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doi:10.1111/jpc.12403

REVIEW ARTICLE

HenochSchnlein purpura in children

Peter Trnka
Queensland Child and Adolescent Renal Service, Royal Childrens Hospital, Herston, Queensland, Australia

Abstract: HenochSchnlein purpura is the most common systemic vasculitis of childhood. In the majority of children, the outcome of
HenochSchnlein purpura is excellent with spontaneous resolution of symptoms and signs. However, a small subset of patients will develop
long-term sequelae in the form of chronic kidney disease. While the clinical presentation and diagnosis of HenochSchnlein purpura is
straightforward, treatment of HenochSchnlein purpura nephritis and long-term renal outcomes of more severely affected children are less
certain. This review article gives a general overview of HenochSchnlein purpura with emphasis on recently published information, including
the new classication of childhood vasculitis, insights into pathogenesis of HenochSchnlein purpura and a summary of various treatments of
established HenochSchnlein purpura nephritis.

Key words: chronic kidney disease; immunoglobulin A; nephritis; purpura; vasculitis.

History described two boys with clinical findings suggestive of HSP

The first clinical description of HenochSchnlein purpura
(HSP) comes from English physician William Heberden, who
Key Points
1 HenochSchnlein purpura is the most common systemic vas-
culitis of childhood presenting with a tetrad of purpura, arthri-
tis or arthralgia, abdominal pain and renal disease. While the
presence of purpura is a compulsory criterion for the diagno-
sis of HenochSchnlein purpura, other signs and symptoms
are more variably present.
2 Abnormal glycosylation of immunoglobulin A1 molecules
predisposes patients with HenochSchnlein purpura to
formation of large immune complexes. Clearance of these
large molecules is impaired, they deposit in small vessel walls
of the affected organs and trigger immune response leading
to inammatory reaction presenting as clinical signs and
symptoms.
3 The long-term morbidity of HenochSchnlein purpura is
related to nephritis. Based on the current evidence, early
immunosuppressive treatment of children with Henoch
Schnlein purpura should be reserved for those presenting
with severe kidney involvement (rapidly progressive glomeru-
lonephritis, nephrotic syndrome). There might be a role for
immunosuppression in patients with ongoing nephritis
(persistent/increasing proteinuria), but this approach will
have to be tested in large prospective studies before it can
be widely accepted in clinical practice.
Correspondence: Dr. Peter Trnka, Queensland Child and Adolescent
Renal Service, Royal Childrens Hospital, Woolworths Building, 5th Floor,
Herston Road, Herston, QLD, 4029, Australia. Fax: (07) 3636 5505; email:
Peter_Trnka@health.qld.gov.au
Conict of interest: None.
Accepted for publication 21 July 2013.
including purpuric rash, arthralgia and abdominal pain.1 HSP
carries the names of two 19th century German physicians,
Johann Schnlein and his student Eduard Henoch. Schnlein
described the association of non-thrombocytopaenic purpura
and joint pain in 1837, which he called purpura rheumatica.2
Henoch added gastrointestinal and renal involvement in 1874.3
Purpura is a latin word for purple, which has its origin in Greek
word porphyra, a name of the Tyrian purple dye secreted by sea
snails Murex trunculus and Murex brandaris, used for centuries as
an imperial dye. Anaphylactoid purpura, another commonly
used name for HSP, is incorrect and should not be used because
anaphylaxis does not play a significant role in the pathogenesis
of this vasculitis.
Denition and Classication
HSP is a systemic vasculitis characterised by the deposition of
immunoglobulin A (IgA)-containing immune complexes in the
walls of small vessels (arterioles, capillaries and venules). According
to the recently endorsed European League Against Rheumatism,
Paediatric Rheumatology European Society and Paediatric
Rheumatology International Trials Organisation classification
of childhood vasculitis, HSP belongs to the group of non-
granulomatous, predominantly small vessel vasculitides (Table 1).4
Epidemiology
HSP is the most common childhood vasculitis with a reported
annual incidence that varies between 10 and 30 cases per
100 000 children < 17 years based on hospital and overall popu-
lation estimates.5,6 These reports are likely to underestimate the
true prevalence of HSP given the voluntary nature of reporting
to these surveys. The mean age of presentation is 6 years with
most cases in children < 10 years of age,7 and recent studies

