Child Health Update

Henoch-Schönlein purpura in children

Limited benefit of corticosteroids
Joel Bluman  Ran D. Goldman MD FRCPC

Abstract
Question  A child recently presented to my office with lower limb petechiae, arthralgia, and abdominal pain
characteristic of Henoch-Schönlein purpura (HSP). Will systemic corticosteroids help relieve these symptoms
and prevent potential HSP complications such as intussusception and nephritis?

Answer  Henoch-Schönlein purpura is a common and self-limiting disease in children. Current evidence
does not support universal treatment of HSP with corticosteroids. Recent trials and meta-analyses found that
corticosteroids do not prevent the onset of renal disease or abdominal complications. However, corticosteroids
are effective as treatment of abdominal pain, arthralgia, and purpura. Clinicians are advised to use their
discretion in choosing which patients might benefit most from oral corticosteroid treatment.

Le purpura d’Henoch-Schönlein chez l’enfant

Bienfaits limités des corticostéroïdes
Résumé
Question  Un enfant s’est récemment présenté à mon cabinet avec des pétéchies aux membres inférieurs
et souffrant d’arthralgie et de douleurs abdominales caractéristiques du purpura d’Henoch-Schönlein (PHS).
Des corticostéroïdes systémiques aideront-ils à soulager ces symptômes et à prévenir des complications
potentielles du PHS comme l’intussusception et la néphrite?

Réponse  Le purpura d’Henoch-Schönlein est une maladie spontanément résolutive commune chez l’enfant.
Les données probantes actuelles n’appuient pas un traitement universel du PHS au moyen de corticostéroïdes.
De récentes études et méta-analyses ont révélé que les corticostéroïdes ne préviennent pas l’apparition
de néphropathies ni les complications abdominales. Toutefois, les corticostéroïdes sont efficaces comme
traitement des douleurs abdominales, de l’arthralgie et du purpura. On conseille aux cliniciens d’exercer leur
discrétion quant aux choix des patients qui pourraient bénéficier le plus d’un traitement aux corticostéroïdes
par voie orale.

H enoch-Schönlein purpura (HSP) is the most com-

mon vasculitis in children, affecting 8 to 20 chil-
dren per 100 000 annually.1-3 It has a male predominance
(male-to-female ratio of 1.5:1), and typically affects chil-
dren aged 3 to 8 years.2 Henoch-Schönlein purpura is an
immunoglobulin A (IgA)–mediated systemic small-vessel
vasculitis, with IgA deposition in vessel walls leading
to symptoms involving the skin, joints, intestines, and
kidneys.4 Although the cause is unknown, HSP is often
preceded by an acute infectious illness and has a sea-
sonal pattern (nonsummer months), providing strong
evidence for an infectious trigger.2
The diagnosis of HSP is best determined by the pres-
ence of purpura or petechiae (usually palpable) with a
lower limb predominance in addition to 1 or more of the
following 4 findings:
• abdominal pain (diffuse and colicky),
• arthritis or arthralgia,
• renal involvement (proteinuria > 0.3 g in 24 hours,
morning urine albumin or creatinine levels of
> 30 µmol/L, or positive dipstick results for hematuria, and
• positive histopathologic findings (leukocytoclastic vas-
culitis with predominant IgA deposits on skin biopsy,
or proliferative glomerulonephritis with predominant
IgA deposit on kidney biopsy).2,5
Additional symptoms include fever, scrotal pain, and
edema in boys, and rarely pulmonary, cardiac, or neuro-
logic manifestations.2
Henoch-Schönlein purpura is a self-limiting condi-
tion, usually resolving within 6 to 8 weeks, but com-
plications might arise.4 Renal involvement occurs in
37% of cases; only 1% of cases result in end-stage renal

