Angioedema of the Periorbital Region that Developed during Treatment with Etanercept in a Case of Refractory Adult-Onset Still's Disease

Abstract

A 78-year-old Japanese man with adult-onset Still's disease that was refractory to conventional treatment, such as prednisolone (PSL) concomitant with methotrexate (MTX). Etanercept (50 mg/week) was added to PSL (12.5 mg/day) and MTX (12 mg/week). His manifestation improved dramatically, nonetheless massive edema of the periorbital region developed by the fourth injection, which kept his palpebral fissure completely closed. There was also a marked injection site reaction to etanercept. A diagnosis of angioedema due to etanercept was thus made, and the drug was discontinued. His angioedema began to ameliorate soon after antihistamines were introduced without any critical involvement, such as laryngeal obstruction.

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

“…Treatment with ETN resulted in significant clinical and serological improvements in 26 of the 51 patients, although effectiveness was variably defined. Among the elderly patients (more than 60 years of age ) , two of the four patients responded to ETN (2,26,28,33). We did not observe any correlations between the response to ETN and age or gender.…”

Elderly-onset adult Still’s disease

“…Biologics such as tocilizumab, etanercept and anakinra have demonstrated good outcomes, especially in refractory EOSD or in case of complications [22,23,28,31,35,38,58,59]. These treatments need to be prescribed carefully in EOSD because of serious adverse events including opportunistic infections, anaphylactic reactions or biologic-associated macrophage activation syndrome [26,31].…”

Elderly-onset Still's Disease Complicated by Macrophage Activation Syndrome: A Case Report and Review of the Literature

“…In contrast, immunosuppressants and biologics may tend to be avoided in EOSD patients due to concerns about infection. A patient with EOSD who discontinued IL-6 inhibitor due to severe infection has been reported (19). Immunosuppressants and biologics need to be prescribed particularly carefully in EOSD cases.…”