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HSP in children P Trnka

Table 1 New EULAR/PRINTO/PRES endorsed classication of childhood

vasculitis (with permission from reference 4)

I Predominantly large vessel vasculitis

Takayasu arteritis
II Predominantly medium sized vessel vasculitis
Childhood polyarteritis nodosa
Cutaneous polyarteritis
Kawasaki disease
III Predominantly small vessel vasculitis
(A) GRANULOMATOUS
Wegeners granulomatosis
Churg-Strauss syndrome
(B) NON-GRANULOMATOUS
Microscopic polyangiitis
HenochSchnlein purpura
Isolated cutaneous leucocytoclastic vaculitis
Hypocomplementic urticarial vasculitis
IV Other vasculitides
Behet disease
Vasculitis secondary to infection (including hepatitis B associated
polyrateritis nodosa), malignancies, and drugs, including
hypersensitivity vasculitis
Vasculitis associated with connective tissue diseases
Isolated vasculitis of the central nervous system Fig. 1 Glycosylation of IgA1 molecule. (a) The hinge region of the IgA1
Cogan syndrome molecule is O-glycosylated by the attachment of N-acetylgalactosamine
Unclassied (GalNAc) to serine residues. (b) The glycan chains may be elongated with
further addition of galactose (Gal) to GalNAc, and a variable degree of
sialylation with N-acetylneuraminic acid (NeuNAc). (with permission from
reference 14).

suggesting an equal incidence in males and females.8 HSP occurs

predominantly in cold months of the year and has been
reported worldwide.
Aetiology and Pathogenesis
The majority of HSP cases are preceded by an upper respiratory
tract infection suggesting a potential infectious trigger. Strepto-
coccus, staphylococcus and parainfluenza are most commonly
implicated but there are multiple case reports describing the
association between virtually all respiratory pathogens and
HSP.9 Anecdotal reports also describe HSP cases after vaccina-
tion, with multiple vaccines implicated, including the recently
developed pandemic influenza A (H1N1) vaccine.10 An associa-
tion between drugs and HSP has also been reported, although
the role of these medications in pathogenesis of HSP is uncertain
given that most were being used at the time of onset of the
disease for treatment of concurrent infection.
The characteristic pathological feature of HSP vasculitis is a
deposition of IgA-containing immune complexes in vessel walls
of affected organs and in the kidney mesangium. Histologically,
the appearance of HSP nephritis is identical to IgA nephropathy
and recent studies in patients with HSP and IgA nephropathy
have detailed a potential role of IgA1 in the pathogenesis of both
of these conditions.11,12
IgA is found in serum and mucosal secretions and is a major
class of immunoglobulin that plays an important role in mucosal
immunity. In man, more IgA is produced than all other immu-
noglobulin classes combined because of the high mucosal
synthesis and short half-life of IgA of 56 days. Of two IgA
subclasses, IgA1 is phylogenetically younger and differs from
IgA2 by insertion of a 1317 amino acid sequence in the hinge
region of the IgA1 molecule.13 The hinge region of IgA1 is heavily
glycosylated in normal individuals. N-acetylgalactosamine
(GalNAc) is O-linked to serine residues (O-glycosylation) and
elongation of the glycan chains is achieved by further addition
of galactose (Gal) and N-acetylneuraminic acid (NeuNAc)
(galactosylation and sialylation) to GalNAc (Fig. 1).14 Glycosy-
lation of IgA1 seems to play important role in facilitating clear-
ance of IgA1 molecules. Normally glycosylated IgA1 molecules
interact with the asialoglycoprotein receptor (ASGP-R)
expressed on the hepatocytes, followed by internalisation and
degradation of these molecules. Patients with HSP and IgA
nephropathy express inherited Gal deficient glycosylation of
IgA1 molecules.11 While the absence of Gal exposes GalNAc as a
terminal glycan, the stimulus for the formation of antibodies
against GalNAc is unknown. However, many microorganisms
express GalNAc-containing sugars on their surface. During
infection by these microorganisms, antibodies to GalNAc on
bacteria or viruses could potentially cross-react with GalNAc on
IgA1 molecule with subsequent formation of large IgA1-IgG
immune complexes that cannot reach ASGP-R in the space of
Disse in the liver but are able to cross endothelial fenestrae in
the glomerulus and deposit in the mesangium (Fig. 2).15 Depos-
ited immune complexes activate the alternative complement
pathway (with deposition of C3) and recruit inflammatory cells