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Child Health Update
failure.1,2 Sixty-six percent of children
experience gastrointestinal symp-
toms such as abdominal pain (44%),
intestinal bleeding (22%), or intus-
susception (≤ 3%).2,3,6-8
Treatment is symptomatic and
might include mild analgesics such
as acetaminophen and nonsteroi-
dal anti-inflammatory drugs for joint
pain and fever. 2,4 However, non-
steroidal anti-inflammatory drugs
should be avoided in the presence
of gastrointestinal or renal manifes-
tations, as they have been shown
to aggravate these symptoms.2,4 All
patients should undergo blood pres-
sure measurement and urinalysis
at the time of diagnosis followed by
periodic urinalysis for 6 months, with
further urinalysis if abnormalities are
present.9
Treatment with corticosteroids
Renal complications.  Although lit-
tle is known about disease-modifying
treatment of HSP, corticosteroids
have been suggested owing to their
immunosuppressive properties and
their usefulness in treating other
childhood vasculitides.10,11 Four ran-
domized, double-blind, placebo-
controlled trials have assessed the
effectiveness of corticosteroids
in preventing HSP nephritis. 6,12-14
Prednisone (1 mg/kg daily for 2
weeks followed by a 2-week wean-
ing period, or 2 mg/kg daily for
1 week plus a 1-week weaning
period) resulted in no reduction in
the severity of hematuria, protein-
uria, or urine protein–to–urine cre-
atinine ratio.6,12,14 A 2009 Cochrane
meta-analysis that evaluated 3 of
these studies (N = 569) determined that
prednisone did not prevent the devel-
opment of kidney disease at 1 month,
3 months, 6 months, or 1 year after
the onset of HSP.15 In a 2012 study,
Jauhola and colleagues16 investigated
the long-term renal outcomes of ste-
roids through reassessing 138 of 171
patients a mean of 7.7 years after
their treatment in one of the above-
mentioned randomized controlled
trials (RCTs). 14 They reported no
1008  Canadian Family Physician • Le Médecin de famille canadien
long-term renal protective effects
of prednisone (1 mg/kg daily for
2 weeks plus a 2-week weaning
period) versus placebo, determined
by urinalysis and blood pressure.16
Pediatric therapies for established
severe HSP nephritis include cor-
ticosteroids, immunosuppressants,
angiotensin-converting enzyme
inhibitors, plasma exchange, and
tonsillectomy. 17 In a systematic
review, Zaffanello and Fanos17 eval-
uated 34 English publications that
assessed the effectiveness of these
various treatment options using
end-stage renal disease, increasing
proteinuria, and increasing serum
creatinine as end points. Although
studies have provided evidence
for the effectiveness of the above-
mentioned treatments, the lack of
prospective RCTs, the small sam-
ple sizes of studies (ranging from
N = 3 to N = 56), and the heterogene-
ity in severity of renal disease cloud
the findings.17 Therefore, the ideal
treatment regimen, including the
role of corticosteroids for children
with severe HSP nephritis, remains
inconclusive.17
Purpura and arthralgia.  A small
body of literature speaks to the role
of corticosteroids in treating pur-
pura and arthralgia symptoms.12,14,18
Although the findings from one small
RCT12 (N = 40) were not statistically
significant, 2 larger RCTs14,18 (N = 171
and N = 176) reported improvement
of purpura and arthralgia in patients
receiving prednisone (1 mg/kg
daily for 2 weeks) versus placebo.
Specifically, prednisone decreased
both the prevalence of purpura dur-
ing the first month after onset and
the patient-reported severity and
duration of joint pain.14,18
Gastrointestinal symptoms. The
RCTs performed by Ronkainen et al14
and Jauhola et al18 demonstrate that
prednisone reduces both the severity
and the duration of abdominal pain
during the first 2 weeks of treatment,
with Ronkainen and colleagues
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Child Health Update
reporting a decrease in mean pain duration from 2.7 to
1.5 days (1.2-day decrease, 95% CI 0.1 to 2.3).14 Another
systematic review11 analyzed results from 3 retrospec-
tive studies and reported that corticosteroids in slightly
higher doses (1 to 2 mg/kg daily) were effective in
reducing abdominal pain within 24 hours after cortico-
steroid administration.
No RCTs to date have found a statistically significant
relationship between early prednisone treatment and a
decrease in abdominal complications, namely hospital-
ization, intussusception, or surgery.11,15,18 However, as
these complications are quite rare, statistical significance
might be difficult to achieve even in large RCTs. A 2010
large retrospective study (N = 1895) of hospitalized HSP
patients from the United States found that early corti-
costeroid use reduced the risk of patients undergoing
abdominal investigations such as endoscopy and abdom-
inal imaging, but did not reduce the risk of abdominal
surgery.19 Because abdominal complications are rare in
HSP and extant research does not show a significant or
practical benefit, corticosteroids should not be adminis-
tered generally as abdominal complication prophylaxis.18
Recurrent HSP.  Henoch-Schönlein purpura might
reccur in up to 33% of affected children.11 Recurrences
appear to be more common in older children (> 8 years
of age at onset) and in children with renal involve-
ment.18 Current research indicates placebo is as good as
prednisone in preventing HSP recurrence after 1 month,
with 1 RCT18 finding recurrence rates of 17% and 25%
in the placebo and prednisone (1 mg/kg daily) groups,
respectively (P = .22). A second RCT,14 as well as a sys-
tematic review of both prospective and retrospective tri-
als,11 supported these findings, reporting no statistically
significant difference in recurrence rates after 1 month
between the placebo and prednisone groups, with con-
