Research abstract:

Cryopyrin associated periodic syndromes (CAPS) is a rare group of autoinflammatory disorders that includes familial cold autoinflammatory syndrome (FCAS), Muckle-wells syndrome (MWS), and Neonatal onset multisystemic inflammatory disease (NOMID) .

CAPS is caused by a mutation in the NLRP3 (NOD like receptor family, pyrin domain containing 3) gene. This ultimately leads to increased production of IL-1β. IL-1β is a biologically active member of the IL-1 family. It is not only a pro-inflammatory cytokine responsible for features such as fever, rash, arthritis, but is also a major mediator in the central pathways of fatigue.

Fatigue is a major component of CAPS and is associated with severely compromised quality of life . In clinical studies, fatigue was measured using FACIT-F (functional assessment of chronic illness therapy-fatigue) and SF-36 (short form-36, physical component score) instruments.

These questionnaires can also be used to monitor improvement of fatigue following initiation of therapy. IL-1 inhibitors block the IL-1 signaling cascade, thereby preventing systemic inflammation in CAPS. The decrease in systemic inflammation is accompanied by improvement in fatigue .

Impact of IL-1 inhibition on fatigue associated with autoinflammatory syndromes, by P Efthimiou & SYadlapati in Mod Rheumatol, 2015 Jul 3: 1-22