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Fig. 2 Pathogenesis of IgA glomerulonephritis. In patients with IgA neph-
ropathy (IgAN), galactose-decient IgA1 is recognized by anti-glycan IgG
antibodies. The formed immune complexes cannot enter the space of
Disse due to their size and interact with the asialoglycoprotein receptor
(ASGP-R) on hepatocytes, but are able to pass through the larger fenestrae
in the glomerular capillaries overlying the mesangium. These deposited
complexes induce glomerular injury by activation of the alternative com-
plement pathway and recruiting inammatory cells (with permission from
reference 15).
causing glomerulonephritis.12,15 Deposition of IgA1-containing
immune complexes in other sites (skin, gut, joints) leads to
organ-specific clinical manifestations of HSP.
Clinical Manifestations
HSP is a systemic vasculitis with multiorgan involvement. The
classic tetrad of signs and symptoms includes: 1/ palpable
purpura, 2/ arthritis or arthralgia, 3/ abdominal pain, and 4/
renal disease.
Purpura
Skin involvement is present in all children with HSP.8 Petechiae
and palpable purpura are the most common, but erythematous,
macular, urticarial or even bullous rashes have also been
observed. Purpura is characteristically distributed symmetrically
over the extensor surfaces of the lower limbs, buttocks and
forearms (Fig. 3) with involvement of trunk and face described
occasionally in younger children. Recurrence of purpura, which
might be associated with more severe renal involvement, is
observed in 25% of children with HSP.
Arthritis/arthralgia
Arthritis/arthralgia is present in three quarters of children with
HSP.16 Joint involvement is usually oligoarticular with large
joints of the lower extremities (knee, ankle, hip) most com-
monly affected. There is usually prominent periarticular swell-
ing, tenderness and pain; erythema and joint effusion are rare.
Arthritis is non-deforming and heals without chronic damage
within a few weeks.
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HSP in children
Fig. 3 Purpuric skin changes in a patient with HSP.
Abdominal pain
Approximately two thirds of children with HSP develop
abdominal pain,17 usually diffuse, increasing after meals, and
sometimes associated with nausea and vomiting. These symp-
toms are caused by submucosal haemorrhage and oedema of
the bowel wall, predominantly affecting the proximal small
bowel. The most severe gastrointestinal complication is intus-
susception, affecting 34% patients with HSP. In 60% of these
cases, it is limited to small bowel. Clinical presentation of intus-
susception is characterised by severe abdominal pain, often
colicky in nature and vomiting. Other significant, though less
common gastrointestinal complications are gangrene of the
bowel, bowel perforation and massive haemorrhage.
Renal disease
Renal involvement is reported in 2055% of children with
HSP.18,19 The most common finding is isolated microscopic hae-
maturia, usually developing within 4 weeks of the onset of the
disease. Proteinuria of variable degree might be present, and
if severe can present as nephrotic syndrome. Hypertension
might develop at the onset or during recovery. Renal function
is usually normal but the occasional patient might present
with a progressive glomerulonephritis with significant renal
impairment.
Other less common clinical manifestations of HSP include
cerebral vasculitis, scrotal or testicular haemorrhage, and inter-
stitial pulmonary haemorrhage.2022 Distal ureteric vasculitis
resulting in ureteric stenosis, presenting as renal colic has also
been described.23 Potential complications of HSP are summa-
rised in Table 2.
Diagnosis
Diagnosis of HSP is based on the presence of purpura (palpable)
or petechiae (without thrombocytopaenia) with lower limb pre-
dominance (mandatory criterion) plus at least one of the flowing
four features: (1) abdominal pain; (2) arthritis or arthralgia; (3)
leukocytoclastic vasculitis or proliferativeglomerulonephritis
with predominant deposition of IgA on histology; (4) renal
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HSP in children P Trnka

