Henoch-Schönlein purpura (HSP) and IgA nephropathy (IgAN) are both IgA1-related vasculitis caused by vascular deposition of IgA1-containing immune complexes. A pathological role of the tonsils in the development of HSP and IgAN has been suggested. Tonsils are a mucosaassociated lymphoid organ. Since oral and sinonasal cavities are anatomically directly connected to the tonsils, delivering exogenous antigens into the tonsils to induce local and systemic antibody responses, we examined the infectious status of these cavities when we treated HSP and IgAN. In 40 HSP children (6.7±2.5 years), apical periodontitis, rhinosinusitis, and otitis media were identified in 21 (53%, 4.9±2.8 affected teeth), 19 (48%), and 4 (10%) of them, whereas in 11 IgAN children (10.4±2.5 years), these diseases were observed in 6 (55%, 5.8±4.6 affected teeth), 2 (18%), and 0 (0%) of them, respectively. We first treated the patients with extensive eradiation of infectious foci, including antimicrobial treatment and root canal therapy. In 31 HSP patients, such dental and/or ENT therapy resulted in a complete cure without development of nephritis or recurrent attacks. For the remaining 9 HSP and 11 IgAN patients, we further performed tonsillectomy plus methylprednisolone (MP) pulse therapy to control their intractable symptoms, including aggravated purpura, recurrent HSP attacks or nephritis. Using this therapeutic strategy, all of the HSP patients attained clinical remission. All of the IgAN patients with various histological grades also achieved normalization of urinalysis by 7.2±5.7 months after the start of steroid therapy. No relapses were observed in both diseases during followup for 2-10 years. In pediatric HSP and IgAN, apical periodontitis and rhinosinusitis may be involved in abnormal immune responses in both the tonsils and whole body. We conclude that extensive elimination of these infectious foci is beneficial to optimize the effect of tonsillectomy plus MP pulse therapy.
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