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HenochSchönlein purpura
E J Tizard and M J J Hamilton-Ayres

Arch. Dis. Child. Ed. Pract. 2008;93;1-8

doi:10.1136/adc.2004.066035

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1
Department of Paediatric
Nephrology, Bristol Royal
Hospital for Children, Bristol, UK;
2 this disorder was probably that of a young boy
Department of Paediatrics,
Cheltenham General Hospital,
Cheltenham, UK
Correspondence to:
Dr E J Tizard, Department of
Paediatric Nephrology, Bristol
Royal Hospital for Children,
Upper Maudlin Street, Bristol
BS2 8BJ, UK; jane.tizard@ubht.
nhs.uk
Accepted 9 July 2007
Arch Dis Child Educ Pract Ed 2008;93:1–8. doi:10.1136/adc.2004.066035
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Henoch–Schönlein purpura
E J Tizard,1 M J J Hamilton-Ayres2
Henoch–Schönlein purpura (HSP) is the common-
est vasculitis of childhood. The first description of
with ‘‘bloody points’’ over the shins of his legs,
abdominal pain, blood in the stools and urine and
painful subcutaneous oedema, described by
William Heberden in 1801. In 1837 Johann
Schönlein described the association of purpura
and joint pain as ‘‘Peliosis rheumatica’’. Eduard
Henoch, Schönlein’s former student, noted gastro-
intestinal involvement in association with purpura
and arthritis in 1868 and subsequently he recorded
renal involvement too.1
DIAGNOSIS
Until recently the 1990 American College of
Rheumatology criteria for HSP were the most
commonly used criteria for diagnosis.2 This classi-
fication was modified but has now been super-
seded by the European League Against
Rheumatism (EULAR) and Paediatric
Rheumatology European Society (PReS) consensus
criteria for the classification of childhood vasculitis.
The group reached consensus that the classification
of HSP should be the finding of palpable purpura in
the presence of one of either diffuse abdominal
pain, a biopsy showing predominant IgA deposi-
tion, arthritis or arthralgia and/or renal involve-
ment (any haematuria and/or proteinuria)
(table 1).3
EPIDEMIOLOGY
Henoch–Schönlein purpura is found in all age
groups, from the age of a few months to late
adulthood but it is more common in young
children, with over 50% under 5 years and over
75% under 10 years. The clinical features of HSP
are often atypical at extremes of age and the illness
is more severe in adults while children under 2
years old are less likely than older children to have
nephritis or abdominal complications. The
reported incidence of HSP varies from 10–20.4 per
100 000 children.4–7 It is highest in younger children
with 22.1/100 000 in the under 14 years and 70.3/
100 000 in the 4–7 year age group being reported in
one series from the West Midlands.4 In this study
Table 1 Classification criteria for Henoch–Schönlein
purpura3
Palpable purpura (mandatory) in the presence of at least one of the
following four features:
c Diffuse abdominal pain
c Arthritis (acute) or arthralgia
c Renal involvement (any haematuria and/or proteinuria)
c Any biopsy showing predominant IgA deposition
Best practice
there was a significantly lower annual incidence in
black children compared with white or Asian
children. A male predominance is found in most
series in a ratio of up to 2:1 but female
predominance has also been reported. HSP is more
common in the winter, autumn and spring than
the summer, which supports the view that an
infectious trigger may have a role in its pathogen-
esis.8 9
In a large Italian cohort two thirds of patients
had a possible infective trigger before the onset of
HSP, 63/150 had a respiratory tract infection and
37/150 had other infections or fever.9 Many
organisms have been implicated in precipitating
HSP but Group A b hemolytic streptococcus has
been the commonest organism cultured in up to
36% of those tested in one series.6
Other organisms that have been identified
include hepatitis A and B, CMV, HIV, adenovirus,
Mycoplasma, herpes simplex, helicobacter pylori,
toxacara canis, human parvovirus B19, varicella,
and scarlet fever but many of the reports are
anecdotal. HSP has also followed vaccinations
including measles, mumps, rubella (MMR), pneu-
mococcal, influenza, meningococcal and hepatitis
B.8 9 Various drugs have been implicated as triggers
although none has been proved to be associated in
controlled studies.
CLINICAL FEATURES
It is the classical rash which often precipitates
presentation of patients with HSP. Other system
involvement may be present from the onset, or
alternatively an evolving picture may develop over
the course of several days to several weeks. In one
study abdominal pain or arthritis preceded the rash
by 1–14 days in 43/100 patients.8 As many cases
follow an upper respiratory tract infection, the
onset of the disorder may be accompanied by
systemic symptoms such as fever and malaise.
Skin
The typical rash is of palpable purpura often
symmetrically distributed over the extensor,
dependent surfaces of the lower limbs and but-
tocks. It may involve the arms, face and ears but
usually spares the trunk. The purpura range from
petechiae to large ecchymoses and may be preceded
by urticarial or erythematous, maculopapular
lesions. Severe bullous lesions are rare in children,
occurring in about 2% of patients (figs 1–3).
Gastrointestinal involvement
The reported incidence of gastrointestinal involve-
ment is generally between 50–75% of cases with
the most common presentation being colicky
abdominal pain.8–10 Other symptoms include
1
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Best practice
Figure 1 Patient A: urticarial rash which may precede
typical vasculitic rash.
vomiting and gastrointestinal haemorrhage mani-
