Role of interleukin‐6 in a patient with tumor necrosis factor receptor–associated periodic syndrome: Assessment of outcomes following treatment with the anti–interleukin‐6 receptor monoclonal antibody tocilizumab

Abstract

In this report, we describe treatment outcomes in the first case of a patient with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) treated with the anti-interleukin-6 (anti-IL-6) receptor monoclonal antibody tocilizumab. Since IL-6 levels are elevated in TRAPS, we hypothesized that tocilizumab might be effective. The patient, a 52-year-old man with lifelong TRAPS in whom treatment with etanercept and anakinra had failed, was administered tocilizumab for 6 months, and the therapeutic response was assessed by measurement of monocyte CD16 expression and cytokine levels. Following treatment, the evolving acute attack was aborted and further attacks of TRAPS were prevented. The patient did not require corticosteroids and showed significant clinical improvement in scores for pain, stiffness, and well-being. Moreover, the acutephase response diminished significantly with treatment. Monocyte CD16 expression was reduced and the numbers of circulating CD14؉CD16؉ and CD14؉؉CD16؊ monocytes were transiently decreased. However, cytokine levels were not reduced. This case supports the notion of a prominent role for IL-6 in mediating the inflammatory attacks in TRAPS, but blockade of IL-6 did not affect the underlying pathogenesis. These preliminary findings require confirmation.

How not to miss autoinflammatory diseases masquerading as urticaria

“…Cryopyrin-associated periodic syndrome and Schnitzler's syndrome, but not isolated chronic urticaria, respond poorly (or not at all) to glucocorticoids except at high doses of the order of a mg per kg (45). In HIDS, TRAPS, AOSD and soJIA however, oral glucocorticoids can be quite effective (7,34,40,46). Suspicion of autoinflammatory disease should prompt appropriate diagnostic measures to rule out or confirm CAPS, SchS or another autoinflammatory condition.…”

Canakinumab efficacy and long-term tocilizumab administration in tumor necrosis factor receptor-associated periodic syndrome (TRAPS)

“…TRAPS treatment is aimed at preventing acute disease attacks, improving quality of life, and preventing the development of systemic reactive amyloidosis, the most troublesome long-term TRAPS complication [4][5][6]. Notably, despite considerable success achieved in recent times, TRAPS treatment is more challenging than other monogenic autoinflammatory diseases probably due to the genetic heterogeneity and protean clinical phenotype [4].…”

Successful treatment of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) with tocilizumab: a case report

“…Vaitla et al (10) reported tocilizumab outcomes in a patient with TRAPS. In their case, the evolving acute attack was aborted, and further attacks of TRAPS were prevented.…”