Henoch-Schoenlein nephritis in adults – clinical features and outcomes in Finnish patients

Rauta, Virpi; Törnroth, T; Grönhagen‐Riska, Carola

doi:10.5414/cnp58001

Cited by 43 publications

(30 citation statements)

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“…This finding is in contrast to the results from several previous studies showing that a large amount of proteinuria predicts adverse outcomes. 10,11,32 Interestingly, Coppo et al 9 suggested that proteinuria at the onset of Henoch-Schö nlein purpura nephritis reflects acute inflammation and potentially reversible damage, thus decreasing its predictive value on long-term renal survival. This hypothesis could explain the lack of association between baseline proteinuria and renal outcome in our study.…”

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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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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“…Our patients can be regarded as having a positive outcome despite severe clinical features, as significant proteinuria (41 g/day) and renal impairment were absent during follow-up, both of which are closely predictive of end-stage renal disease. [11][12][13][14] One possible explanation for this positive outcome is the use of renin-angiotensin system blockers in nearly all patients. A number of studies have shown a renoprotective effect for angiotensin-converting enzyme inhibitors and angiotensin receptor blockers in IgA nephropathy.…”

Section: Discussionmentioning

confidence: 98%

Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schönlein Purpura

Pillebout¹,

Alberti

Guillevin

et al. 2010

Kidney International

136

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Henoch Schönlein Purpura (HSP) is a common disease in children, usually associated with a good prognosis. In adults there are no prospective studies concerning its prognosis or treatment, especially in cases of severe visceral involvement. Here we compared steroid therapy without or with cyclophosphamide co-treatment in adults with severe HSP in a 12-month, multi-center, prospective, open-label trial that treated 54 adults with biopsy-proven HSP including proliferative glomerulonephritis and severe visceral manifestations. All received steroids; however, 25 were randomized to also receive cyclophosphamide. The primary endpoint that occurred in three patients in each group was complete disease remission defined as zero on the Birmingham Vasculitis Activity Score with no persistent or new clinical and/or biological vasculitis at 6 months. No patient had active visceral involvement. The secondary endpoints were renal outcome, deaths, and adverse events at 12 months. Renal function, proteinuria, safety data, incidence of diabetes, and severe infections were similar between the two groups. At the last follow-up, renal function remained stable. The small population size of our study does not permit definitive conclusions; however, we suggest that treatment of adults with severe HSP by adding cyclophosphamide provides no benefit compared with steroids alone.

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“…[8] Others have examined the predictive value of clinical factors, such as age, sex, serum parameters, biopsy results, and urinalysis, but the results have been mixed. [9][10][11][12] Although the mechanism remains unknown, there appears to be a correlation between purpuric rash on the trunk and renal involvement in patients with HSP. Lower extremity purpura is often considered the sine qua non of cutaneous involvement in HSP, but truncal purpura has been reported in 37-58% of adults and 11-60% of children.…”

Section: Discussionmentioning

confidence: 99%

Periumbilical Purpura Prior to Gastrointestinal Involvement in Henoch-Schönlein Purpura

Brown

Haught

English

2009

American Journal of Clinical Dermatology

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Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving the skin, joints, gastrointestinal tract, and kidneys. While HSP is traditionally considered a disease of children, it also occurs in adults. In both populations, acute gastrointestinal complications and chronic renal involvement are major predictors of mortality. While it has been previously demonstrated that truncal lesions in HSP are associated with underlying renal involvement, a significant proportion of cases with gastrointestinal involvement also have associated truncal lesions. It is unclear at this time whether or not the association of truncal involvement with visceral disease is related to an increased burden of circulating immune complexes or a more specific mechanism. We describe a report of periumbilical purpura antecedent to gastrointestinal involvement in an adult with HSP.

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Henoch-Schoenlein nephritis in adults – clinical features and outcomes in Finnish patients

Cited by 43 publications

References 0 publications

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

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

Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schönlein Purpura

Periumbilical Purpura Prior to Gastrointestinal Involvement in Henoch-Schönlein Purpura

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