 and CD86 receptors on antigen-presenting cells (APCs) and preventing their interaction with CD28 receptor on T cells. Recent findings support a potential role of the latter in AOSD cases refractory to conventional DMARDs, anti-TNF- α agents, and even to IL-1 receptor antagonists [81, 82]. …”

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

“…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).…”