Early Tonsillectomy for Dual Remission of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome and Immunoglobulin A Nephropathy: A Case Report.
Despite sharing tonsillar immune dysregulation as a common pathogenic mechanism, concurrent presentation of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome with immunoglobulin A (IgA) nephropathy (IgAN) remains exceptionally rare, with only 1 previous case reported in the literature receiving multidrug immunosuppressive therapy before tonsillectomy. In this study, we report the first pediatric case in which both conditions achieved sustained remission following early tonsillectomy without additional immunosuppression. A 6-year-old boy with a 3-year history of PFAPA developed gross hematuria coinciding with febrile episodes. Kidney biopsy confirmed IgAN. Owing to the poor response to cimetidine and significant disruption to schooling, palatine tonsillectomy was performed at 7 years. Following surgery, PFAPA episodes completely resolved, and hematuria ceased. Repeat kidney biopsy at 11 months postoperatively showed marked histological improvement, with resolution of mesangial hypercellularity and reduced endocapillary proliferation. This case provides the first pediatric case demonstrating that early tonsillectomy alone can effectively treat coexisting PFAPA and IgAN without the need for additional immunosuppressive therapy. The simultaneous clinical and histological remission supports a shared pathogenic mechanism involving tonsillar immune dysfunction and highlights tonsillectomy as a potentially curative intervention for select patients with concurrent tonsil-driven diseases.