Table 2 Possible complications of HenochSchnlein purpura

Renal
Glomerulonephritis
Nephrotic syndrome
Renal failure
Ureteric obstruction
Gastrointestinal
Intussusception
Gangrene of the bowel
Bowel perforation
Gastrointestinal haemorrhage
Central nervous system
Cerebral haemorrhage
Seizures
Paresis
Peripheral neuropathy
Other
Pulmonary haemorrhage
Testicular haemorrhage
Scrotal haemorrhage Fig. 4 Target sign on transverse ultrasound of an intussusception. The
Myositis concentric mass represents the tissue layers in the bowel wall of the
Myocarditis intussusceptum and the intussuscipiens. The curved, echogenic (bright)
area is due to the trapped mesenteric fat (with permission from
reference 26).

involvement (haematuria, red blood cell casts or proteinuria).24

Laboratory tests are complementary in assessing renal involve-
ment (urinalysis, urine microscopy, serum creatinine), and
imaging studies are helpful in the evaluation of abdominal
involvement and its potential complications (intussusception). In
children with incomplete or unusual presentation, biopsy of the
affected organ (skin, kidney) confirms the diagnosis.
approaching 100% in experienced hands.26 Concentric rings of
tissue representing components of bowel and mesenteric fat
create a classic target sign (Fig. 4). The classic meniscus sign of
intussusception on contrast enema, where the apex of the intus-
susception projects into the contrast material, is not present in
cases of intussusception limited to small bowel.

Urinalysis
Every child with HSP should have urinalysis performed at diag-
nosis and during follow-up. Dipstick assessment of urine for
blood and protein is a good screening test for nephritis. Urine
microscopy may reveal dysmorphic red cells and red-cell casts.
Positive dipstick reading for protein requires quantification of
protein excretion either by measuring protein/creatinine ratio
on a first morning urine sample or protein excretion on a timed
urine sample (24-hour collection).
Blood tests
There are no blood tests specific for HSP and measurement of
serum levels of total IgA is not helpful in confirming the diag-
nosis or providing prognostic information. Galactose-deficient
IgA1 serum levels seem to distinguish patients with HSP nephri-
tis from patients without nephritis, and might become an impor-
tant commercially available biomarker in the future.14,25
Imaging
Not all patients with HSP require diagnostic imaging, which is
generally reserved for children with abdominal pain in whom
intussusception is suspected. Abdominal ultrasound is the tech-
nique of choice with the accuracy in diagnosing intussusception
Histology
Biopsy of the affected skin reveals leukocytoclastic vasculitis
with deposition of IgA-containing immune complexes, pre-
dominantly in small vessels in the papillary dermis (primarily
venules). Neutrophils undergo destruction (leukocytoclasis)
with destructive fragmentation of the nuclei of dying cells
(karyorrhexis) during apoptosis or necrosis (Fig. 5). Deposits of
IgA and C3 in the dermal capillaries of purpuric lesions and
uninvolved skin by immune-fluorescent staining are considered
valid diagnostic criterion, with 100% specificity in combination
with leukocytoclastic vasculitis.8
Kidney biopsy is usually performed in patients with uncer-
tain diagnosis and in those with more severe kidney involve-
ment (rapidly progressive nephritis, nephrotic syndrome). In
general, there is a correlation between the severity of renal
manifestations and findings on kidney biopsy. Light microscopy
findings can range from mild mesangial proliferation to severe
crescentic glomerulonephritis. Diffuse mesangial IgA deposits
seen on immunofluorescence are the hallmark of HSP nephritis
(Fig. 6) and co-deposition of C3 complement (75%) might also
be present. The absence of the classical complement pathway
components (C1q and C4) distinguishes HSP nephritis from
other forms of immune-mediated glomerulonephritis, such as
lupus nephritis. Electron microscopy shows electron dense

