Gurusamy, Pari Shanmuga Raman

Janagaraj, Priya Darshene

English

Journal of the Asian Medical Students Association

JAMSA Vol 1(1) 
 
The Role of Prophylactic Corticosteroids in Henoch Schonlein 
Purpura 
Authors: Pari Shanmuga Raman Gurusamy (psgur1@student.monash.edu), Priya Darshene 
Janagaraj (pdjan1@student.monash.edu) 
 
Introduction 
Henoch Schonlein Purpura (HSP) is the 
commonest vasculitis seen in the pediatric 
population [1], with an incidence of 10-20 per 
100, 000 children. Although treatment with 
corticosteroids is routine during the acute 
phase of the disease, controversy remains as to 
the merit of corticosteroids in preventing 
persistent renal disease in children presenting 
with HSP. Although several sporadic case 
series have claim demonstration of efficacious 
usage of prednisone and immuomodulators in 
preventing development of HSP nephropathies, 
data from randomized controlled trials are 
limited. 
 
Case Presentation 
John
1
, a 7 years old Indian male, presented 
with a 3-day history of bilateral knee pain and 
swelling, associated with a 5-day history of 
non-itchy, non-painful rash over the lower 
limbs and a week history of fever and coryza, 
in the absence of trauma. Rest of the systems 
review was negative. Apart from the recent 
upper respiratory tract infection, which 
spontaneously resolved, John has been well 
with no past history of similar episodes. He 
was not on any medications and has no known 
allergies. Perinatal history was unremarkable: 
spontaneous vertex delivery at term with birth 
weight of 3.4 kg. His development has been up 
to age, with satisfactory performance in school. 
His immunization was up to date. Family 
history was unremarkable. He was a single 
child, looked after by his parents in a 
comfortable home environment. 
Vital Signs: Pulse: 75 beats per minute, with 
regular rhythm and good volume, Blood 
                                                          
1
 Patient is de-identified to protect confidentiality 
Pressure: 110/70, Respiratory Rate: 16 and 
Temperature: 37.5
o
C 
Upon examination, John appeared alert, active, 
oriented, responsive and non-toxic. Knees 
were bilaterally tender with periarticular 
edema, but neither hot nor erythematous. 
Crops of palpable, non-blanching, purpuric 
rash were distributed over the extensor 
surfaces of the lower extremities and buttocks. 
Rest of examination findings were within 
normal limits. Relevant negative findings 
included the absence of lip-cracking, cervical 
lymphadenopathy, peeling of skin over the 
extremities and organomegaly. 
Provisional diagnosis was HSP and 
differential diagnoses included 
meningococcemia, ITP, Reactive/Post 
infective Arthritis, JIA
2
, Septic Arthritis and 
connective tissue diseases. Laboratory work 
up including full blood count (Hb, WBC 
differential & platelet count), blood culture, 
coagulation studies, renal profile (BUSE
3
, 
Creatinine), CRP, and autoantibody screen 
(ANA, ANCA to rule out collagen vascular 
diseases) yielded normal results. Urine 
dipstick revealed 2+ blood and 1+ protein in 
the absence of nitrites and WBC, and 
consequent Urine FEME revealed microscopic 
hematuria and mild proteinuria – suggesting 
glomerulonephritis. In this context, given a 
normal renal profile (which ruled out alarming 
causes of proteinuria), raised ASOT
4
 and 
reduced C3, John was diagnosed to have HSP 
and appropriately treated with Steroids. 
Follow up for a period of six months from the 
initial presentation, with regular urinanalysis 
and a minimum of 2 blood pressure checkups, 
                                                          
2
 Juvenile Idiopathic Arthritis 
3
 Blood Urea & Serum Electrolytes 
4
 Anti-Streptolysin O Titre 
JAMSA Vol 1(1) 
 
has been planned. Prompt referral to a 
paediatric nephrologist for long term follow up 
in a situation of persistent renal involvement 
has been discussed with the family. 
 
