WHEN IT IS NOT PFAPA
Patients should be evaluated for the following features since the presence of any of them precludes the diagnosis of PFAPA syndrome:
- Neutropenia – Rule out cyclical neutropenia
- Atypical symptoms – PFAPA attacks have stereotypical symptoms that are easily recognizable by caregivers. The following symptoms as a part of most attacks should trigger evaluation for other diagnoses: cough, coryza, severe abdominal pain, significant vomiting or diarrhea, chest pain, rash, arthritis, or significant neuromuscular symptoms.
- Elevated acute-phase reactants between attacks
- Lack of episode resolution after one or two doses of a glucocorticoid
PFAPA is a self-limited disease with no long-term sequelae. A decision to treat PFAPA is based upon severity and duration of episodes, impact on quality of life, and preference of the child and their family.
One may also chose to use antipyretics only at each recurrence, though there has been no reports of any unacceptable effects with repeated single doses of prednisolone.
Oral Prednisolone : A 1 to 2 mg/kg oral dose of prednisone given as a single dose or two doses 12 to 48 hours apart. In cases, where response was achieved with 2 doses of 1mg/kg each, subsequent episodes can be given a single dose of 2mg/kg/dose in subsequent episodes.
Preventive or prophylactic treatment — Prophylactic therapy (cimetidine or colchicine) is an option in patients who have an increase in frequency of episodes on glucocorticoids.
Tonsillectomy — Tonsillectomy is an effective surgical treatment for many patients with PFAPA, particularly in patients who fail to respond to medical management (glucocorticoids with or without preventive or prophylactic therapy) or have considerable disruption in their lives due to episode frequency.
A Specialist Opinion by a Pediatric Rheumatologist should be obtained if possible before labelling a case.