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

Vol 60:  november • novembre 2014 | Canadian Family Physician • Le Médecin de famille canadien  1007
Child Health Update

failure.1,2 Sixty-six percent of children long-term renal protective effects


experience gastrointestinal symp- of prednisone (1 mg/kg daily for
toms such as abdominal pain (44%), 2 weeks plus a 2-week weaning
intestinal bleeding (22%), or intus- period) versus placebo, determined
susception (≤ 3%).2,3,6-8 by urinalysis and blood pressure.16
Treatment is symptomatic and Pediatric therapies for established
might include mild analgesics such severe HSP nephritis include cor-
as acetaminophen and nonsteroi- ticosteroids, immunosuppressants,
dal anti-inflammatory drugs for joint angiotensin-converting enzyme
pain and fever. 2,4 However, non- inhibitors, plasma exchange, and
steroidal anti-inflammatory drugs tonsillectomy. 17 In a systematic
should be avoided in the presence review, Zaffanello and Fanos17 eval-
of gastrointestinal or renal manifes- uated 34 English publications that
tations, as they have been shown assessed the effectiveness of these
to aggravate these symptoms.2,4 All various treatment options using
patients should undergo blood pres- end-stage renal disease, increasing
sure measurement and urinalysis proteinuria, and increasing serum
at the time of diagnosis followed by creatinine as end points. Although
periodic urinalysis for 6 months, with studies have provided evidence
further urinalysis if abnormalities are for the effectiveness of the above-
present.9 mentioned treatments, the lack of
prospective RCTs, the small sam-
Treatment with corticosteroids ple sizes of studies (ranging from
Renal complications.  Although lit- N = 3 to N = 56), and the heterogene-
tle is known about disease-modifying ity in severity of renal disease cloud
treatment of HSP, corticosteroids the findings.17 Therefore, the ideal
have been suggested owing to their treatment regimen, including the
immunosuppressive properties and role of corticosteroids for children
their usefulness in treating other with severe HSP nephritis, remains
childhood vasculitides.10,11 Four ran- inconclusive.17
domized, double-blind, placebo-
controlled trials have assessed the Purpura and arthralgia.  A small
effectiveness of corticosteroids body of literature speaks to the role
in preventing HSP nephritis. 6,12-14 of corticosteroids in treating pur-
Prednisone (1 mg/kg daily for 2 pura and arthralgia symptoms.12,14,18
weeks followed by a 2-week wean- Although the findings from one small
ing period, or 2 mg/kg daily for RCT12 (N = 40) were not statistically
1 week plus a 1-week weaning significant, 2 larger RCTs14,18 (N = 171
period) resulted in no reduction in and N = 176) reported improvement
the severity of hematuria, protein- of purpura and arthralgia in patients
uria, or urine protein–to–urine cre- receiving prednisone (1 mg/kg
atinine ratio.6,12,14 A 2009 Cochrane daily for 2 weeks) versus placebo.
meta-analysis that evaluated 3 of Specifically, prednisone decreased
these studies (N = 569) determined that both the prevalence of purpura dur-
prednisone did not prevent the devel- ing the first month after onset and
opment of kidney disease at 1 month, the patient-reported severity and
3 months, 6 months, or 1 year after duration of joint pain.14,18
the onset of HSP.15 In a 2012 study,
Jauhola and colleagues16 investigated Gastrointestinal symptoms. The
the long-term renal outcomes of ste- RCTs performed by Ronkainen et al14
roids through reassessing 138 of 171 and Jauhola et al18 demonstrate that
patients a mean of 7.7 years after prednisone reduces both the severity
their treatment in one of the above- and the duration of abdominal pain
mentioned randomized controlled during the first 2 weeks of treatment,
trials (RCTs). 14 They reported no with Ronkainen and colleagues