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Fig. 5 Leukocytoclastic vasculitis of the skin in a child with Henoch
Schnlein purpura. Supercial dermal vessels showing inammatory inl-
trate consisting predominantly of neutrophils and eosinophils (arrows)
[haematoxyillin/eosin; magnication 200]. (Courtesy of Dr Leo Francis,
Pathology Queensland, Royal Brisbane and Womens Hospital, Brisbane).
Fig. 6 Deposition of IgA immunoglobulin in HenochSchnlein purpura
nephritis. Immunohistological staining demonstrates granular deposition
of IgA immunoglobulin in the mesangium of the affected glomerulus [mag-
nication 200]. (Courtesy of Dr Leo Francis, Pathology Queensland, Royal
Brisbane and Womens Hospital, Brisbane).
deposits in the mesangial areas. The current classification of
HSP nephritis is based on the extent of proliferation and the
presence of crescents on light microscopy,27 but other histologi-
cal findings, such as mesangial/subendothelial deposits, the
extent of tubulointerstitial damage or glomerular sclerosis
might be better predictors of the outcome.28,29
Management of HSP
Management of HSP includes supportive care, symptomatic
therapy and, in some cases, immunosuppressive treatment.
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HSP in children
The basic principles of supportive care consist of maintenance
of good hydration, symptomatic pain relief and monitoring
for the development of complications. If adequate hydration
cannot be maintained orally, intravenous fluids should be con-
sidered. Parenteral nutrition is usually unnecessary, except in
cases with prolonged severe abdominal involvement precluding
enteral feeding. Patients with severe abdominal pain need
prompt evaluation and investigations to exclude intussuscep-
tion. In cases with sudden change of mental status, intracranial
haemorrhage should be excluded with appropriate imaging.
Arthritis/arthralgia usually responds well to non-steroidal anti-
inflammatory drugs (NSAIDs), but occasionally requires opioids
for adequate symptomatic relief.30 Treatment is usually well
tolerated and is not associated with an increased risk of gastro-
intestinal bleeding. Patients with compromised renal function
taking NSAIDs need close monitoring of their fluid status, blood
pressure and renal function.
The use of glucocorticosteroids (GCS) in HSP has been source
of controversy for many years. While the suggested benefits of
early GCS treatment have included shortened duration of
abdominal pain, decreased risk of intussusception and decreased
risk of surgical intervention,3133 the quality of evidence is gen-
erally poor, having come from mostly from small studies or case
reports. In clinical practice, short courses of GCS are being used
in patients with severe abdominal pain, usually with rapid
symptomatic improvement.30 This treatment cannot be recom-
mended in all patients with HSP since the majority will improve
spontaneously. While some reports have suggested that early
treatment with GCS might prevent development of nephritis
and chronic kidney disease,32 a recent Cochrane review con-
cluded that there is no evidence from randomised controlled
trials that the use of GCS prevents kidney disease in children
with HSP.34
Immunosuppressive treatment of HSP nephritis is used in
patients with severe kidney involvement (nephrotic range pro-
teinuria and/or progressive renal impairment). In these cases,
renal biopsy should be considered before treatment. Mild renal
involvement (microscopic haematuria or mild proteinuria) does
not require biopsy or immunosuppressive treatment, but these
children need close follow-up.
In patients with rapidly progressive glomerulonephritis or
nephrotic syndrome (usually accompanied by crescents on
kidney biopsy), pulse intravenous methylprednisolone followed
by 3 to 6-month course of oral steroids is most commonly
used.35 A current KDIGO guideline suggests adding cyclophos-
phamide to steroid treatment for crescentic glomerulonephri-
tis36 even though the quality of evidence is low with a lack of
demonstrated improvement in renal outcome.37,38 Plasmapher-
esis has also been used in children with rapidly progressive
glomerulonephritis, but it is difficult to assess its efficacy due to
selection bias (used in the most severe cases) and concurrent
administration of other immunosuppressive treatments.39
Recent studies in children with HSP nephritis and nephrotic
syndrome suggest a potential benefit of cyclosporine A (CsA) in
achieving remission of proteinuria and histological improve-
ment of nephritis on follow-up kidney biopsies.40,41 Other treat-
ments used with some success in small studies include
intravenous immunoglobulin, combined therapy of immuno-
suppression and anti-clotting therapy (warfarin, dipyridamol
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HSP in children
and acetylsalicylic acid), tonsillectomy, and B-cell depletion
with rituximab and mycophenolate mofetil.4246 The efficacy of
these treatments is yet to be tested in prospective clinical trials.
Use of angiotensin-converting enzyme inhibitors (ACEIs) or
angiotensin receptor blockers (ARBs) has become an accepted
treatment of HSP nephritis with persistent proteinuria, with
beneficial effects not only on reduction of proteinuria but also
on inhibition of renal fibrosis. Although there are no available
studies on the efficacy of ACEIs or ARBs in HSP nephritis,
long-term data showing their beneficial effect on renal survival
and improvement of proteinuria in patients with IgA nephropa-
