Henoch Schonlein Purpura – A 5-Year Review and Proposed Pathway

Abstract

Henoch Schonlein Purpura (HSP) is the commonest systemic vasculitis of childhood typically presenting with a palpable purpuric rash and frequently involving the renal system. We are the first group to clinically assess, critically analyse and subsequently revise a nurse led monitoring pathway for this condition.A cohort of 102 children presenting with HSP to a secondary/tertiary level UK paediatric hospital over a five year period, were monitored using a nurse led care pathway. Using this cohort, the incidence (6.21 cases per 100,000 children per year) and natural disease course of HSP nephritis (46% initial renal inflammation; 9% subsequent renal referral; 1% renal biopsy and immunosuppression) was determined. Older patients were at higher risk of requiring a renal referral (renal referral 12.3 (8.4–13.5) years vs. normal outcome 6.0 (3.7–8.5) years; p<0.01). A normal urinalysis on day 7 had a 97% (confidence interval 90 to 99%) negative predictive value in predicting a normal renal outcome.Using this data and existing literature base, The Alder Hey Henoch Schonlein Purpura Pathway was developed, a revised pathway for the screening of poor renal outcome in HSP. This is based on a six-month monitoring period for all patients presenting with HSP, which importantly prioritises patients according to the urine findings on day 7 and thus intensively monitors those at higher risk of developing nephritis. The pathway could be easily adapted for use in different settings and resources.The introduction of a standardised pathway for the monitoring of HSP will facilitate the implementation of disease registries to further our understanding of the condition and permit future clinical trials.

Analysis of 135 Cases of Pediatric Henoch-Schönlein Purpura in Gansu, Northwestern China

“…The degree of renal damage is an important factor in determining the longterm prognosis of HSP. 20% to 54% of children with HSP may develop renal involvement (36,37). However, 11% of children in our study had renal involvement which is relatively low.…”

Unusual Eruptions Associated with <b><i>Mycoplasma pneumoniae</i></b> Respiratory Infections: Review of the Literature

“…We found 14 case series addressing Henoch-Schönlein syndrome in a total of 2,186 cases published in English-language journals between 1984 and 2014 [63,64,65,66,67,68,69,70,71,72,73,74,75,76]. None of the reports included cases associated with M. pneumoniae respiratory infection.…”

Prevention of renal disease in Henoch-Schonlein purpura: clear evidence against steroids

“…The Alder Hey study4 reported similar numbers: over a period of 5 years, 102 children presenting with HSP were followed and the natural course of the disease described. Initial renal inflammation was seen in 46%, subsequent renal referral in 9%, with 1% undergoing a renal biopsy and receiving immunosuppressive treatment.…”