1008  Canadian Family Physician • Le Médecin de famille canadien | Vol 60:  november • novembre 2014
Child Health Update

reporting a decrease in mean pain duration from 2.7 to worsening proteinuria or hematuria should be urgently
1.5 days (1.2-day decrease, 95% CI 0.1 to 2.3).14 Another referred to a pediatric nephrologist for prompt assess-
systematic review11 analyzed results from 3 retrospec- ment and treatment. 
tive studies and reported that corticosteroids in slightly Competing interests
None declared
higher doses (1 to 2 mg/kg daily) were effective in
Correspondence
reducing abdominal pain within 24 hours after cortico- Dr Ran D. Goldman, BC Children’s Hospital, Department of Pediatrics, Room
steroid administration. K4-226, Ambulatory Care Bldg, 4480 Oak St, Vancouver, BC V6H 3V4; tele-
phone 604 875-2345, extension 7333; fax 604 875-2414;
No RCTs to date have found a statistically significant e-mail rgoldman@cw.bc.ca
relationship between early prednisone treatment and a References
decrease in abdominal complications, namely hospital- 1. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of
Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of
ization, intussusception, or surgery.11,15,18 However, as different ethnic origins. Lancet 2002;360(9341):1197-202.
these complications are quite rare, statistical significance 2. Rostoker G. Schönlein-Henoch purpura in children and adults: diagnosis, patho-
physiology and management. BioDrugs 2001;15(2):99-138.
might be difficult to achieve even in large RCTs. A 2010 3. Ting TV. Diagnosis and management of cutaneous vasculitis in children. Pediatr Clin
North Am 2014;61(2):321-46. Epub 2014 Jan 21.
large retrospective study (N = 1895) of hospitalized HSP 4. Duvuru G, Stone JH. Henoch-Schönlein purpura. In: Imboden JB, Hellmann DB,
patients from the United States found that early corti- Stone JH, editors. Current diagnosis and treatment. Rheumatology. 3rd ed. New York,
NY: McGraw-Hill; 2013.
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abdominal investigations such as endoscopy and abdom- PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, child-
hood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part
inal imaging, but did not reduce the risk of abdominal 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,
surgery.19 Because abdominal complications are rare in double-blind, placebo-controlled trial to determine whether steroids reduce the inci-
HSP and extant research does not show a significant or dence and severity of nephropathy in Henoch-Schonlein purpura (HSP). Arch Dis
Child 2013;98(10):756-63. Epub 2013 Jul 11.
practical benefit, corticosteroids should not be adminis- 7. Peru H, Soylemezoglu O, Bakkaloglu SA, Elmas S, Bozkaya D, Elmaci AM, et al.
tered generally as abdominal complication prophylaxis.18 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.
Recurrent HSP.  Henoch-Schönlein purpura might 9. Narchi H. Risk of long term renal impairment and duration of follow up recom-
reccur in up to 33% of affected children.11 Recurrences 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.
appear to be more common in older children (> 8 years 10. Mollica F, Li Volti S, Garozzo R, Russo G. Effectiveness of early prednisone treat-
of age at onset) and in children with renal involve- ment in preventing the development of nephropathy in anaphylactoid purpura. Eur J
Pediatr 1992;151(2):140-4.
ment.18 Current research indicates placebo is as good as 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.
prednisone in preventing HSP recurrence after 1 month, 12. Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo-
with 1 RCT18 finding recurrence rates of 17% and 25% controlled trial of prednisone in early Henoch Schönlein Purpura [ISRCTN85109383].
BMC Med 2004;2:7.
in the placebo and prednisone (1 mg/kg daily) groups, 13. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, et al.
respectively (P = .22). A second RCT,14 as well as a sys- 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.
tematic review of both prospective and retrospective tri- 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-
als,11 supported these findings, reporting no statistically blind, placebo-controlled trial. J Pediatr 2006;149(2):241-7.
significant difference in recurrence rates after 1 month 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
between the placebo and prednisone groups, with con- Database Syst Rev 2009;(3):CD005128.
siderable heterogeneity in the retrospective observa- 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
tional trials. 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.
Conclusion 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
Current evidence does not support universal treatment prospective study. Arch Dis Child 2010;95(11):871-6. Epub 2010 Sep 16.
of HSP with corticosteroids, as they do not appear to 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-
prevent the onset of renal disease or abdominal com- pura. Pediatrics 2010;126(4):674-81. Epub 2010 Sep 20.
plications, nor do they alter recurrence rates. However,
corticosteroids do seem to have a role in the symp-
Child Health Update is produced by the
tomatic management of HSP, specifically in treating Pediatric Research in Emergency Therapeutics
abdominal pain, arthralgia, and purpura. As research (PRETx) program (www.pretx.org) at the BC
Pediatric Research in Emergency Therapeutics

is limited, clinicians are advised to use their discre- Children’s Hospital in Vancouver, BC. Mr
tion when choosing which patients would benefit most Bluman is a member and Dr Goldman is Director of the PRETx program. The
from corticosteroids. If prednisone is used, doses of 1 mission of the PRETx program is to promote child health through evidence-based
to 2 mg/kg daily given for 1 to 2 weeks (followed by an research in therapeutics in pediatric emergency medicine.
Do you have questions about the effects of drugs, chemicals, radiation, or
appropriate weaning period) appear to be effective, but
infections in children? We invite you to submit them to the PRETx program by fax
further research is required to determine the optimal at 604 875-2414; they will be addressed in future Child Health Updates.
dose. Patients with severe renal symptoms, specifically Published Child Health Updates are available on the Canadian Family Physician
nephrotic syndrome, nephritic syndrome, or rapidly website (www.cfp.ca).

1010  Canadian Family Physician • Le Médecin de famille canadien | Vol 60:  november • novembre 2014