Periodic Fever Aphthous Stomatitis Pharyngitis Adenitis – PFAPA Syndrome: Part 1

PFAPA is a common autoinflammatory syndrome in childhood. The onset is usually before 5 years of age and attacks typically cease by 10 years of age in most patients, though a minority of patients continue to have episodes till 2nd decade also. It is an autoinflammatory disease characterized by attacks of seemingly unprovoked inflammation and are distinguished from autoimmune disorders by the lack of significant levels of either autoantibodies or autoreactive T cells. 

CLINICAL SYMPTOMS

Fever — Begins abruptly, for two to seven days and then falls also abruptly. Prolonged fever episodes should prompt consideration of other diagnoses. Fever episodes typically occur every two to eight weeks. The regularity in timing of fever flares is a cardinal feature of PFAPA. This type of fever pattern is distinct from prolonged fever or fever of unknown origin in which a single illness leads to a protracted, continuous or near continuous period of fever. 

Aphthous ulcers — Seen in 40 to 80 percent of patients. Occasionally, ulcerations are seen in the posterior pharynx. Aphthous ulcers in PFAPA are not very large or painful and they do not scar.

Pharyngitis — Seen in 65 to 100 percent of patients. Exudates are often seen, may be unilateral, and may be associated with ulcerations on the palatine tonsils.

Adenitis — Cervical adenopathy seen in 60 to 100 percent of patients. The adenopathy may be tender.

DIAGNOSIS

PFAPA is a diagnosis of exclusion that is made on clinical grounds. Prior to recognition of the periodicity, episodes are usually ascribed to infectious tonsillitis or a viral syndrome. The primary reasons for making this diagnosis are to relieve parental anxiety, avoid repeated laboratory tests or empirical treatment with antimicrobials every time.

There is no diagnostic laboratory test for PFAPA syndrome.

  • Presence of any one i.e. Adenitis/Pharyngitis/Stomatitis is also sufficient if other clinical and laboratory parameters are very suggestive in a case.
  • Symptoms with each episode are nearly identical
  • recurrence with 3 or more episodes(with regular intervals),  is usually sufficient to suspect the diagnosis.
  • Moderate leukocytosis and elevation of ESR and CRP usually present during episodes but normalize between attacks. Neutrophilia, monocytosis and mild lymphopenia are seen. 
  • Throat Cultures do not isolate any pathogenic bacteria.
  • Procalcitonin concentrations do not increase proportionally to the increase in other acute-phase reactants, such as CRP and this may help discriminate PFAPA attacks from acute bacterial infections. 
  • Absence of signs of respiratory tract infection during flares and exclusion of cyclic neutropenia, other known periodic fever syndromes, immunodeficiency, or autoimmune diseases is important.
  • Resolution of symptoms (fever and pharyngitis) within a matter of hours after treatment with a 1 to 2 mg/kg oral dose of prednisone given as a single dose or two doses 12 to 48 hours apart. The diagnosis should be reconsidered if fever recurs after the patient receives a total of 2 mg/kg prednisolone/prednisone per episode.
  • Between febrile episodes, children with PFAPA are healthy and have normal growth and development.

Next Post: What is not PFAPA & Treatment Options

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