Prospective study of children with PFAPA

PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) is the most common periodic fever condition in children. Vaccination in this population can be challenging due to concerns about immune hyperreactivity, the potential to trigger febrile episodes, and uncertainty regarding vaccine efficacy. Data on immunisation coverage and vaccine response in PFAPA remain limited. This study aimed to describe the clinical characteristics of children with PFAPA, with a particular focus on vaccination coverage and serological responses.

The team of Dr Géraldine Blanchard-Rohner conducted a prospective study of children with PFAPA followed at Geneva University Hospitals (HUG) between February 2022 and April 2025. Clinical, laboratory, and genetic data were collected, along with vaccination status according to the Swiss national immunisation schedule and serological results for major vaccine antigens.

Forty-one patients were included. All exhibited elevated inflammatory markers during febrile episodes, and none had a monogenic cause identified. Vaccination coverage was high, with 90–95% of patients receiving the primary DTaP-IPV-Hib-HBV series on time. Minor delays were observed for the third pneumococcal and second MMR doses. Meningococcal (MenC/MenACWY) vaccination was administered on time in 58% of patients. Serological testing confirmed protective antibody levels for most antigens, including diphtheria, tetanus, and measles, but only 40% achieved protective pneumococcal titers. Only 65% showed varicella immunity because of past infection, as none had received the vaccine, which was only introduced in Switzerland in 2023.

Children with PFAPA demonstrate high adherence to vaccination schedules and adequate immune responses, supporting the safety and effectiveness of routine immunisation in this group. The reduced pneumococcal seroprotection suggests that monitoring antibody levels and considering booster doses may be warranted. Larger controlled studies are needed to assess vaccine immunogenicity and reactogenicity, particularly for highly reactogenic vaccines such as meningococcal B.

Read the full article: https://doi.org/10.1186/s12969-025-01174-4

WHY IS THIS IMPORTANT?

PFAPA is the most common cause of recurrent fever in young children, yet many parents and clinicians worry that vaccines could trigger fever episodes or be less effective in these patients. This study shows that children with PFAPA are generally well vaccinated and develop protective immune responses to most routine childhood vaccines. Importantly, vaccination coverage was similar to that of the general population, reassuring families and healthcare providers about vaccine safety in PFAPA. The study also identified lower protection against pneumococcal infections in some children, suggesting that closer monitoring or booster doses may be needed. These findings support continuing routine vaccinations in children with PFAPA while helping clinicians better tailor follow-up care to protect this vulnerable paediatric population.

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