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Henoch Schonlein Purpura A proposed pathway for follow-up. Watson L 1,2 , Richardson A 1 , Holt R.C.L 1 , Jones C.A 1 , Beresford M.W 2 . Departments of Paediatric Nephrology 1 and Rheumatology 2 , Alder Hey Children ’ s NHS Foundation Trust Hospital & Institute of Translational Medicine, - PowerPoint PPT Presentation
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Henoch Schonlein Purpura A proposed pathway for follow-up
Watson L1,2, Richardson A1, Holt R.C.L1, Jones C.A1, Beresford M.W2.Departments of Paediatric Nephrology1 and Rheumatology2, Alder Hey Children’s NHS Foundation
Trust Hospital & Institute of Translational Medicine, University of Liverpool, UK
Henoch Schonlein Purpura• Small vessel vasculitis
• IgA complex, C3 deposition • Arterioles, Capillaries, Venules • Inflammatory neutrophils, monocytes
• Typically presents with rash• Scrotal involvement• Abdominal pain, bleeding, intussusception• Non-erosive arthritis, arthralgia• Renal involvement• Rarely neurological, lung
Diagnosis• More common preschool; 90% <10 years old
• EULAR classification criteria1
• Purpura/petechiae rash
Plus any one of; • Abdominal involvement, • Renal involvement, • Joint involvement (arthritis/arthralgia), • Histological evidence of IgA deposits.
1. Ozen, 2010
• Commonest childhood vasculitis• Incidence 10-20 cases per 100,000 child population2
• (SSNS 3 cases per 100,000; IDDM 207 cases per 100,000)
Average North West DGH; • Catchment population of 60,000 children3
• ≈ 6-12 cases of HSP diagnosed by a DGH/year
Rare for GP population• Average GP 2000 patients, 18% (274) children; 1 case for approx.
every 36 GP’s
Henoch Schonlein Purpura
2. Gardner-Medwin et al, 2002, 3. http://www.ons.gov.uk
HSP nephritis (HSPN)• Seen in up to 40%
– Asymptomatic & only long term consequence– Requires active screening
• Long term outcome of HSPN– Unselected cohorts risk of renal impairment 1%
• Risk rises if nephritic or nephrotic1
• Up to 20% nephrotic range proteinuria
– Cohorts with established HSPN 15-20% ESRF2,3
– Accounts for 1.7% all UK ESRF4
1. Mir et al 2007, 2. Shenoy et al, 2007, 3. Butani et al, 2007, 4. UK Renal Registry 2005
Screening for HSPN
• Screening varies1
– Within a centre, region, national & international• Centre 1: Paediatrician led follow up• Centre 2: GP led follow up ‘uncomplicated cases’
• Screening imposes financial burden, parental anxiety
• Variations also in renal referral process and biopsy indications
1. Weiss P et al J Ped 2009
HSP diagnosis Diagnosis; EULAR criteria
Screening for nephritis
No renal involvement
Renal involvement
Resolved renal involvement
Persistent/resolve
20% ESRF
HSPN
Diagnosis; Renal biopsy ISKDC classification
Evidence-based treatment of HSPNSystematic review of RCTs: no difference
• Early corticosteroids V’s placebo, total n=3791
• Cyclophosphamide V’s supportive, n=56• Cyclosporin V’s methylprednisolone RCT, n=242
Other studies• Cyclophosphamide + methylprednisolone, n=123
• Azathioprine + steroids, n=214
• Cochrane: Few RCTs5
–Sparse data, no proven benefit of treatment• Challenges: self resolving, high risk groups, no
standardised care
1. Tizard et al, unpublished, personal communication; Dudley 2007, Huber 2004, Mollica 2004, Ronkainen 2006.2. Jauhola et al, 2011 3. Flynn et al, 2001 4. Bergstein et al, 1998 5. Chartapisak W et al. 2009
HSP diagnosis Diagnosis; EULAR criteria
Screening for nephritis
No renal involvement
Renal involvement
Resolved renal involvement HSPN
20% ESRFPersistent/resolve
Diagnosis; Renal biopsy ISKDC classification
?
?
?
