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Henoch Schonlein Purpura A proposed pathway for follow-up

Henoch Schonlein Purpura A proposed pathway for follow-up

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Henoch Schonlein Purpura A proposed pathway for follow-up

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  1. HenochSchonleinPurpuraA 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

  2. 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

  3. 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

  4. Henoch Schonlein Purpura • 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 2. Gardner-Medwin et al, 2002, 3. http://www.ons.gov.uk

  5. 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 • Mir et al 2007, 2. Shenoy et al, 2007, 3. Butani et al, 2007, 4. UK Renal Registry 2005

  6. 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 • Weiss P et al J Ped 2009

  7. HSP diagnosis Diagnosis; EULAR criteria Screening for nephritis No renal involvement Renal involvement Diagnosis; Renal biopsy ISKDC classification Resolved renal involvement HSPN 20% ESRF Persistent/resolve

  8. Evidence-based treatment of HSPN Systematic 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, 20014. Bergstein et al, 19985. Chartapisak W et al. 2009

  9. HSP diagnosis Diagnosis; EULAR criteria ? Screening for nephritis No renal involvement Renal involvement ? Diagnosis; Renal biopsy ISKDC classification Resolved renal involvement HSPN ? 20% ESRF Persistent/resolve

  10. 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

  11. Aims Primary • To describe renal involvement in an unselected cohort of children with HSP Secondary • To revise our nurse led HSP monitoring pathway

  12. Primary outcome Primaryoutcome; 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

  13. Investigations Presence of proteinuria Presence of exit criteria

  14. HSP coding: Identified n=176 Excluded: Other diagnosis n=11 No care pathway n=61 HSP & sufficient data n=104 46% renal involvement at diagnosis DNA n=2 Day 7: allocation n=102 Intensive FU: Proteinuria n=22 Standard FU: No proteinuria n=80 Developed proteinuria n=13 Moved area n=2 Intensive FU (n=35): Outcome n=8 renal; n=27 normal Standard FU (n=65): Outcome n=1 renal; n=64 normal Month 12: outcome n=100 Outcome Discharged n=91; renal n=9

  15. Results

  16. Older patients more likely to develop HSPN P<0.01

  17. 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

  18. 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%)

  19. 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

  20. The Alder Hey HSP pathway Presentation & diagnosis Day 7 review Intensive monitoring Standard monitoring Day 14 review 1 month review 1 month review 2 month review 3 month review 3 month review 4 month review 6 month review Discharge 6 month review Refer for medical review

  21. Exit criteria

  22. Robust peer review

  23. Future strategies • Universal follow up • Clinical improvements; standardise care, equity, improved awareness • Research opportunities; describe ‘at risk’ patients, early intervention, facilitate RCTs • Regional standardisation

  24. 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)

  25. HSP diagnosis Diagnosis; EULAR criteria National screening Reliable data ? Screening for nephritis Characterise ‘at risk’ patients No renal involvement Renal involvement Develop renal biopsy indications ? Diagnosis; Renal biopsy ISKDC classification Resolved renal involvement HSPN Evidence based management ? 20% ESRF Persistent/resolve

  26. Phased development (3-years) Phase 1: Universal screening, HSP registry Pathway revalidation Phase 2: HSPN Working Group, HSPN registry Data biopsy indications & management Phase 3: Standardise HSPN management, Renal biopsy indications & consensus management Randomised controlled trials

  27. 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

  28. Acknowledgements Clinicians: • Dr. Henry Morgan • Dr. Brian Judd • Dr. Eileen Baildam • Dr. Liza McCann • Ward D2 staff Patients, families: • Alder Hey patients and families Authors: • Professor Michael Beresford • Dr. Caroline Jones • Dr. Richard Holt • Dr. Amanda Richardson Original HSP pathway committee: • Dr. Gavin Cleary • Dr. Briar Stewart • Dr. Dave Casson • Elvina White • Pauline Stone