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How to get rid of PFAPA? Management and Treatment Options

3 min read

While PFAPA syndrome often resolves on its own by the second decade of life, the recurrent episodes can be challenging. This guide will explore current medical strategies for how to get rid of PFAPA symptoms and manage the condition effectively.

Quick Summary

Though PFAPA syndrome has no cure, its symptoms can be effectively managed with medical treatments like corticosteroids, prophylactic medications, and in some cases, tonsillectomy. The condition frequently resolves spontaneously in adolescence, but management offers significant relief in the meantime.

Key Points

  • No Cure, But Manageable: PFAPA syndrome does not have a cure, but its recurrent episodes are effectively managed with various treatment strategies.

  • Steroids for Flares: Oral corticosteroids can dramatically shorten or end a fever episode, though it may also shorten the time until the next flare.

  • Preventive Medication: Daily medications like colchicine can help reduce the frequency of PFAPA episodes in some patients.

  • Tonsillectomy Option: For children with severe and frequent episodes, tonsillectomy can be a highly effective, even curative, treatment.

  • Self-Limiting Nature: The condition is benign and often resolves on its own during the second decade of life, without causing any long-term health complications.

  • Professional Guidance: It is crucial to work with a healthcare provider, such as a pediatric rheumatologist, for diagnosis and to determine the best treatment approach.

In This Article

Understanding PFAPA: The Basics

PFAPA, or Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis, is the most common cause of non-infectious periodic fever in children. It is an autoinflammatory disorder, meaning it's caused by an abnormal immune system response, not a virus or bacteria. The episodes are characterized by a sudden onset of high fever, typically lasting three to seven days, alongside other key symptoms.

Symptoms often include:

  • High fever, often over 102°F (38.9°C)
  • Aphthous stomatitis (mouth sores)
  • Pharyngitis (sore throat), sometimes with white patches on the tonsils
  • Cervical adenitis (swollen lymph nodes in the neck)

Between episodes, children are typically completely healthy and show normal growth and development. The exact cause of PFAPA is unknown, and the condition is not contagious.

Medical Management of PFAPA Episodes

Aborting a PFAPA Flare with Steroids

For many patients, oral corticosteroids, such as prednisone or prednisolone, given at the onset of symptoms, can dramatically shorten or end the fever episode within hours. However, a key consideration with this approach is that while it provides quick relief, it may also shorten the time between episodes for some individuals. Repeated use can also carry side effects, so this is a treatment option to be carefully discussed with a healthcare provider. Standard fever reducers like acetaminophen or ibuprofen often have limited effectiveness against PFAPA fevers.

Prophylactic Treatment to Prevent Flares

For individuals with very frequent episodes, some doctors may recommend daily medication to help reduce the number of flares. Two medications are sometimes used for prophylaxis:

  1. Colchicine: Originally used for Familial Mediterranean Fever (FMF), colchicine is an anti-inflammatory medication that can reduce the frequency of PFAPA episodes in some children. It is a long-term treatment option, but its effectiveness varies among patients.
  2. Cimetidine: This is an older medication with immune-modulating properties. It has been used with some success in the past, but studies on its effectiveness are limited, and it is less commonly prescribed for PFAPA today.

Surgical Intervention: Tonsillectomy

For patients with severe or frequent episodes that don't respond well to medication, tonsillectomy (surgical removal of the tonsils) can be a highly effective treatment option. Studies have shown that tonsillectomy can cure PFAPA in a high percentage of children. While surgery carries its own set of risks, it is a definitive option for many and should be considered after consultation with a specialist, such as a pediatric rheumatologist or an ENT doctor. It is important to note that PFAPA can occasionally persist into adulthood, sometimes requiring more complex surgical approaches like lingual tonsillectomy, particularly if the initial tonsillectomy didn't fully resolve symptoms. For more information on tonsillectomy, consult resources like the American College of Rheumatology.

Managing Triggers and Lifestyle Factors

While PFAPA is not caused by infections, certain factors can sometimes precede or trigger flares. Stress, in particular, has been identified as a potential trigger in some individuals. Focusing on overall wellness can help manage the condition:

  • Stress Management: Identify and minimize emotional or physiological stress where possible. Encourage a balanced lifestyle with plenty of rest.
  • Maintain a Healthy Diet: While no specific diet cures PFAPA, ensuring a well-balanced diet rich in vitamins and nutrients supports overall immune health. Some research has explored the role of Vitamin D supplementation, though further studies are needed.
  • Adequate Rest: Ensuring the patient gets enough sleep can help manage the immune system and general well-being.

PFAPA Management Comparison

Treatment Approach Primary Goal Effectiveness Potential Drawbacks Application
Corticosteroids Abort episodes quickly High, for ending an acute flare May shorten the interval between flares; side effects with repeated use Short-term, acute flare management
Colchicine Prevent future episodes Moderate, works for some individuals Gastrointestinal side effects possible Long-term prophylactic treatment
Tonsillectomy Provide a definitive cure High success rate, especially in children Surgical risks; not effective for everyone Option for frequent, severe cases refractory to medication

Conclusion

For those asking how to get rid of PFAPA, it is crucial to understand that while there is no universal cure, effective management strategies exist. For most, the condition resolves spontaneously by late childhood or adolescence, and in the meantime, treatment options can significantly improve quality of life. Whether through abortive steroid treatment for flares, daily prophylactic medication like colchicine, or surgical tonsillectomy, a personalized treatment plan can be developed with a healthcare professional. Consulting with a pediatric rheumatologist is the best step for diagnosis and tailoring a management plan that works for you or your child.

Frequently Asked Questions

Oral corticosteroids often work quickly to abort a fever episode within hours. However, this should only be used under a doctor's supervision.

Yes, PFAPA is generally a self-limiting condition that usually resolves on its own as a child enters their second decade of life, typically around age 10.

Tonsillectomy has been shown to cure PFAPA in a high percentage of cases, especially in children, making it a potentially permanent solution for many.

There is no specific diet proven to cure PFAPA. However, maintaining a healthy, balanced diet with sufficient vitamins, including vitamin D, supports overall health.

Unlike a regular infection, PFAPA fevers recur on a periodic cycle (every 2-8 weeks), often don't respond to typical fever reducers, and are accompanied by mouth sores, sore throat, and swollen lymph nodes.

PFAPA can occasionally persist into adulthood, but management strategies like medication and surgery (including lingual tonsillectomy) are also options for adults to control symptoms.

While the exact cause is unknown, some potential triggers for PFAPA episodes can include emotional or physiological stress and, less commonly, certain infections.

References

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Medical Disclaimer

This content is for informational purposes only and should not replace professional medical advice.