[BLOG] Periodic Fever Syndromes in Adults

Links checked 2/03/17.

SAID Support has posted a very informative blog article on autoinflammatory conditions in adults.

Periodic Fever Syndromes in Adults.

Posted on 28th October 2015 by Jennifer Tousseau (author) & Karen Durrant RN (editor).

‘Autoinflammatory diseases, also commonly known as periodic fever syndromes, are systemic inflammatory diseases caused by malfunctions of the innate immune system. Most autoinflammatory diseases are genetic and the first symptoms start in childhood, and most are chronic lifelong diseases.  However, due to the complex range of symptoms and their rarity, patients with these conditions may not get diagnosed or helpful treatment until they are adults.

In addition, some patients may be diagnosed with autoinflammatory disease symptoms, but the cause may not have been classified, or determined yet, and these patients are still seeking a diagnosis, often into adulthood.  But the following common symptoms and labs may be noted in these patients.  Experts on autoinflammatory diseases estimate that up to 2/3 of their patient population has a systemic undifferenciated autoinflammatory syndrome (SURFS), so it is important to consider systemic autoinflammatory diseases as a possible diagnosis in a patient that has the following symptoms, even if the genetic cause has not yet been identified.

Typical Autoinflammatory Symptoms and Lab Results.

Symptoms vary between the different autoinflammatory syndromes, but there are some common symptoms that many share:

  • recurrent fevers (without an infectious cause)
  • rashes
  • joint pain and/or arthritis
  • mouth ulcers
  • gastrointestinal symptoms including vomiting and/or diarrhoea
  • stomach or chest pain
  • red eyes (non-infectious conjunctivitis) – some may even get uveitis and/or iritis (inflammation in the eyes).

Many autoinflammatory diseases have similar lab results including:

  • high inflammatory markers (CRP, ESR, SAA)
  • high WBC
  • most have negative ANA and other autoantibody tests.

Some autoinflammatory diseases cause chronic inflammation that can affect any organ system in the body over time. This includes:

  • sensorineural hearing loss
  • enlarged liver and/or spleen
  • bone involvement
  • secondary amyloidosis
  • aseptic meningitis (either during flares or chronically, depending on the autoinflammatory disease).

Depending on the syndrome, many will have flares of symptoms that can last for a few days to several weeks, while some will have continuous inflammatory symptoms. Between flares, some patients may be asymptomatic with normal lab values. But for those with mild to moderate chronic symptoms, even when they do not have a fever, they may have elevated inflammatory markers, other abnormal labs and/or symptoms related to chronic inflammation.

It’s important to note that the above list represents the most common presentation of many autoinflammatory diseases, but there are some who do not have typical symptoms.

It is essential that physicians consider autoinflammatory diseases as a possible diagnosis when they are presented with a patient who has recurrent fevers, especially since childhood, along with other symptoms such as rash and/or arthritis.  Although these are rare diseases, they are often misdiagnosed for years, or patients go undiagnosed into adulthood. With genetic testing and resources to compare symptoms doctors should be able to diagnose, and treat more patients.’

Read the rest of the blog here.

N.B. The autoinflammatory condition Sweet’s syndrome (SS) rarely affects children. Unlike in most autoinflammatory conditions, a genetic cause for SS is extremely rare, but it is more common in those with the genetic marker HLA-B54.

© 2012-2017 Sweet’s Syndrome UK

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