Efficacy of Anti-Interleukin-1 Receptor Antagonist Anakinra (Kineret®) in a Case of Refractory Sweet’s Syndrome

Abstract

Sweet’s syndrome is a neutrophilic dermatosis characterized by fever, an elevated neutrophil count, and painful erythematous cutaneous lesions. Histopathological analysis reveals a neutrophilic dermal infiltrate. Systemic corticosteroid therapy remains the mainstay of treatment. We report the case of a 66-year-old male patient who had a 5-year history of Sweet’s syndrome refractory to various conventional treatments. Anti-interleukin-1 receptor antagonist anakinra was initiated and this resulted in a dramatic clinical and biological improvement. Anakinra is a promising treatment for neutrophilic dermatoses and sheds light on the interleukin-1/inflammasome pathway as central in the physiopathology of neutrophilic dermatosis.

Severe Sweet's Syndrome with Elevated Cutaneous Interleukin-1β after Azathioprine Exposure: Case Report and Review of the Literature

“…Evidence for any of these therapeutic regimens is however limited as based on case reports and small case series. Recently very encouraging responses to Kineret have been reported in patients with refractory SS, further suggesting that IL-1α or IL-1β play a significant role in the pathophysiology of this disease [30,31]. …”

Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome

“…So far, there is no evidence that treatment with any specific blockade of IL-1 adversely affects the behavior of the associated B-cell clone. Indeed, there is a single report suggesting the opposite, with the monoclonal protein concentration having fallen in 1 Schnitzler syndrome patient following anakinra therapy 28 ; benefits of anakinra have also been reported in patients with smoldering myeloma evidenced by reduction of the proliferative index. 29 To date, clinically significant progression of the clonal B-cell disorder has not occurred in any of our patients, and only 1 has seen a doubling of their low-level paraprotein; none have yet required chemotherapy.…”

First Clinical Description of an Infant With Interleukin-36-Receptor Antagonist Deficiency Successfully Treated With Anakinra

“…A good response of skin involvement with normalization of inflammatory markers has been recently reported in 2 patients with GPP on anakinra treatment. 7 Anecdotal reports have also evidenced the efficacy of this agent in neutrophilic dermatosis such as Sweet syndrome, 8 neutrophilic panniculitis, 9 and neutrophilic urticarial dermatosis. 10 Conversely, anakinra showed no significant efficacy in psoriatic arthritis in an open-label pilot study.…”