siderable heterogeneity in the retrospective observa-
tional trials.
Conclusion
Current evidence does not support universal treatment
of HSP with corticosteroids, as they do not appear to
prevent the onset of renal disease or abdominal com-
plications, nor do they alter recurrence rates. However,
corticosteroids do seem to have a role in the symp-
tomatic management of HSP, specifically in treating
abdominal pain, arthralgia, and purpura. As research
is limited, clinicians are advised to use their discre-
tion when choosing which patients would benefit most
from corticosteroids. If prednisone is used, doses of 1
to 2 mg/kg daily given for 1 to 2 weeks (followed by an
appropriate weaning period) appear to be effective, but
further research is required to determine the optimal
dose. Patients with severe renal symptoms, specifically
nephrotic syndrome, nephritic syndrome, or rapidly
1010  Canadian Family Physician • Le Médecin de famille canadien
worsening proteinuria or hematuria should be urgently
referred to a pediatric nephrologist for prompt assess-
ment and treatment. 
Competing interests
None declared
Correspondence
Dr Ran D. Goldman, BC Children’s Hospital, Department of Pediatrics, Room
K4-226, Ambulatory Care Bldg, 4480 Oak St, Vancouver, BC V6H 3V4; tele-
phone 604 875-2345, extension 7333; fax 604 875-2414;
e-mail rgoldman@cw.bc.ca
References
1. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of
Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of
different ethnic origins. Lancet 2002;360(9341):1197-202.
2. Rostoker G. Schönlein-Henoch purpura in children and adults: diagnosis, patho-
physiology and management. BioDrugs 2001;15(2):99-138.
3. Ting TV. Diagnosis and management of cutaneous vasculitis in children. Pediatr Clin
North Am 2014;61(2):321-46. Epub 2014 Jan 21.
4. Duvuru G, Stone JH. Henoch-Schönlein purpura. In: Imboden JB, Hellmann DB,
Stone JH, editors. Current diagnosis and treatment. Rheumatology. 3rd ed. New York,
NY: McGraw-Hill; 2013.
5. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/
PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, child-
hood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part
II: final classification criteria. Ann Rheum Dis 2010;69(5):798-806.
6. Dudley J, Smith G, Llewelyn-Edwards A, Bayliss K, Pike K, Tizard J. Randomised,
double-blind, placebo-controlled trial to determine whether steroids reduce the inci-
dence and severity of nephropathy in Henoch-Schonlein purpura (HSP). Arch Dis
Child 2013;98(10):756-63. Epub 2013 Jul 11.
7. Peru H, Soylemezoglu O, Bakkaloglu SA, Elmas S, Bozkaya D, Elmaci AM, et al.
Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year
period. Clin Rheumatol 2008;27(9):1087-92. Epub 2008 Feb 28.
8. Chang WL, Yang YH, Lin YT, Chiang BL. Gastrointestinal manifestations in Henoch-
Schönlein purpura: a review of 261 patients. Acta Paediatr 2004;93(11):1427-31.
9. Narchi H. Risk of long term renal impairment and duration of follow up recom-
mended for Henoch-Schonlein purpura with normal or minimal urinary findings: a
systematic review. Arch Dis Child 2005;90(9):916-20. Epub 2005 May 4.
10. Mollica F, Li Volti S, Garozzo R, Russo G. Effectiveness of early prednisone treat-
ment in preventing the development of nephropathy in anaphylactoid purpura. Eur J
Pediatr 1992;151(2):140-4.
11. Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Effects of corticosteroid
on Henoch-Schönlein purpura: a systematic review. Pediatrics 2007;120(5):1079-87.
12. Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo-
controlled trial of prednisone in early Henoch Schönlein Purpura [ISRCTN85109383].
BMC Med 2004;2:7.
13. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, et al.
Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study
of 223 children. Arch Dis Child 2010;95(11):877-82. Epub 2010 Sep 18.
14. Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J,
et al. Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-
blind, placebo-controlled trial. J Pediatr 2006;149(2):241-7.
15. Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC. Interventions for pre-
venting and treating kidney disease in Henoch-Schönlein purpura (HSP). Cochrane
Database Syst Rev 2009;(3):CD005128.
16. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, et al.
Outcome of Henoch-Schönlein purpura 8 years after treatment with a placebo or
prednisone at disease onset. Pediatr Nephrol 2012;27(6):933-9. Epub 2012 Feb 5.
17. Zaffanello M, Fanos V. Treatment-based literature of Henoch-Schönlein purpura
nephritis in childhood. Pediatr Nephrol 2009;24(10):1901-11. Epub 2008 Dec 9.
18. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, et al.
Clinical course of extrarenal symptoms in Henoch-Schonlein purpura: a 6-month
prospective study. Arch Dis Child 2010;95(11):871-6. Epub 2010 Sep 16.
19. Weiss PF, Klink AJ, Localio R, Hall M, Hexem K, Burnham JM, et al. Corticosteroids
may improve clinical outcomes during hospitalization for Henoch-Schönlein pur-
pura. Pediatrics 2010;126(4):674-81. Epub 2010 Sep 20.
Child Health Update is produced by the
Pediatric Research in Emergency Therapeutics
(PRETx) program (www.pretx.org) at the BC
Pediatric Research in Emergency Therapeutics
Children’s Hospital in Vancouver, BC. Mr
Bluman is a member and Dr Goldman is Director of the PRETx program. The
mission of the PRETx program is to promote child health through evidence-based
research in therapeutics in pediatric emergency medicine.
Do you have questions about the effects of drugs, chemicals, radiation, or
infections in children? We invite you to submit them to the PRETx program by fax
at 604 875-2414; they will be addressed in future Child Health Updates.
Published Child Health Updates are available on the Canadian Family Physician
website (www.cfp.ca).
| Vol 60:  november • novembre 2014