thy, a disease with the same pathophysiology, are encouraging.47
Prognosis of HSP
In the majority of children, the outcome of HSP is excellent with
spontaneous resolution of symptoms and signs. HSP recurs in
approximately one third of patients, typically within 4 months
of the initial presentation. Recurrent purpura can be occasion-
ally associated with joint complaints and episodes of gross hae-
maturia although each subsequent episode is generally milder
and shorter. The long-term morbidity of HSP is related to the
degree of HSP nephritis.
In unselected cohorts of children, HSP nephritis is a mild
disease, characterised by microscopic haematuria and minimal
proteinuria, with <1% risk of progression to end-stage kidney
disease (ESKD).18 Reports from tertiary centres indicating that
1030% of children will develop ESKD are likely to overesti-
mate the true risk of ESKD due to the selection of cases with
more severe renal impairment seen in these centres.48,49 Chil-
dren at risk are those with nephrotic or nephritic/nephrotic
syndrome or renal failure at presentation, and those with
impaired kidney function and persistent proteinuria after
several years of follow-up.29 Children with uncomplicated HSP
are usually managed in the primary care setting either by a GP
or a paediatrician. The aim of the initial follow-up is to iden-
tify patients with worsening kidney involvement and is based
on serial urinalyses, blood pressure measurement, blood tests
to assess kidney function and exclusion of other causes of glo-
merulonephritis. A practical pathway for detection and referral
of children with HSP nephritis to a paediatric nephrologist
during the first 612 months after diagnosis has been devel-
oped (Fig. 7).50 The involvement of a paediatric rheumatologist
in cases of severe arthritis/arthralgia might also be warranted.
Histological recurrence of HSP nephritis (IgA deposition)
in transplanted kidneys can be as high as 60% but is rarely
associated with clinical recurrence.51 Long-term outcomes of
transplanted kidneys in patients with HSP nephritis are com-
parable to other primary diseases with 90% survival at 10
years.52
Conclusion
HSP is a common childhood vasculitis with a good outcome in
the majority of affected children. However, there is a small
subgroup of children who will develop significant renal impair-
ment and some of them will eventually progress to ESKD and
require kidney transplantation. To predict which patients are at
risk of long-term renal sequelae, we need better biomarkers
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reflecting the tissue damage, such as urinary biomarkers of
tubulointersitial (profibrotic cytokines) or glomerular (urinary
podocytes) injury. Furthermore, we need well-designed multi-
centre randomised controlled prospective studies on treatment
of HSP nephritis to answer questions such as who should be
treated, when and with what medication. It is a hope that with
the early detection of HSP patients at risk of developing ESKD
and their appropriate and timely treatment, the outcomes of
these children will improve.
Acknowledgements
I thank Dr Leo Francis, Pathology Queensland, Royal Brisbane
and Womens Hospital, Brisbane for providing the histological
samples and Dr Steve McTaggart, Queensland Child and Ado-
lescent Renal Service, Brisbane for reviewing this manuscript.
Multiple Choice Questions
Q1. Which one of the following mechanisms plays the most
important role in pathogenesis of HenochSchnlein
purpura:
a. Anaphylactoid reaction to various medications (non-
steroidal anti-inflammatory drugs, antibiotics)
b. Immune reaction to infective agents (bacteria, viruses)
involving the classical complement pathway
c. Inherently deficient glycosylation of IgA1 molecules pre-
disposing to formation of immune complexes and poor
clearance of IgA1 from circulation
d. Abnormally expressed asialoglycoprotein receptor on
hepatocytes leading to poor clearance of IgA1 molecules
from circulation with subsequent formation of immune
complexes
e. Increased production of normally glycosylated IgA1
immunoglobulin in affected individuals leading to the
deposition of this immunoglobulin in the vessels of
affected organs
A1. Correct answer is c.
People with HSP (and IgA nephropathy) have inherited predis-
position to abnormal glycosylation of IgA1 molecules. Following
intercurrent infection, abnormally glycosylated molecules
form large immune complexes that are poorly cleared by
the liver and deposit in the vessels of the affected organs.
Anaphylactoid reactions do not play role in pathogenesis of HSP.
Activation of the alternative complement pathway plays a role
in organ damage; the classical complement pathway is not
involved in pathogenesis of HSP. Individuals with HSP have
normal asialoglycoprotein receptors in the liver. Abnormal
glycosylation of IgA1 molecules rather than increased produc-
tion of normally glycosylated IgA1 is the main predisposing
factor in pathogenesis of HSP.
Q2. Which one of the following statements regarding diagnosis
of HenochSchnlein purpura is correct:
a. There is no specific test for HSP and the diagnosis is
made based on the clinical findings
b. Total IgA in serum is elevated in the majority of patients
with HSP
c. Measurement of galactose-deficient IgA1 in serum is a
useful and widely available test for HSP
Journal of Paediatrics and Child Health 49 (2013) 9951003
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P Trnka HSP in children

