Periodic fever syndromes are a group of rare genetic diseases that cause the immune system to become overactive, leading to inflammation in the body.1 People with periodic fever syndromes experience repeated episodes of fever, rashes and joint pain (called “flares”) that can last from a few days to a few weeks.1,2

Periodic fever syndromes include diseases such as1:

  • Familial Mediterranean fever (FMF)
  • Hyperimmunoglobulin D syndrome/mevalonate kinase deficiency (HIDS/MKD)
  • Tumour necrosis factor receptor-associated periodic syndrome (TRAPS)
  • Cryopyrin-associated periodic syndromes (CAPS).
  • FMF is most common in people from the Mediterranean region.2
  • Flares can last from a few hours to 3–4 days. The main symptoms are fever, abdominal pain and chest pain, but other symptoms may include joint inflammation, skin rash and spleen enlargement.2
  • The cause of flares is not always known, but possible triggers include stress, cold, infections, some medications and the menstrual cycle.3
  • HIDS/MKD is a rare condition that happens when a person has abnormally high levels of an antibody called immunoglobulin D.4 It usually begins in the first year of life.5
  • Only 200 cases have been reported worldwide; of these, Europeans are most commonly affected.4
  • Flares occur every 2–8 weeks and last 3–7 days. The main symptoms are fever, abdominal pain, vomiting and diarrhoea, but other symptoms can include joint pain, swollen lymph nodes, skin rash and headaches.5
  • Flares can occur spontaneously or be triggered by vaccinations, infections, or emotional or physical stress.5
  • TRAPS is a rare condition that happens when there is a mutation in a gene called TNFRSF1A, which provides instructions to make one of the proteins involved in inflammation.6
  • Only 1000 cases have been reported worldwide; of these, Europeans are most commonly affected.7
  • Flares occur anywhere between every 6 weeks to every few years. Each flare typically lasts about 3 weeks, but it can vary from a few days to a few months. Symptoms include fever, abdominal pain, muscle pain and skin rash.6
  • Flares can occur spontaneously or be triggered by minor injuries, infection, stress, exercise or hormonal changes.6
  • CAPS include three different conditions with overlapping symptoms that differ in severity8:
    • Familial cold autoinflammatory syndromes (FCAS), also known as familial cold urticaria (FCU) (mildest)
    • Muckle-Wells syndrome
    • Neonatal-onset multisystem inflammatory disorder (NOMID), also known as chronic infantile neurological cutaneous articular syndrome (CINCA) (most severe).
  • Common symptoms include rash, fatigue, headache, fever, joint pain and swelling and red eyes. The more severe CAPS may also have more serious symptoms such as hearing loss and damage to the brain.8,9
  • CAPS are very rare and are thought to affect only 1 in 1 million people worldwide.10

References

  1. PeriodicFevers.com. Periodic fever syndromes. Available at: https://www.periodicfevers.com/about-periodic-fevers/periodic-fevers-syn... [Accessed July 2020].
  2. Ciccarelli F et al. Curr Med Chem 2014;21(3):261–269.
  3. Orpha.net. Familial Mediterranean fever. Available at: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=342 [Accessed June 2020].
  4. PeriodicFevers.com. HIDS: Hyperimmunoglobulinemia D syndrome. Available at: https://www.periodicfevers.com/thescience/hids/ [Accessed June 2020].
  5. Orpha.net. Hyperimmunoglobulinemia D with periodic fever. Available at: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=343# [Accessed June 2020].
  6. Genetics Home Reference. Tumor necrosis factor receptor-associated periodic syndrome. Available at: https://ghr.nlm.nih.gov/condition/tumor-necrosis-factor-receptor-associa... [Accessed June 2020].
  7. PeriodicFevers.com. TRAPS: Tumor necrosis factor receptor associated periodic syndrome. Available at: https://www.periodicfevers.com/thescience/traps/ [Accessed June 2020].
  8. PeriodicFevers.com. CAPS: Cryopyrin-associated periodic syndromes. Available at: https://www.periodicfevers.com/thescience/caps/ [Accessed June 2020].
  9. Goldbach-Mansky R. Curr Rheumatol Rep 2011;13(2):123–131.
  10. The NOMID Alliance. CAPS: Cryopyrin-Associated Periodic Syndromes. Available at: http://www.autoinflammatory.org/downloads/finalCAPSbrochure_web.pdf [Accessed June 2020]. ILA20-C008i August 2020.
ILA20-C008i September 2020.
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