This month, an interesting article about PFAPA appeared in the journal Rheumatology. PFAPA (periodic fevers, aphthous stomatitis, pharyngitis, and adenitis) is an autoinflammatory disease that affects children. It is manifested by episodes of fevers lasting a few days, in addition to the features that make up the name of the disease. Unlike other autoinflammatory diseases, episodes of fever occur at very specific intervals (parents mention that the fevers occur “like clockwork,” and they can predict when the next episode will occur on a calendar). Fortunately, most patients respond well to steroids during fever episodes, and the disease usually resolves within a few years without sequellae.
This study represents the largest group of patients with PFAPA ever published. They found that the average age of onset of symptoms was about 2 years of age, and it often took several years to make the diagnosis of PFAPA. Most patients were diagnosed before age 6. On average, episodes of fever lasted 4 days, and recurred every 4 weeks. Only 44% of patients with PFAPA had the classic triad of apthous ulcers (oral ulcers), adenitis (swollen, tender lymph nodes in the neck), and pharyngitis (sore throat); the rest had only one or two of these symptoms. Interestingly, children often complained of abdominal pain, joint pain, muscle pain, and headaches during episodes.
This study highlights one of the biggest difficulties of PFAPA–making an accurate diagnosis. Patients don’t need to have the classic triad of symptoms to be diagnosed with PFAPA, and many children complain of additional symptoms during episodes that are likely underrecognized. Perhaps it is time to create new diagnostic criteria for this disease!
What do you think? Have you or a loved one been diagnosed with PFAPA? How long did it take for your doctors to make the diagnosis?