ABSTRACT:We report a case of recurrent Henoch-Schönlein purpura nephritis (HSPN) treated successfully with a tonsillectomy and steroid pulse therapy in a kidney transplant patient. A 29-year-old woman was admitted to our hospital for an episode biopsy; she had a serum creatinine (S-Cr) of 1.0 mg/dL and 1.34 g/day proteinuria 26 months after kidney transplantation. Histological examination revealed increased amounts of mesangial matrix and mesangial hypercellularity with IgA deposition. Of note, one glomerulus showed focal endocapillary proliferation and tuft necrosis. We diagnosed active recurrent HSPN. Considering both the histological findings and refractory clinical course of the native kidney, she was treated for 3 consecutive days with steroid pulse therapy and a tonsillectomy. The patient's proteinuria decreased gradually to less than 150 mg/day 6 months later. A second biopsy 6 years after kidney transplantation showed an excellent response to treatment and revealed a marked reduction in both the mesangial matrix and mesangial hypercellularity, with trace IgA deposition. We conclude that a tonsillectomy and steroid pulse therapy appeared to be useful in this patient with active recurrent HSPN. This paper is the first to report a tonsillectomy and steroid pulse therapy as a therapeutic option for active recurrent HSPN. Further studies are needed to elucidate the efficacy and mechanisms of tonsillectomy with recurrent HSPN in kidney transplant patients.Henoch-Schönlein purpura is a systemic small vessel vasculitis thought to be mediated by IgA immune complex deposition (IgA vasculitis). Henoch-Schönlein purpura affects the kidney in 20-80% of patients and most patients have a good prognosis. However, 3-20% of Henoch-Schönlein purpura nephritis (HSPN) has a poor outcome with a higher incidence of endstage renal disease, especially in adults. [1][2][3] Compared with IgA nephropathy, relatively little is known about recurrent HSPN in renal allografts. The recurrence rate ranges from 15% to 53%, and graft loss due to recurrent HSPN was 7.5% to 21% in different observation periods. 4 Therapy for severe HSPN usually involves methylprednisolone pulse therapy with oral corticosteroids, immunosuppressive agents, antiplatelet drugs, and plasmapheresis. Tonsillectomy has also been used in some HSPN patients, with a marked improvement in renal function and proteinuria, 3,5 but its benefit in the setting of recurrent disease in a renal allograft has not been reported. To our knowledge, this is the first report of the efficacy of tonsillectomy and steroid pulse therapy in a kidney transplant patient who developed active HSPN.
CASE REPORTA 29-year-old Japanese woman was admitted to our hospital for an episode biopsy 26 months after kidney transplantation. She first presented with purpura on her lower legs, abdominal pain, macrohematuria, and proteinuria (0.5-1.0 g/ day) at 15 years of age, at which time a renal biopsy was performed and the histology revealed focal segmental