TOPLINE

In children presenting with recurrent oral aphthosis, the presence of genital aphthosis during initial evaluation was associated with an increased likelihood of a systemic or chronic diagnosis. Recurrent fever and tonsillitis or pharyngitis were independently associated with lower odds of systemic disease.

METHODOLOGY

• Researchers conducted a retrospective cohort study of 404 children referred to a pediatric rheumatology clinic in Italy for recurrent oral aphthosis (September 2010-October 2024); children referred for the condition had experienced a minimum of two oral mucosal ulceration episodes in the prior year.
• Patients were monitored with reassessments scheduled quarterly to biannually; diagnostic classification required a minimum of one follow-up assessment following the baseline consultation.
• Researchers examined eight clinical variables recorded during the first rheumatology visit: simultaneous presence of minor and major oral aphthae, genital aphthosis, recurrent fever, lymphadenopathy, tonsillitis or pharyngitis, abdominal pain, arthralgia/arthritis, and changes in bowel habits.
• Patients were divided into groups on the basis of final diagnoses, with group 1 comprising benign or self-limited diagnoses (n = 267) and group 2 comprising systemic or chronic or incompletely classified diagnoses (n = 137); periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis dominated in group 1 (n = 169), whereas Behçet disease and Behçet-like disease dominated in group 2 (n = 30 and 50, respectively).
• Associations between baseline clinical features and diagnostic category were assessed, with group 2 as the outcome.

TAKEAWAY

• Genital aphthosis at baseline was associated with higher odds of group 2, which included Behçet disease, Behçet-like disease, familial Mediterranean fever, juvenile idiopathic arthritis, celiac disease, and other conditions (adjusted odds ratio [OR], 13.08; P < .001). 
• Among group 2 patients with genital aphthosis, 22 of 23 had Behçet disease or Behçet-like disease. 
• Recurrent fever and tonsillitis or pharyngitis were associated with reduced odds of group 2 (adjusted OR, 0.25; P < .001 and adjusted OR, 0.17; P < .001, respectively).
• Among 218 patients tested for HLA-B51, positivity was more frequent among patients in group 2 than among those in group 1 (40.8% vs 4.8%; OR, 13.81; P < .001).

IN PRACTICE

"Baseline manifestations present at the first rheumatology assessment were associated with the diagnostic category during follow-up," the authors wrote. "These are adjusted associations, not a validated prediction model," they added.

SOURCE

The study was led by Rosario Francesco Dipasquale, Magna Graecia University of Catanzaro, Catanzaro, Italy. It was published online on September 15 in the European Journal of Pediatrics.

LIMITATIONS

The precise follow-up time for each individual could not be accurately determined, possibly missing subsequent development of pediatric Behçet phenotypes. Demographic information about patients was not retained, preventing evaluation of how age variations affected the observed patient composition. 

DISCLOSURES

The authors reported receiving no specific funding for the study and having no relevant conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.