Fig. 7 Suggested clinical pathway for detection and referral of patients with HSP nephritis. This pathway has been adapted from local guidelines developed
by Dr D Hothi and Bristol Paediatric Nephrologists, and reprinted with permission from reference 50. Abbreviations: EMU early morning urinalysis; UP:PC
urine protein/creatinine ratio.

d. Contrast enema is an imaging test of choice for diagnosis
of intussusception in children with HSP
e. Kidney biopsy is usually performed in children with HSP
who have haematuria or proteinuria on presentation
A2. Correct answer is a.
Diagnosis of HSP is clinical and is based on presence of purpura or
petechiae plus one of the following: abdominal pain, arthritis or
arthralgia, histological presence of leukocytoclastic vasculitis or
proliferative glomerulonephritis, or renal involvement (haema-
turia, red blood cell casts or proteinuria). There are no tests
specific for HSP. Serum level of total IgA is not clinically useful
test since it is elevated in 50% of patients with HSP. Serum levels
of galactose-deficient IgA1 can distinguish patients with HSP from
healthy controls, but this test is not widely available for clinical
purposes. Contrast enema would miss intussusception limited to
the small bowel; abdominal ultrasound is the imaging test of
choice. Kidney biopsy is usually done in patients with uncertain
diagnosis and in those with more severe kidney involvement
(rapidly progressive nephritis, nephrotic syndrome).
Q3. Which one of the following is the correct answer with
regard to management of HenochSchnlein purpura:
a. All children with HSP should be admitted to hospital for
close monitoring and intravenous hydration
b. Treatment with non-steroidal anti-inflammatory drugs is
contraindicated in children with HSP because of the
potential adverse effects on the kidneys

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Journal of Paediatrics and Child Health 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians)
HSP in children
c. Early treatment with glucocorticosteroids will prevent
development of HSP nephritis and chronic kidney
disease
d. Children with HSP who have persistent microscopic
haematuria require kidney biopsy and immunosuppres-
sive treatment
e. Treatment with angiotensin-converting enzyme inhibi-
tors or angiotensin receptor blockers is an accepted
treatment of HSP nephritis in children with persistent
proteinuria
A3. Correct answer is e.
Majority of children with HSP can be managed out of hospital
with close monitoring in an outpatient setting. NSAIDs are
useful treatment for arthralgia/arthritis in children with HSP,
but potential side effects on the kidney must be kept in mind
and close monitoring of kidney function is important. There is
no evidence from randomised controlled trials that early use of
glucocorticoids prevents kidney disease in children with HSP.
Persistent microscopic haematuria is a common finding in chil-
dren with mild HSP nephritis; most of these children continue to
have normal kidney function and will do well. In children with
HSP nephritis and persistent proteinuria (especially those who
are also hypertensive), treatment with angiotensin-converting
enzyme inhibitors or angiotensin receptor blockers seems to
slow down the progression of kidney disease. At what level of
proteinuria one should start this treatment is, however, unclear.
References
1 Rook A. William Heberdens cases of anaphylactoid purpura. Arch.
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