What are the management options for a patient with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome?

PFAPA Syndrome Management

Definitive Treatment Recommendation

Tonsillectomy (with or without adenoidectomy) is the most effective definitive treatment for PFAPA syndrome, achieving complete symptom resolution in the vast majority of patients and should be strongly considered for those with significant quality of life impact, persistent symptoms, or history of peritonsillar abscess. 1, 2, 3

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When to Consider Tonsillectomy

The American Academy of Otolaryngology-Head and Neck Surgery recognizes PFAPA as a modifying factor that favors tonsillectomy even when patients don't meet standard frequency criteria for recurrent throat infections (i.e., <7 episodes in past year, <5 episodes/year for 2 years, or <3 episodes/year for 3 years). 1, 3

Specific indications for tonsillectomy include:

• Significant impact on quality of life (school absences, family disruption) 2
• History of more than one peritonsillar abscess 1, 3
• Patient/family preference for definitive solution 2
• Persistent symptoms despite medical management 2

Surgical outcomes are excellent: Complete symptom resolution occurs in 96-100% of patients (26 of 27 patients in one series, 18 of 18 in another). 4, 5 Meta-analysis confirms tonsillectomy is significantly more effective than antibiotics or cimetidine for long-term resolution. 4

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Medical Management Options

Acute Episode Treatment

Corticosteroids are the most effective medical therapy for acute episodes:

• Single-dose prednisone reduces symptoms in 94% of patients during acute flares 6
• Provides rapid symptom relief (often within hours) 6
• Critical limitation: Does NOT prevent future fever cycles—only treats individual episodes 4, 6

Analgesics/antipyretics for symptom control:

• Acetaminophen or ibuprofen for fever and pain 3
• Avoid aspirin in children due to Reye's syndrome risk 2

Prophylactic Medical Therapy

Colchicine may be considered for prophylaxis:

• Can significantly decrease flare frequency 7
• Particularly useful in adult-onset cases or when surgery is declined 7

Ineffective therapies to avoid:

• Antibiotics are ineffective for PFAPA 4, 6
• Cimetidine shows no benefit 4

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Diagnostic Workup Essentials

Document each episode meticulously:

• Temperature readings 2
• Specific symptoms present (fever, aphthous stomatitis, pharyngitis, cervical adenitis) 2, 3
• Episode duration and interval between episodes 2
• Days of school absence and quality of life impact 2

Laboratory testing:

• Throat culture to rule out Group A Streptococcal infection 2, 3
• Do NOT order anti-streptococcal antibody titers (reflect past, not current infection) 2
• Do NOT test asymptomatic household contacts 2

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Critical Pitfalls to Avoid

Do not confuse PFAPA with recurrent streptococcal pharyngitis:

• PFAPA patients may be chronic streptococcal carriers 2
• They can also experience concurrent viral infections 2
• Diagnosis is clinical based on pattern: regularly recurring episodes with predictable intervals 3, 6

If proceeding to tonsillectomy:

• Do NOT prescribe perioperative antibiotics—this is strongly recommended against by current guidelines 1
• Administer single intraoperative dose of IV dexamethasone 1
• Use ibuprofen, acetaminophen, or both for postoperative pain control 1

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Treatment Algorithm

Step 1: Confirm diagnosis

• Regular fever episodes with aphthous stomatitis, pharyngitis, and/or cervical adenitis 3
• Rule out streptococcal infection with throat culture 3
• Document pattern over time 2

Step 2: Assess severity and impact

• Frequency of episodes 2
• School absences and quality of life disruption 2
• History of peritonsillar abscess 1, 3
• Patient/family preferences 2

Step 3: Choose treatment pathway

For mild disease with minimal impact:

• Episodic prednisone for acute flares 6
• Consider colchicine prophylaxis if frequent episodes 7

For moderate-to-severe disease or significant quality of life impact:

• Proceed directly to tonsillectomy 2, 4, 5
• This provides definitive cure in >95% of cases 4, 5

Step 4: Monitor and follow-up

• Regular clinical visits to assess episode frequency and severity 2
• Reassess need for surgical intervention if medical management fails 2

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Comparative Effectiveness

Meta-analysis demonstrates that surgery versus steroids shows no statistically significant difference in effectiveness (P=0.83), confirming both are highly effective. 4 However, the key distinction is that steroids only treat individual episodes without preventing future cycles, while tonsillectomy provides permanent resolution. 4, 6 Given the benign nature of tonsillectomy in pediatric patients and the burden of chronic episodic illness, surgery represents the superior long-term solution for most patients. 4, 5