Tonsillectomy plus steroid pulse therapy for pediatric IgA nephropathy

Abstract

Recently, it has been shown that a deficiency in the IgA1 hinge region sugar chain participates in the onset of IgA nephropathy. Activated B cells produce IgA1 carbohydrate chain deficient immune complex upon antigen stimulation and this is deposited in mesangial cells, activating complements, macrophages, and mesangial cells and resulting in inflammation. Treatment, stratified according to disease severity, is chosen to control these immunoresponses in patients. Multidrug therapy based on a combination of steroids, immunosuppressants and anticoagulant/fibrinolytic agents has been are used for severe cases and is shown to improve prognosis. On the other hand, tonsillectomy plus steroid pulse therapy does not require the use of immunosuppressants and has been found to be as effective as multidrug therapy when used in primary care, and has also been shown to be effective for steroid-resistant or recurrent cases as well as cases of recurrence after renal transplantation. In future, to establish a treatment that minimizes side effects in consideration of the developmental stage of the patient, it is necessary to undertake multicenter prospective comparative trials using multidrug therapy and tonsillectomy plus steroid pulse therapy.