Autoinflammation of Unknown Cause

Links checked 3/04/17.

There are several autoinflammatory conditions that are already known about, including Sweet’s syndrome. However, there are some people that demonstrate the signs and symptoms associated with autoinflammatory conditions, but these signs and symptoms do not quite fit in with any known or specific autoinflammatory condition. This is called autoinflammation of unknown cause (Simon et al, 2015).

What are the symptoms of autoinflammation of unknown cause?

Common symptoms of autoinflammation of unknown cause include:

  • Fever.
  • Skin rash.
  • Muscle pain.
  • Joint pain or inflammation of one or more joints with redness, swelling and warmth.
  • Abdominal pain.
  • Diarrhoea.
  • Nausea.
  • Chest pain (caused by inflammation of the pleura or pericardium).
  • Enlarged lymph nodes.
  • Enlarged liver or spleen.
  • Red eyes.
  • Aphthous ulcers in the mouth or on the genitals.

How is it diagnosed?

Autoinflammation of unknown cause can be difficult to diagnose, and be left undiagnosed for many years. However, it has certain characteristics that can aid in diagnosis.

  • Marked inflammatory response. This can be measured in the blood. Blood tests often show a raised erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and white blood cell count during an attack or flare-up. Outside of an attack, blood results will be normal.
  • No other cause for the symptoms is found, e.g. infection, autoimmune disease or a known autoinflammatory condition. To know for sure whether or not another disease is causing the symptoms, extensive investigation has to be performed prior to the diagnosis, e.g. numerous blood tests, X-rays and scans.

How is it treated?

The biologic and anti-inflammatory medication anakinra (Kineret) is the initial treatment for autoinflammation of unknown cause.

The information above has been taken from the AUTOINFLAMMATION.EU website and provided by Dr. Anna Simon and other autoinflammatory disease experts. Many thanks to Karen Durrant of the Autoinflammatory Alliance for passing this information on to us.


Simon, A. et al (2015) Autoinflammation of Unknown Cause. AUTOINFLAMMATION.EU (online). Updated 8/01/15, and accessed on 3/04/17.

© 2012-2017 Sweet’s Syndrome UK


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