Plasmapheresis therapy for rapidly progressive Henoch-Sch�nlein nephritis

Abstract

Six Japanese children with rapidly progressive Henoch-Schönlein purpura nephritis (HSPN) received multiple drug therapy combined with plasmapheresis (PP). After five courses of PP, multiple drug therapy, including methylprednisolone and urokinase pulse therapy, oral prednisolone, cyclophophamide, dipyridamole, and warfarin was given. At presentation, urine protein excretion and histological indices of the mean activity and chronicity were 245+/-101 mg/m(2) per hour, 6.6+/-1.2, and 1.5+/-1.3, respectively. After 6 months of therapy, urinary protein excretion had decreased significantly ( P<0.001). The activity index decreased significantly at the second renal biopsy performed at a mean interval of 4.3 months after the first (2.8+/-1.4, P<0.05), while the chronicity index did not change. At the most recent observation, all showed clinical improvement. Two patients had normal urine, three had proteinuria of <20 mg/m(2) per hour, one had proteinuria of >20 mg/m(2) per hour, and none had renal insufficiency. Although this case series is without controls, our treatment protocol may be of benefit to children with rapidly progressive HSPN.

HENOCH-SCH^|^Ouml;NLEIN PURPURA NEPHRITIS IN CHILDHOOD: PATHOGENESIS, PROGNOSTIC FACTORS AND TREATMENT

“…There have been a number of reports on plasmapheresis (PP) for HSPN in childhood 50,51) . Hattori et al retrospectively evaluated the clinical courses of nine children with a rapidly progressive type of HSPN who were treated with plasmapheresis (PP) as the sole therapy.…”

“…Two patients had normal urine, three had proteinuria of <20 mg/m 2 per hour, one had proteinuria of >20 mg/m 2 per hour, and none showed any renal insufficiency. Although this case series was examined without controls, this treatment protocol may be of benefit to children with rapidly progressive HSPN 51) . The benefits of the abovementioned treatments for treating HSPN deserve to be assessed further in larger randomized controlled trials.…”

A Case of Henoch-Schoenlein Purpura Nephritis Recurring After 3 Years Remission

“…The mechanisms by which PP benefits the patients with severe HSPN remain largely obscure. However, experimental and clinical immunologic findings suggest that the rationale for using PP in the treatment of patients with HSPN relies on the removal of IgA -containing immune complexes or aggregates, or proinflammatory mediators including cytokines and complements ; reduction in fibrinogen or other coagulation factors ; and possibly desaturation of the mononuclear phagocyte system 20,21) . In addition, the rationale for using prednisolone and MZB in case of severe HSPN is that corticosteroids and immunosuppressive agents reduce IgA production and minimize the abnormal immune response and inflammatory events that follow IgA deposition in the glomeruli.…”

Therapeutic plasma exchange for the treatment of pediatric renal diseases in 2013

“…Retrospective studies of children with crescentic HSP nephritis have demonstrated improvement in those treated with TPE alone [77,78] and in combination with medical immunosuppression [79], although no prospective data exist. Neither HSP nor IgA nephropathy is a recommended indication for TPE at this time [3].…”