Comparison of conventional and low dose steroid in the treatment of PFAPA syndrome: Preliminary study

Yazgan, Hamza; Gültekin, Erhan; Yazicilar, Osman; Sagun, Ömer Faruk; Uzun, Lokman

doi:10.1016/j.ijporl.2012.07.020

Cited by 36 publications

(20 citation statements)

References 13 publications

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“…The usage of short‐course corticosteroids is associated with adverse reactions, mainly vomiting, behavioural changes and sleep disturbances in children. Altogether, 15–35% of PFAPA patients report side effects, most commonly restlessness, due to corticosteroid treatment .…”

Section: Resultsmentioning

confidence: 99%

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

2019

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Aim: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of a periodic fever in childhood. The exact pathogenesis and the aetiology of PFAPA are still unknown. Methods:We conducted a non-systematic review of published articles about PFAPA syndrome and summarised the evidence for diagnostic criteria and treatment options for PFAPA.Results: The first proposed diagnostic criteria for PFAPA, in addition to periodic fever, included aphthous stomatitis, pharyngitis or cervical lymphadenitis in children younger than five years at the beginning of the symptoms. C-reactive protein (CRP) levels and leucocyte counts increase in most patients during episodes. Recent research reveals that tonsillectomy provides an immediate and long-lasting cure for PFAPA, even in the absence of classic criteria of aphthous stomatitis, pharyngitis or cervical adenitis and in children older than five years. Conclusion:We suggest that PFAPA can be diagnosed in children with at least five regularly occurring fever episodes without any other explanation, even in the absence of aphthous stomatitis, pharyngitis or cervical lymphadenitis and also in children older than five years. Key notesAfter the introduction of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) in 1987, cumulating research evidence has clarified clinical features of the syndrome. Tonsillectomy provides an immediate and long-lasting cure for PFAPA, even in the absence of classic criteria of stomatitis, pharyngitis or cervical adenitis and in children older than five years. It is time to reconsider the diagnostic criteria for PFAPA in future research and in clinical practice.

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Section: Resultsmentioning

confidence: 99%

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

2019

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“…A smaller scale study of 12 patients found a 75% resolution rate following tonsillectomy [9]. In a 2010 meta-analysis of 374 patients examining treatment resolution rates, no immediate advantage could be found when directly comparing tonsillectomy and steroids, but tonsillectomies in general led to better long-term resolution rates [34]. In a recent study of 23 patients, 91% of patients had complete resolution of PFAPA symptoms immediately after surgery, with 100% of patients achieving resolution within 3 months [48].…”

Section: Resultsmentioning

confidence: 98%

“…The initial management of febrile episodes is oral corticosteroids, which can resolve symptoms within 6 hours, but do not prevent future episodes and can shorten the interval between episodes [2,[4][5][6][7]9,[33][34][35]. The histamine type 2 receptor antagonist cimetidine has also been used preventively but has recently been shown to have inconsistent febrile episode resolution rates [3,15,24].…”

Section: Research Articlementioning

confidence: 99%

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

J¹,

Jonathan²,

Lee³

et al. 2018

Int J Aller Medications

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Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is characterized by periodic cyclical fever, cervical adenopathy, pharyngitis, and aphthous stomatitis. Administration of oral corticosteroids at the onset of fever is the current initial treatment. Tonsillectomy resolves PFAPA symptoms in 50-100% of patients. Objective: The goal of this retrospective observational study was to characterize aspects of PFAPA patients seen at a medium size children's hospital between 2008 and 2016 and evaluate treatment outcomes.

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“…17 Yazgan et al randomized 41 patients with PFAPA to either 2 mg/kg/day or 0.5 mg/kg/day of prednisone, showing that the lower dose was as effective as the higher dose. 55 Tasher et al found that nearly 90% of their patients with PFAPA promptly responded to a corticosteroid, but they also reported a shortening of the episodes in nearly 20% of subjects. 21 Feder initially proposed cimetidine for prevention (not therapy) of episodes in patients with PFAPA.…”

Section: The Journal Of Pediatrics • Wwwjpedscom Volume 193mentioning

confidence: 97%

“…Exploratory therapies have included glucocorticoids, cimetidine, colchicine, IL-1 receptor inhibition with anakinra, and tonsillectomy. 17,18,21,22,25,[27][28][29][55][56][57][58][59] Most series report prompt responses to corticosteroids. Feder and Salazar 18 reported that a corticosteroid was highly effective in aborting fever episodes, but one-half of treated patients experienced more frequent episodes after use.…”

Section: The Journal Of Pediatrics • Wwwjpedscom Volume 193mentioning

confidence: 99%

The First International Conference on Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

Harel

Hashkes

Lapidus

et al. 2018

The Journal of Pediatrics

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Table I. The major autoinflammatory syndromes FMF Mevalonate kinase deficiency, formerly hyperimmunoglobulinemia D and periodic fever syndrome Tumor necrosis factor associated periodic syndrome Cryopyrin-associated periodic syndromes Familial cold autoinflammatory syndrome Muckle-Well syndrome Neonatal onset multisystem inflammatory disease or Chronic infantile neurological cutaneous and articular syndrome Deficiencies in receptor antagonists Deficiency in IL-1 receptor antagonist Deficiency in IL-36 receptor antagonist Interferonopathies Nakajo-Nishimura syndrome Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome Joint contractures, muscular atrophy, microcytic anemia, and panniculitis-induced lipodystrophy syndrome Stimulator of interferon genes associated vasculopathy with onset in infancy Aicardi Goutières syndrome Others PFAPA Chronic recurrent multifocal osteomyelitis Pyogenic arthritis, pyoderma gangrenosum, acne syndrome Blau syndrome/early-onset sarcoidosis Deficiency of IL-10 or IL-10 receptor Deficiency of adenosine deaminase 2

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Comparison of conventional and low dose steroid in the treatment of PFAPA syndrome: Preliminary study

Cited by 36 publications

References 13 publications

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

The First International Conference on Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

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