Abstract
There is no consensus-based treatment for adult-onset immunoglobulin A vasculitis with nephritis (IgAV nephritis). Tonsillectomy is a treatment option for primary IgA nephropathy, which has similar histopathological features and pathogenesis to IgAV nephritis. The present case series aimed to describe the clinical course of patients with IgAV nephritis who underwent tonsillectomy in our institution. Adult patients with biopsy-proven IgAV nephritis who received tonsillectomy from 2015 to 2022 were systematically reviewed at six hospitals in Japan. Hematuria, proteinuria and slope of the estimated glomerular filtration rate (eGFR) were evaluated before and after tonsillectomy. Patients with IgAV nephritis who underwent tonsillectomy was identified in 12 of 2626 kidney biopsies performed during the study period. The median observation periods before and after tonsillectomy were 20.7 and 48.6 months, respectively. The following drugs were administered concurrent with tonsillectomy: corticosteroids (n = 8), mizoribin (n = 1), and rituximab (n = 1). Three patients were not treated with corticosteroids or immunosuppressants. During post-tonsillectomy observation, 5 patients showed remission of hematuria. Of the 10 patients whose proteinuria was not at a remission level prior to tonsillectomy, 7 showed remission of proteinuria after tonsillectomy. The eGFR slope was attenuated in 9 patients after tonsillectomy relative to before tonsillectomy. This study suggests that some patients may benefit from tonsillectomy in the treatment of IgAV nephritis. The efficacy of tonsillectomy or combination therapy with immunosuppression for IgAV nephritis requires further case series to clarify the clinicopathologic picture of patients associated with a response to tonsillectomy.