Pathogenesis of Henoch-Schönlein purpura nephritis

Lau, Keith K.; Suzuki, Hitoshi; Novák, Jan; Wyatt, Robert

doi:10.1007/s00467-009-1230-x

Cited by 133 publications

(127 citation statements)

References 82 publications

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“…[4][5][6] Similar to IgA nephropathy, increased levels of galactosedeficient IgA1 (Gd-IgA1) are detected in patients with Henoch-Schönlein purpura complicated by nephritis, and Gd-IgA1 is now believed to have a pivotal role in the pathogenesis of both IgA nephropathy and Henoch-Schö nlein purpura nephritis. 15,25 In addition, IgA-containing complexes have been found in the glomeruli of IgA nephropathy and Henoch-Schö nlein purpura nephritis patients. Furthermore, we previously showed that HenochSchö nlein purpura nephritis and IgA nephropathy had comparable prognoses when matched at presentation using propensity score analysis.…”

Section: Discussionmentioning

confidence: 99%

Using the Oxford classification of IgA nephropathy to predict long-term outcomes of Henoch–Schönlein purpura nephritis in adults

et al. 2014

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Recently, there has been emerging concern that crescents, the main histologic feature of Henoch-Schö nlein purpura nephritis, merely reflect active inflammation, and may not be useful in predicting long-term outcomes. We therefore conducted a single-center retrospective study to evaluate whether the new Oxford classification of immunoglobulin A nephropathy can be used to predict long-term outcome in patients with Henoch-Schö nlein purpura nephritis. We included 61 biopsy-proven patients with Henoch-Schö nlein purpura nephritis between January 1991 and August 2010. In addition to the International Study of Kidney Disease in Children classification, pathologic findings were also evaluated by the Oxford classification. Primary outcomes were defined as either the onset of estimated glomerular filtration rate o60 ml/min per 1.73 m 2 with Z30% decrease in estimated glomerular filtration rate from baseline or end-stage renal disease. During a median follow-up of 49.3 months, 13 (21%) patients reached the primary end point. A Kaplan-Meier plot showed that renal event-free survival was significantly longer in patients with o50% crescents than in those with crescents in Z50% of glomeruli (P ¼ 0.003). Among the components of the Oxford classification, patients with endocapillary hypercellularity (E1; P ¼ 0.016) and tubular atrophy/interstitial fibrosis (T1/T2; P ¼ 0.018) had lower renal survival rates than those with E0 and T0. In a multivariate Cox model adjusted for clinical and pathologic factors, E1 (hazard ratio ¼ 8.91; 95% confidence interval ¼ 1.47-53.88; P ¼ 0.017) and T1/T2 (hazard ratio ¼ 8.74; 95% confidence interval ¼ 1.40-54.38; P ¼ 0.020) were independently associated with reaching a primary outcome, whereas the extent of crescentic lesions was not. Our findings suggest that the Oxford classification can be used in predicting long-term outcomes of Henoch-Schö nlein purpura nephritis.

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confidence: 99%

Using the Oxford classification of IgA nephropathy to predict long-term outcomes of Henoch–Schönlein purpura nephritis in adults

et al. 2014

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“…The annual incidence of HSP has been reported to be 13.5 to 22.1 per 100,000 9−11) . While HSP is often a self -limited condition, approximately 40% of pediatric patients develop nephritis within 4 to 6 weeks of the initial presentation 12) . An age of more than 10 years at onset, persistent purpura, severe bowel angina, and relapse were identified as factors for the nephritis of HSP 13) .…”

Section: Discussionmentioning

confidence: 99%

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

Ushijima

Kawasaki

Kaneko

et al. 2010

FJMS

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: Henoch -Schoenlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries with IgA immune complexes deposition, and about 7% of patients with HSP experience recurrence. Most patients with recurring of HSP nephritis show a recurrence of clinical symptoms over a period ranging from 2 to 5 months, even after the disappearance of initial symptoms. Here we report a 9 -year -old girl diagnosed with recurrent HSP and severe crescentic glomerulonephritis 3 years after complete resolution of the initial symptoms of HSP. Our case is unique in respect of the recurrence at more than 3 years after the complete resolution of initial symptoms, suggesting that careful followup is required in spite of improved renal symptoms in cases of HSP.

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“…Outros tratamentos específicos vão depender do surgimento de complicações como anemia mais severa, intussuscepção, hipertensão arterial sistêmica, síndrome nefrótica, insuficiência renal, artrite severa ou alterações de sensório. 4,6 O prognóstico a curto e longo prazo geralmente é excelente. Dois terços das crianças não apresentam novos episódios, o restante tem pelo menos uma recorrência normalmente quatro meses após a apresentação inicial, de menor intensidade e/ou duração.…”

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“…Menos de 1% desenvolve complicações a longo prazo, quando desenvolve geralmente é doença renal. 4 A PHS é uma vasculite leucocitoclástica associada a depósitos de IgA. Quanto à patogênese, fatores genéti-cos, ambientais e imunológicos parecem estar envolvidos, mas a causa subjacente permanece desconhecida.…”

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“…Estas manifestações clínicas podem se desenvolver ao longo de dias ou semanas e podem variar na ordem de apresentação. 2,4 O diagnóstico geralmente é baseado nas manifestações clínicas, principalmente quando o quadro é clássico, como o caso em questão, com púrpura palpável em extremidades e nádegas. Nos pacientes com apresentações incompletas ou incomuns a biópsia de um órgão afetado, como pele ou rim, confirma o diagnósico.…”

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