IL-1 inhibitors have been studied to mitigate symptoms and downregulate the inflammatory response. Anakinra is a synthetic form of IL-1 RA, acting within the body to inhibit IL-1β binding to its receptor type, preventing IL-1β biologic activity.5 Individuals with MWS don’t produce enough endogenous IL-1 receptors to control the excess amounts of circulating IL-1β. Anakinra has been used in pregnant patients with CAPS diseases, with successful symptom resolution and minor side effects on the fetus.6
A study consisting of 26 patients, 57% females, with severe MWS was conducted to assess the efficacy of both anakinra and canakinumab, a monoclonal IL-1β antibody that prevents IL-1β from binding to the receptor site. 75% of patients on anakinra achieved clinical remission compared with 99% of patients in the canakinumab treatment arm, with similar safety profiles.7 Both drugs led to clinical improvement and decreased ESR, CRP and MWS disease activity scores (MWS-DAS).
In patients refractory to anakinra, canakinumab has shown sustained disease control.8 A recently published study shows canakinumab crosses the placenta when administered during pregnancy.8 The safety profile and long-term effects of these medications on the fetus need to be explored.
Aiza Tariq, MD, is an internal medicine resident at the Icahn School of Medicine at Mount Sinai Beth Israel, New York. She completed her medical degree at the University of Florida. Her primary research interests include rheumatic disease.
Harry D. Fischer, MD, is chief of the Division of Rheumatology and associate chairman of medicine at Icahn School of Medicine at Mount Sinai Beth Israel, New York. He is also associate professor of medicine at the Icahn School of Medicine at Mount Sinai.
References
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- Muckle T, Wells M. Urticaria, deafness, and amyloidosis: A new heredo-familial syndrome. Q J Med. 1962 Apr;31:235–248. PubMed: 14476827.
- Sönmez HE, Özen S. Clinical update on inflammasomopathies. Int Immunol. 2017 Apr 6.
- Kuemmerle-Deschner JB, Haug I. Canakinumab in patients with cryopyrin-associated periodic syndrome: An update for clinicians. Ther Adv Musculoskelet Dis. 2013 Dec;5(6):315–329.
- Koné-Paut I. Galeotti C. Anakinra for cryopyrin-associated periodic syndrome. Expert Rev Clin Immunol. 2014 Jan;10(1):7–18.
- Chang Z, Spong CY, Jesus AA, et al. Anakinra use during pregnancy in patients with cryopyrin-associated periodic syndromes (CAPS). Arthritis Rheumatol. 2014 Nov;66(11):3227–3232.
- Kuemmerle-Deschner JB, Wittkowski H, Tyrrell PN, et al. Treatment of Muckle-Wells syndrome: Analysis of two IL-1 blocking regimens. Arthritis Res Ther. 2013;15(3):R64.
- Egawa M, Imai K, Mori M. et al. Placental transfer of canakinumab in a patient with Muckle Wells syndrome. J Clin Immunol. 2017 May;37(4):339–341.