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

Examination

• Well child

• BP 106/60

• Urine – NAD

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

Investigations

Only if diagnosis uncertain

• FBC• Coagulation• ASO titre• C3 and C4• Igs• ANA, ANCA

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

Can early steroid therapy prevent onset of HSP

nephritis?

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

Cochrane review 2009

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

HSPN - Presentation

22%

43%

15%

20%Hematuria

Haematuria+proteinuria

Nephritic

Nephrotic+nephritic

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

Case history

• Weekly review

• Upcr improves 154 and then 75mg/mmol

• BP and creatinine normal

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