Pfapa syndromeE85.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 09.07.2022

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HistoryThis section has been translated automatically.

Marshall, 1987

DefinitionThis section has been translated automatically.

Most frequent, non-heritable or only sporadically inherited, periodic fever syndrome in children in our latitudes PFAPA is an acronym that summarizes the main symptoms of the disease: periodic fever, aphthae with stomatitis, pharyngitis and lymphadenitis.

EtiopathogenesisThis section has been translated automatically.

Unclear, family disposition does not seem to exist.

ManifestationThis section has been translated automatically.

Infancy; childhood

Clinical featuresThis section has been translated automatically.

The disease typically begins in early childhood with recurrent fever attacks (often > 39 °C), non-exudative pharyngitis, cervical lymphadenopathy, aphthae (similar in aspect to habitual aphthae). The fever episodes last on average 3-6 days and recur with almost fixed periodicity of 2-6 weeks (mostly about 3-4 weeks). Often the parents describe: "I can set the clock accordingly". Between the fever episodes, there is freedom of appearance.

LaboratoryThis section has been translated automatically.

During the fever episodes, the inflammatory signs common to all periodic fever syndromes, such as leukocytosis, elevated CRP (50-150 mg/l) and accelerated BSG (30-60 mm/hr), are found in the laboratory. Elevated serum IgG levels have been described in isolated cases.

DiagnosisThis section has been translated automatically.

There are no specific parameters for the diagnosis of PFAPA, so it is a pure exclusion diagnosis.

Differential diagnosisThis section has been translated automatically.

Differentiation from other forms of periodic fever such as Familial Mediterranean fever; Muckle-Wells syndrome; Tumor necrosis factor receptor-associated periodic syndrome (TRAPS); Hyper IgD syndrome (HID); Familial cold urticaria.

Differentiation from other diseases associated with aphthous changes, especially Behçet 's disease and habitual aphthae.

TherapyThis section has been translated automatically.

The therapy is symptomatic and is based on a single administration of cortisone (prednisone 2 mg/kg bw) at the beginning of the febrile episode. With this therapy, the acute attack can be stopped, but the result can also be a shortened fever-free interval. Long-term but slow improvement can be achieved in 30% of cases with cimetidine in therapeutic doses (administered for a period of 6 to 12 months).

If cimetidine remains ineffective, tonsillectomy with simultaneous adenotomy is another therapeutic option (it is recommended by most authors). Studies in this area, however, with only a very small number of cases, show a positive curative effect in about 72% of patients.

Progression/forecastThis section has been translated automatically.

Favorable; prolongation of fever-free intervals, then remission.

LiteratureThis section has been translated automatically.

  1. Atas B et al (2003) PFAPA syndrome mimicking familial Mediterranean fever: report of a Turkish child. J Emerg Med 25: 383-385
  2. Berlucchi M et al (2003) Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature. Ann Otol Rhinol Laryngol 112: 365-369
  3. Galanakis E et al (2002) PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 86: 434-435
  4. Parikh SR et al (2003) Utility of tonsillectomy in 2 patients with the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Arch Otolaryngol Head Neck Surgery 129: 670-673
  5. Lee WI et al (1999) PFAPA syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis, adenitis). Clin Rheumatol 18: 207-213
  6. Marshall GS et al (1987) Syndromes of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatrics 110: 43-46

Authors

Last updated on: 09.07.2022

Author: Prof. Dr. med. Peter Altmeyer