Discussion 
Though the general consensus remains that 
HSP resolves spontaneously, with the use of 
appropriate analgesics (simple analgesics for 
joint pain, and corticosteroids for sever 
abdominial colic), is the prophylactic use of 
corticosteroids or other immunomodulators 
warranted to prevent persistent renal disease? 
Systematic review of 10 Randomised 
Controlled Trials by W Chartapisak et al [1], 
which involved 1230 children aged less than 
18 years, in a setting of Secondary and tertiary 
paediatric and paediatric nephrology services, 
with the main outcome measures persistent 
proteinuria and/or haematuria, concluded that 
short-term prednisone has not been shown to 
be effective in treating or preventing renal 
involvement in HSP: Meta-analyses of four 
well designed placebo-controlled RCTs 
revealed insignificant difference in the risk of 
persistent kidney disease at 6 months (n=379; 
RR: 0.51 with 95% CI: 0.24-1.11, CI spans 
across 1) and 12 months (n=498; RR: 1.02 
with 95% CI: 0.40- 2.62, CI spans across 1) in 
children given prednisone for 14–28 days at 
presentation of HSP in comparison to those 
who received  supportive treatment or placebo.  
All 4 studies demonstrated adequate allocation 
concealment with computer-generated random 
allocation sequence, blinding of participants, 
investigators and outcome assessors and 
attrition bias was nominal with loss to 
following-up patients ranging from 0% - 10%. 
However, none of the studies adopted an 
intention-to treat analysis. 
A Cochrane review by Opastirakul S et al [2] 
of RCTs and quasi-RCTs from the Cochrane 
Renal Group's specialised register, the 
Cochrane Central Register of Controlled Trials 
(CENTRAL), MEDLINE and EMBASE 
concluded that cyclophosphamide does not 
offer any additional protection against renal 
diseases in children with HSP who were given 
supportive treatment (n=56; RR: 1.07 with 95% 
CI: 0.65-1.78, CI spans across 1) or 
methylprednisolone (n=19; RR: 0.39 with 95% 
CI: 0.14-1.06, CI spans across 1). However, 
the data offered by both the studies are 
questionable because of small sample sizes. 
A randomized, double-blind, placebo 
controlled study by Huber AM [3] involving 
40 children with HSP, seen in the setting of 
emergency room of a tertiary-care, paediatric 
centre, compared co-primary outcomes of the 
rates of renal involvement at 1 year using 
Fisher's Exact test. Huber AM states that there 
were no significant difference [P>0.05(=1.0)] 
in the rate of renal involvement between the 
prednisone group (3/21) and placebo group 
(2/19). The study, however, showed some 
benefit in the prednisone group in reducing 
risk of intussusception, though not statistically 
significant [(P>0.05(=0.2)].  
A randomized, double-blind, placebo-
controlled trial by Ronkainen J [4,5] to 
evaluate the efficacy of early prednisone 
therapy in both preventing and treating renal 
symptoms included 171 patients 
(prednisone=84; placebo=87) and was 
conducted on an intent-to-treat basis. After 6 
months follow-up with endpoints as renal 
involvement at either 1, 3, or 6 months, 
revealed that, though prednisone was not 
effective in preventing renal involvement, it 
was effective in treating them 
[(difference=27%; 95% CI: 3% to 47%; 
P<0.05(=.024)] – thereby playing a crucial 
role of altering the course of the renal 
involvement. 
Conclusion 
In conclusion, current literatures [1-3] do not 
demonstrate any statistically significant 
benefits that are rendered by prednisone or 
other immunomodulators in preventing the 
development of persistent renal disease in 
children diagnosed with HSP, when 
administered at the time of presentation. 
Therefore, routine prophylactic usage 
prednisone in uncomplicated HSP should not 
JAMSA Vol 1(1) 
 
be recommended at this moment as any 
apparent benefits offered by such drugs could 
well be due to chance or other unknown 
confounders. However, the role of prednisone 
in altering the disease course in HSP 
complicated with nephropathy is well-known 
and is warranted by evidences conferred by 
literatures discussed above. Hence, continuous 
monitoring and early identification of children 
who develop HSP nephropathy and prompt 
treatment with prednisone is the ideal 
management to reduce disease burden of HSP. 
 
 
References 
1. Chartapisak W, Willis NS, Hodson EM 
(2009) Prevention and treatment of 
renal disease in Henoch-Schönlein 
purpura: a systematic review. Arch 
Dis Child 94:132–137 
2. Opastirakul, S., Craig, J. C., et al. 
(2010), Cochrane review: Interventions 
for preventing and treating kidney 
disease in Henoch-Schönlein Purpura 
(HSP). Evidence-Based Child Health: 
A Cochrane Review Journal, 5: 637–
700. doi: 10.1002/ebch.545 
3. 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. doi: 10.1186/1741-7015-2-7.  
4. Ronkainen J, Koskimies O, Ala-
Houhala M, Antikainen M, Merenmies 
J, Rajantie J, et al. Early prednisone 
therapy in Henoch-Schonlein purpura: 
a randomized, double-blind, placebo-
controlled trial. J Pediatr. 
2006;149:241-7.    
5. Koskimies O, Mir S, Rapola J, Vilska J. 
Henoch-Schönlein nephritis: long-term 
prognosis of unselected patients. Arch 
Dis Child. 1981;56:482-4. 
 


The Role of Prophylactic Corticosteroids in Henoch Schonlein Purpura

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The Role of Prophylactic Corticosteroids in Henoch Schonlein Purpura

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