Can Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome be triggered by dengue fever?

Can Dengue Fever Trigger PFAPA Syndrome?

There is no evidence in the current medical literature to suggest that dengue fever can trigger PFAPA syndrome. While both conditions involve fever and inflammatory responses, they have distinct etiologies, clinical presentations, and management approaches.

Understanding PFAPA Syndrome

PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) is an autoinflammatory condition characterized by:

• Recurrent episodes of high fever
• Aphthous ulcers in the mouth
• Pharyngitis
• Cervical lymphadenopathy

Key characteristics of PFAPA include:

• Primarily affects children under 5 years of age, though adult-onset cases exist 1
• Regular periodicity of fever episodes (typically every 3-8 weeks)
• Patients are completely asymptomatic between episodes
• No specific biomarkers are available for diagnosis 1
• Diagnosis is clinical and requires exclusion of other causes of periodic fever

Dengue Fever vs. PFAPA Syndrome

Dengue fever is a mosquito-borne viral infection caused by the dengue virus, with distinct characteristics:

• Transmitted by Aedes mosquitoes
• Endemic in over 100 countries throughout tropics and subtropics 2
• Presents with fever, headache, myalgia, arthralgia, and rash
• Diagnosed through RT-PCR, NS1 antigen detection, or antibody testing 2
• Can progress to severe forms including dengue hemorrhagic fever and dengue shock syndrome

Why Dengue is Unlikely to Trigger PFAPA

1. Different etiologies:

   • Dengue is caused by a specific virus
   • PFAPA's etiology remains unknown but is considered autoinflammatory with possible genetic factors 3

2. Different clinical patterns:

   • Dengue is an acute infection with a single episode
   • PFAPA shows recurring episodes with regular periodicity and symptom-free intervals 4

3. No documented association:

   • Current medical literature, including the CDC guidelines on dengue fever 5, does not mention any association between dengue infection and subsequent development of PFAPA syndrome

Management Considerations

If a patient presents with symptoms suggestive of PFAPA following dengue infection:

1. Rule out persistent dengue complications:

   • Perform appropriate testing for dengue as recommended by CDC 5
   • Monitor for warning signs including abdominal pain, persistent vomiting, fluid accumulation, mucosal bleeding, lethargy, hepatomegaly, and increased hematocrit with decreasing platelets 2

2. Consider PFAPA diagnosis if:

   • Regular periodicity of fever episodes emerges
   • Complete resolution of symptoms between episodes
   • Characteristic constellation of symptoms (fever, aphthous stomatitis, pharyngitis, cervical adenitis)
   • Other causes of periodic fever have been excluded

3. PFAPA treatment options:

   • Corticosteroids (prednisone 0.6-1 mg/kg/day) are highly effective for acute episodes 4
   • Colchicine may be used for prophylaxis to reduce flare frequency 1
   • Tonsillectomy has shown success in selected patients 6, 4

Clinical Pearls

• PFAPA syndrome typically resolves during adolescence but may persist into adulthood in some cases 6
• The diagnosis of PFAPA is often delayed (average 15 months) due to low awareness among clinicians 4
• Abdominal pain is a common symptom in PFAPA (65% in some studies) but not typically emphasized in diagnostic criteria 4
• Lower doses of corticosteroids than previously thought (as low as 0.15-0.6 mg/kg/day) may be effective in treating acute episodes 4

In conclusion, while both dengue fever and PFAPA syndrome involve inflammatory processes, there is no established causal relationship between dengue infection and the subsequent development of PFAPA syndrome.