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Clin Immunol. 2019 Sep;206:23-32. doi: 10.1016/j.clim.2018.10.016. Epub 2018 Oct 28.

Targeting cytokines to treat autoinflammatory diseases.

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Autoinflammatory Disease Center, Beth Israel Deaconess Medical Center, 110 Francis Street, Suite 4b, Boston, MA 02215, United States; Autoinflammatory Diseases Clinic, Boston Children's Hospital, 300 Longwood Avenue, Fegan 6, Boston, MA 02115, United States; Harvard Medical School, Boston, United States. Electronic address:


Autoinflammatory diseases are rare group of conditions manifested by recurrent fevers, systemic inflammation, and dysfunctions of the innate immune system. These conditions are characterized by overproduction or lack of inhibition of various cytokines, and the advent of biologic drugs that block specific cytokines involved in these conditions has revolutionized their treatment. In this review, I will discuss the most common autoinflammatory conditions of adulthood including familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), mevalonate kinase deficiency/hyperimmunoglobulinemia D Syndrome (MKD/HIDS), TNF receptor-associated autoinflammatory syndrome (TRAPS), and systemic juvenile idiopathic arthritis/adult-onset Still's disease (SJIA/AOSD). I will discuss how IL-1, IL-6, IL-18, and TNF play pathogenic roles in these conditions and will review the evidence behind cytokine blockade for these diseases. Throughout the paper, I will reflect on gaps in knowledge of autoinflammatory diseases and will highlight the latest advances and newest drugs in development.


Adult-onset Still's disease; Autoinflammatory diseases; Biologics; CAPS; Cytokine inhibitors; FMF; HIDS; Interleukin-1; Interleukin-18; Interleukin-6; MKD; TRAPS; Tumor necrosis factor


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