Your child with frequent fevers: is it autoinflammatory?

Los Roques, VenezuelaWhen I was little, I used to get a lot of fevers.  With my fevers, I would develop pain in one or both of my ears, and I felt so drained that I just wanted to be left alone to watch TV (or play Atari).  If my fever spiked in the middle of the night, my parents would soak me in a tub of lukewarm water to lower my temperature (how I hated those baths!).  On the following day, I would visit my pediatrician, who would invariably diagnose me with an ear infection.  He prescribed a delicious bubble-gum flavored antibiotic that my parents kept at the top shelf in the refrigerator (out of my reach so I wouldn’t overdose).  I’m not sure if it was the taste or the active ingredient, but this antibiotic always made me feel better.  After what seemed like the millionth episode of fever, my pediatrician recommended that I get tubes in my ears, and the episodes of fever and ear pain subsided.

Recurrent infections, like those I had when I was a child, are the most common cause of recurrent fevers in children.  Usually, these infections are due to viruses, such as the ones that cause the common cold.  Children with colds often develop symptoms attributable to the virus, including a runny nose, nasal congestion, or cough.  Multiple infections, one after the other, are especially common in children attending daycare or school, where they trade viruses like I used to trade baseball cards with my friends.  When I see these the worried parents of these children in my office, I have to remind them that it is normal for a child to have 9-12 colds every year.  Fortunately, these children continue to grow and gain weight without difficulty, and they are healthy between episodes. “As your child grows,” I tell the parents, “his immune system will become better at fighting infections, and the frequency and severity of fevers will diminish.”  Aside from stressing the importance of hand washing among all family members, there is not much that can (or needs) to be done about these episodes. 

However, if the episodes do not clearly match the above description, other diagnoses should be considered.  In this post, I will provide some tips that may help parents and physicians recognize a rare cause of fever in children: autoinflammatory diseases.  The most common autoinflammatory disease in children is called PFAPA, which stands for Periodic Fevers, Aphthous stomatitis (canker sores or oral ulcers), Pharyngitis (sore throat) and Adenitis (enlarged glands in the neck).   This disease usually presents in children less than 5 years of age, and causes episodes of fevers in addition to the features that make up the name of the disease (oral ulcers, sore throat, enlarged glands in the neck).  The fever usually lasts about 4 days, and it recurs at regular intervals, usually every month.  What is unique about PFAPA is that parents can usually predict when the child is “due” to get ill.  The episodes of fever are so regular as to be predictable.  This regularity is rare in most other diseases.  PFAPA is commonly misdiagnosed as frequent Strep infections, and testing for Strep throat is usually negative.  Patients do not readily respond to antibiotics, although their symptoms can disappear with one dose of steroids.

Another clue that the child may have an autoinflammatory disease is that all of the episodes of fever look alike.  For example, if the child always develops abdominal and joint pain with the fevers, then he may have familial Mediterranean fever (FMF).  If, in addition to fever, she always develops a painful rash, muscle pain, and red eyes, then she may have TNF-receptor associated periodic fever (TRAPS).  If he develops fevers, rashes, and joint pain after exposure to cold weather, then the child may have familial cold-induced autoinflammatory syndrome (FCAS).

The length and frequency of fever, associated symptoms, and age at which the fevers began can help differentiate between these (and other) autoinflammatory diseases.   Thus, it is very helpful to keep a fever diary,  specifying the characteristics of each episode.  Because many of these autoinflammatory diseases are inherited (genetic), there is often a history of other family members having similar symptoms.  In addition, it is important to note that some autoinflammatory diseases are more common in people of certain ethnic backgrounds, such as FMF in Sephardic Jews, Turks, and Armenians.

In addition to autoinflammatory diseases, there are some features of recurrent fevers that should warrant a closer look by a child’s  pediatrician.  These include prolonged episodes of fever (more than one week), recurrent fever without symptoms attributable to a viral infection, difficulty growing or gaining weight, or if the child does not return to his baseline between episodes.  Other causes of fever that should be considered in these instances include:

  • Immunodeficiencies: problems in the immune system that makes the child more susceptible to infections
  • Anatomic and metabolic abnormalities: abnormalities in certain organs that make them more prone to infections (like my ears), or diseases such as cystic fibrosis
  • Inflammatory bowel disease: inflammation of the intestines
  • Cancer: such as leukemia or lymphoma

With this knowledge, parents and pediatricians alike can help distinguish between the majority of children that develop recurrent fevers because of frequent viruses, from causes which may require additional workup or treatment.

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5 thoughts on “Your child with frequent fevers: is it autoinflammatory?

  1. This is a really helpful article. Thanks for all that you are doing to get more information and awareness out to the public about autoinflammatory diseases

  2. The only issue I have….yes, good information, but very simplified. My son’s PFAPA symptoms often sounds more like the other disorders, but bloodwork is negative, but as we all know, only a certain percentage of positives occur with bloodwork, leaving some kids with false negatives and often parents with more questions than answers. When I listen to others talk about their child’s symptoms, it’s never so simple and cut and dry. It really seems like there are some usual patterns but the actual symptoms vary. So much more research needs to continue on these disorders. I still have to wonder if at some point PFAPA will be found to be a spectrum of varying autoinflammatory conditions, which could explain the varying symptoms many of these kids get.

    1. Hi Wendy. You’re right that autoinflammatory diseases are more complex than what I’ve written here. In fact, they’re more complex than anyone knows! About 25-50% of patients that I see with autoinflammatory diseases have negative genetic workup, like your son. We still don’t know many of the genes that cause these diseases (new diseases are discovered every month!). In addition, some diseases like perhaps PFAPA and systemic arthritis (adult onset Still’s disease) do not seem to be completely genetic. There doesn’t seem to be one gene that causes the disease, like there is for Familial Mediterranean Fever, for example. Perhaps there are environmental triggers (such as infections) that, combined with a person’s genetic makeup, contribute to the disease. I hope that by continuing to study these diseases, we’ll be able to identify the causes of these illnesses, including your son’s. In the meantime, we are left to try different medicines to see if they work for a patient’s recurrent fevers.

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