case presentation dr mohan shenoy consultant paediatric nephrologist royal manchester children’s...
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Case Presentation
Dr Mohan Shenoy
Consultant Paediatric Nephrologist
Royal Manchester Children’s Hospital
History
• 6yr girl
• Presents with non blanching palpable purpuric rash over extensor surface of arms and legs
• Ankle pain
HSP: Background
• Most common childhood vasculitis
• Incidence of HSP: 135-200 pmcp
• Highest among 4-6 year olds – 700 pmcp
Stewart M et al, Eur J Pediatr 147:113-115, 1988Gardner-Medwin J et al, Lancet 360:1197-202, 2002
HSP: Diagnostic criteria
Palpable purpura (mandatory) in the presence ofat least one of the following four features:
– Diffuse abdominal pain– Arthritis (acute) or arthralgia– Renal involvement (any haematuria and/or proteinuria)– Any biopsy showing predominant IgA deposition
Ozen S et al Ann Rheu Dis 65:936-41, 2006
Evaluation of a child with HSP
• Weight
• Blood pressure
• Urine dipstix for blood and protein
• If dipstix positve for blood or protein:– Urine microscopy– Urine protein creatinine ratio– U&E, LFT
Case history
• So…
• In our patient with HSP with no renal manifestation, what follow-up and monitoring is required?
HSP – Onset of nephritis
Time of onset of urinary abnormalities after the diagnosis of HSP
Weeks after HSP diagnosis1 2 4 6 8 24
% 37 54 84 90 91 97
Narchi H Arch Dis Child 90:916-20, 2005
Recommended follow-up
• BP & urine dipstix for – week 1-6 weekly– Week 7-24 monthly
• Discharge at 6 months if no urinary abnormality
Narchi H Arch Dis Child 90:916-20, 2005
Early steroids to prevent onset of HSP nephritis
• A large UK prospective study
• 353 children randomised to steroids or placebo
• No difference in the incidence of proteinuria at 12 months– 19/145 steroid vs 15/145 placebo
Dudley J et al Pediatr Nephrol 22:1457, 2007
Therefore…
Early steroid therapy to prevent onset of HSP nephritis cannot be recommended in children presenting with HSP
Case history
• Child presents 3 weeks later– Frank haematuria– Protein +++– BP 110/70– Not oedematous– Creat 45, albumin 34– Urine protein creatinine ratio 285mg/mmol
Indications for Renal Biopsy
• Acute nephritis
• Nephrotic syndrome
• Persisting heavy proteinuria – Urine protein creatinine ratio >200mg/mmol
for 2 weeks
Discuss with Nephrologist
• Hypertension
• Abnormal renal function
• Macroscopic haematuria > 5 days
• Persisting proteinuria
Prognosis of HSP nephritis
• Significant variability
• Chronic kidney disease 2-20%
• 2% of children with ESKD in UK
Outcome of HSP nephritis
• Unselected study
• 270 children with HSP over 13 years
• Renal involvement at presentation – 20%
• Mean follow-up 8.3 years
• CKD in only 3 (1.1%)
Stewart M et al, Eur J Pediatr 147:113-115, 1988
Clinical Presentation and Outcome
0
20
40
60
80
100
IHP NS AN AN &NS
ESKD
Active disease
Minor disease
Normal
Cameron JS et al Oxford Textbook of Clinical Nephrology
Biopsy grade and Outcome
0
20
40
60
80
100
1 2 3 4 5
ESKD
Active disease
Minor disease
Normal
ISKDC Biopsy grade
Cameron JS et al, Oxford Textbook of Clinical Nephrology
Long-term outcome of HSP nephritis
• 78 children with HSP nephritis
• Various immunosuppressive regimens
• F/U 23 years
• Active renal disease: 7.5%
• ESKD: 14%
Goldstein et al Goldstein et al Lancet 339:280–282Lancet 339:280–282, 1992, 1992
Outcome of HSP nephritis
• 16/44 pregnancies – proteinuria+/- hypertension
• 7 patients – deterioration following complete recovery at 5 year follow-up
Goldstein et al Goldstein et al Lancet 339:280–282Lancet 339:280–282, 1992, 1992
Take home messages
• No risk of CKD if urinalysis normal at 6 months
• In unselected patients, the risk of CKD < 2%
• Presentation with acute nephritis and nephrotic syndrome high risk of CKD
• Late deterioration in renal function can occur and all children with significant nephritis require life long monitoring