Current approaches to diagnosis, treatment, and monitoring in patients with cryopyrin-associated periodic syndromes (CAPS)

Abstract

Cryopyrin-associated periodic syndromes (CAPS) are the most prominent representatives of monogenic autoinflammatory diseases (AIDs). They are characterized by a concurrence of fever, rash, and other symptoms that are accompanied by disabling or life-threatening complications. Based on the current guidelines for the management of patients with AIDs and on their own experience, the authors have elaborated a diagnostic algorithm for CAPS, which can optimize their differential diagnosis with systemic juvenile arthritis and other AIDs and the early use of effective therapy and management tactics. Interleukin-1 inhibitors are target agents, by which one can monitor activity and prevent complications. The given algorithm will be able to improve the timely diagnosis of CAPS and to optimize treat-to-target treatment and management in patients.

Keywords

• cryopyrin-associated periodic syndromes (caps)
• guidelines
• treatment
• monitoring
• interleukin-1 inhibitors
• canakinumab