HSP screening at Alder Hey
• Designed in 2004, multi-disciplinary• Paediatric nurse led• Urine dipstick, blood pressure• Parent education• Hand held records
• Triaged according to urinalysis (day 7)– Intensive (8 visits over 12 months)– Standard (5 visits)
• Total of 12 months monitoring
Aims
Primary•To describe renal involvement in an unselected cohort of children with HSP Secondary•To revise our nurse led HSP monitoring pathway
Primary outcomePrimary outcome;
Need to exit the nurse led pathway for a medical review
Exit criteria (excluding patients from nurse led monitoring)• Hypertension• Urine albumin:creatinine ratio (UACR) > 200mg/mmol• Serum albumin <30g/l• eGFR < 80 ml/min/1.73m2
• Macroscopic haematuria >28 days• 12 months completed monitoring with urine abnormalities
InvestigationsPresence of proteinuria
Presence of exit criteria
HSP coding: Identified n=176
Standard FU: No proteinuria n=80
Intensive FU: Proteinuria n=22
Excluded: Other diagnosis n=11No care pathway n=61
HSP & sufficient data n=10446% renal involvement at diagnosis
DNA n=2Day 7: allocation n=102
Developed proteinuria n=13Moved area n=2
Standard FU (n=65):Outcome n=1 renal; n=64 normal
Intensive FU (n=35): Outcome n=8 renal; n=27 normal
Outcome Discharged n=91; renal n=9
Month 12: outcome n=100
Results
Older patients more likely to develop HSPN
P<0.01
Outcome• Primary outcome; 9 patients required review
– 2 patients early review (<3 months)– 7 patients referred after 12 months monitoring
• All patients who developed proteinuria were <6m from diagnosis
• Proteinuria triggered medical review prior to other criteria
• Follow up;– 2 patients early review; grade 3b HSPN, 1 resolved– 7 patients late review; monitored+/- ACEi, 4 under FU
Day 7 Urinalysis: Predicting outcome
Proteinuria: Poor predictor Confidence Interval
– Positive predictive ratio 32% (15 to 55%)
– Sensitivity 78% (45 to 94%)
Absence of proteinuria: Good predictor of normal outcome
– Negative predictive ratio 97% (90 to 99%)
– Specificity 84% (75 to 90%)
Revised HSP Monitoring Pathway
• Updated our current practice– ‘The Alder Hey HSP Monitoring Pathway’
• 6 month monitoring period• Paediatric led
– Availability of BP cuffs, paediatric phlebotomists, easy referral for paediatric advice, parental anxiety
• Stratified according to day 7 urinalysis• All urine testing undertaken by trained nurses• Revised exit criteria
The Alder Hey HSP pathway
Standard monitoring
1 month review
3 month review
6 month review Discharge
Intensive monitoringDay 14 review
1 month review
2 month review
3 month review
4 month review
6 month review
Refer for medical review
Presentation & diagnosisDay 7 review
Exit criteria
Robust peer review
Future strategies
• Universal follow up – Clinical improvements; standardise care, equity,
improved awareness– Research opportunities; describe ‘at risk’ patients,
early intervention, facilitate RCTs
• Regional standardisation
National interest• Adoption; NW centres, Scottish region, Evelina Hospital
• UK support to adopt pathway – Welsh Paediatric Society– British Association of General Paediatrics– Scottish Paediatric Network (SPARN)– Paediatric Nephrology CSG (Prof Saleem)– Paediatric Rheumatology CSG (Prof Beresford)– General Paediatric CSG (Dr Powell)
HSP diagnosis Diagnosis; EULAR criteria
Screening for nephritis
No renal involvement
Renal involvement
Resolved renal involvement HSPN
20% ESRFPersistent/resolve
Diagnosis; Renal biopsy ISKDC classification
?
?
?
National screeningReliable data
Characterise ‘at risk’ patients
Develop renal biopsy indications
Evidence based management
Phased development (3-years)Phase 1:
Universal screening, HSP registry
Pathway revalidationPhase 2:
HSPN Working Group, HSPN registry
Data biopsy indications & managementPhase 3:
Standardise HSPN management, Renal biopsy indications & consensus management
Randomised controlled trials
Conclusions• All HSP patients require 6m renal screening
– Renal involvement common– Majority will have a normal renal outcome– High risk groups - proteinuria, older, non-Caucasian– Evidence based renal monitoring
• Universal monitoring with phased development
AcknowledgementsPatients, families:•Alder Hey patients and familiesAuthors:•Professor Michael Beresford•Dr. Caroline Jones•Dr. Richard Holt•Dr. Amanda RichardsonOriginal HSP pathway committee:•Dr. Gavin Cleary•Dr. Briar Stewart•Dr. Dave Casson•Elvina White•Pauline Stone
Clinicians:•Dr. Henry Morgan•Dr. Brian Judd•Dr. Eileen Baildam•Dr. Liza McCann
Ward D2 staff