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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 Childrens 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 criteria
1
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
population
2
(SSNS 3 cases per 100,000; IDDM 207 cases per 100,000)
Average North West DGH;
Catchment population of 60,000 children
3

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 GPs

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 nephrotic
1
Up to 20% nephrotic range proteinuria
Cohorts with established HSPN 15-20% ESRF
2,3
Accounts for 1.7% all UK ESRF
4



1. Mir et al 2007, 2. Shenoy et al, 2007, 3. Butani et al, 2007, 4. UK Renal Registry 2005
Screening for HSPN
Screening varies
1
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/r
esolve
20% ESRF
HSPN
Diagnosis;
Renal biopsy ISKDC classification
Evidence-based treatment of HSPN
Systematic review of RCTs: no difference
Early corticosteroids Vs placebo, total n=379
1
Cyclophosphamide Vs supportive, n=56
Cyclosporin Vs methylprednisolone RCT, n=24
2

Other studies
Cyclophosphamide + methylprednisolone, n=12
3

Azathioprine + steroids, n=21
4

Cochrane: Few RCTs
5
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% ESRF
Persistent/r
esolve
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 outcome
Primary 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/1.73m
2
Macroscopic haematuria >28 days
12 months completed monitoring with urine abnormalities

Investigations
Presence 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=11
No care pathway n=61
HSP & sufficient data n=104
46% renal involvement at diagnosis
DNA n=2
Day 7: allocation n=102
Developed proteinuria n=13
Moved 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 monitoring
Day 14 review
1 month review
2 month review
3 month review
4 month review
6 month review
Refer for medical review
Presentation & diagnosis
Day 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% ESRF
Persistent/r
esolve
Diagnosis;
Renal biopsy ISKDC classification
?
?
?
National screening
Reliable data
Characterise at risk patients
Develop renal biopsy indications
Evidence based management
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
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

Acknowledgements
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
Clinicians:
Dr. Henry Morgan
Dr. Brian Judd
Dr. Eileen Baildam
Dr. Liza McCann

Ward D2 staff

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