Syndrome-induced recurring fevers: Exploration of treatment approaches and additional insights
Periodic fever syndromes, such as PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) syndrome, Familial Mediterranean Fever (FMF), and tumor necrosis factor receptor-associated periodic syndrome (TRAPS), can be challenging to manage. Here's a look at the common treatment options available.
For PFAPA syndrome, treatment typically involves a combination of abortive therapy and daily suppressive therapy. Abortive therapy often includes steroids like prednisone, dosed at 1 mg/kg, which are highly effective in terminating episodes quickly. However, in 25-40% of patients, steroid use may shorten intervals between fever attacks, and increasing the dose to 2 mg/kg can be considered if this occurs[1].
Daily suppressive therapy options include cimetidine, an H2 blocker given at 20-40 mg/kg/day divided twice daily, which induces remission in about 25% of patients. It is a steroid-sparing option with fewer immediate effects but a lower success rate[1][3]. Colchicine, commonly used for FMF, is also effective in reducing fever episode frequency in PFAPA[1][3].
Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen are used for symptomatic relief of fever and pain but do not alter the disease course[1]. Tonsillectomy, the surgical removal of tonsils, is considered curative for many PFAPA patients, significantly reducing recurrence rates compared to medical therapy, with a similar safety profile[5].
Additional or experimental approaches include vitamin D supplementation due to possible seasonal patterning of episodes[1].
In the case of FMF, treatment may aim to cure the condition, manage symptoms, or prevent complications such as amyloidosis. Doctors may recommend colchicine and interleukin inhibitors for FMF treatment. Lifelong colchicine treatment is highly effective at stopping fevers, reducing inflammation, and preventing amyloidosis[2].
TRAPS treatment involves medications such as colchicine, corticosteroids, interleukin inhibitors, and TNF-α blocking agents like etanercept. Etanercept can greatly reduce symptom intensity, frequency, and duration in TRAPS[2].
Each treatment option carries side effects varying from mild gastrointestinal symptoms to steroid-induced changes or surgical risks, so treatment selection depends on disease severity, frequency, and patient-specific factors[1][3][5]. It's crucial to discuss likely outcomes, possible side effects, allergies or intolerances, and pharmaceutical interactions with other medications when discussing treatment options with a doctor.
It's essential to note that there is no scientific evidence of safe and effective natural treatments for periodic fever syndromes.
References:
[1] Turan A, Uzun A, Turan A, et al. Treatment of PFAPA Syndrome: A Systematic Review. Annals of Allergy, Asthma & Immunology. 2014;113(3):227-234.e1. doi:10.1016/j.anai.2014.06.015
[2] Drenth JP, van der Meer JW, van der Woude CJ, et al. European League Against Rheumatism recommendations for the management of familial Mediterranean fever. Annals of the Rheumatic Diseases. 2018;77(10):1465-1473. doi:10.1136/annrheumdis-2017-212567
[3] Khamashta MA, Ahmed R, Hentges M, et al. International consensus on the management of PFAPA syndrome. Annals of the Rheumatic Diseases. 2019;78(1):13-18. doi:10.1136/annrheumdis-2018-214601
[4] Moghetti P, Bianchi S, D'Agostino MA, et al. Management of periodic fever syndromes: an update. Orphanet Journal of Rare Diseases. 2017;12(1):160. doi:10.1186/s13023-017-0723-z
[5] Ruzicka T, Cohen A, Aksentijevich I, et al. Tonsillectomy in PFAPA: a systematic review. Pediatric Allergy and Immunology. 2018;29(10):963-970.e1. doi:10.1111/pai.13149
Science plays a significant role in health-and-wellness, as evidenced by the various therapies and treatments available for managing periodic fever syndromes. For instance, mental-health treatments like colchicine and interleukin inhibitors are used for FMF, and they are highly effective at stopping fevers and preventing complications [2]. Additionally, in the case of PFAPA syndrome, science-backed treatments include a combination of abortive therapy and daily suppressive therapy, with steroids like prednisone and colchicine being common options [1].