festing as overt bleeding or positive stools for
occult blood. Massive gastrointestinal haemor-
rhage is rare, being reported in about 2% of
patients. The symptoms result from oedema and
haemorrhage of the bowel wall due to vasculitis.
Intussusception is also a rare complication but is
important to exclude as delay in management can
result in ischaemic bowel. A protein losing entero-
pathy, pancreatitis and hydrops of the gall bladder
are also described. It must be remembered that
oedema secondary to hypoalbuminaemia is likely
to be due to either nephrotic syndrome or a protein
losing enteropathy or a combination of both.
Joints
Arthritis or arthralgia may be the presenting
symptom in 15–25% of cases but overall up to
82% of patients have a degree of joint involvement
during the illness.8 9 The arthritis usually affects
the large joints of the lower limbs including knees,
ankles, feet and hips although upper limbs may be
affected too. In a retrospective review of 100
patients, 72% had feet and ankle involvement,
50% had knee involvement, 26% had hands and
wrist involvement and 10% had elbow involve-
ment.8 Symptoms include pain, swelling and
Figure 2 Patient A: two weeks later. Typical vasculitic
rash.
2 Arch Dis Child Educ Pract Ed 2008;93:1–8. doi:10.1136/adc.2004.066035
Figure 3 Haemorrhagic bullae in Henoch–Schönlein
purpura.
decreased range of movement and although joint
involvement can be debilitating it does not result
in permanent damage.
Renal
Renal involvement in HSP is reported to occur in
12–92% of cases but in unselected series it is more
commonly between 20–60%.7–9 11–14 Renal disease is
manifest as haematuria, proteinuria, nephrotic
syndrome/nephritis, renal impairment and hyper-
tension. This develops within four weeks in 75–
80% and within three months in 97–100% of
cases.8 12 14 15 However, a few cases will develop
even years after the initial presentation.16 In
unselected series the incidence of severe renal
disease including acute nephritis, nephrotic syn-
drome or renal impairment is 5–7%.7 11
Hypertension may be found in the presence of
renal involvement. However, occasionally patients
with HSP may develop hypertension without
evidence of renal involvement.17 If it does not
resolve as the HSP resolves further investigations
are warranted.
Neurological
Neurological symptoms are rare although non-
specific headache followed by subtle encephalo-
pathy with minimal changes in mental status, such
as labile mood, apathy and hyperactivity may be
more common than previously thought. In a
prospective study 31% of 26 patients had headache
and 46% had abnormal EEGs.18 Seizures occurred in
2 out of 100 patients in one series.8 Subdural
haematoma, subarachnoid haemorrhage, cerebellar
haemorrhage, intraparenchymal bleeding and
infarction have also been documented in case
reports.
Pulmonary
Pulmonary involvement in HSP is rare. Although it
occurs more often in adults, isolated case reports
have been published describing pulmonary compli-
cations in children.19 The most common and severe
manifestation is diffuse alveolar haemorrhage
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(DAH) but it may present as interstitial pneumo-
nia or interstitial fibrosis.
Urological
Orchitis is a relatively common finding in boys
with HSP being reported in up to 24%.9 20 In a
series of 93 boys with HSP, 27 had involvement of
the male genital system.20 Twenty two of these
had a swollen and tender scrotum, 10 bilateral and
12 unilateral and five had swelling of the penis.
Three of the 22 patients with scrotal swelling were
investigated radiologically and eight had surgical
exploration. None of these patients had torsion but
surgical exploration remains indicated if the
diagnosis of the syndrome is not beyond doubt
and torsion cannot be excluded on clinical grounds.
Genital swelling may also be due to more general-
ised oedema secondary to hypoalbuminaemia from
renal or gastrointestinal involvement.
INVESTIGATIONS
There are no specific laboratory tests that have
been shown to be helpful in making the diagnosis
which is usually based on clinical features. The
investigations are aimed at excluding other diag-
nostic possibilities and assessing the extent of
organ involvement such as the kidneys.
Initial investigations should include a full blood
count, clotting, urea, electrolytes and creatinine,
liver and bone profile and urinalysis to identify
haematuria and proteinuria. If proteinuria is
identified on dipstick then an early morning
protein:creatinine ratio should be requested. If
the diagnosis is in doubt then a full autoimmune
profile (AIP) including ANA, dsDNA, ANCA,
immunoglobulins, C3 and C4 should be performed.
These investigations may show anaemia, leuko-
cytosis, a raised erythrocyte sedimentation rate
and in a few, abnormal renal or liver function
tests.9 Thrombocytosis has been associated with
more severe disease. Standard coagulation studies
are usually normal; however Factor XIII activity
has been found to be reduced and associated with
more severe disease,21 but is currently not recom-
mended in routine assessment.
IgA is elevated in half the children; however
there is no correlation with disease severity. C3 and
C4 have been reported to be low in a few patients
but in general the basic immunological investiga-
tions are normal.
If the child is systemically unwell evidence for a
site of infection should be sought with blood
cultures, swabs, urine for microscopy and culture
and a chest x ray if clinically indicated. Evidence of
recent streptococcal infection may be indicated by
raised ASOT and antiDNAse B titres. If renal
disease is present it is also appropriate to measure
these as sometimes a post-streptococcal glomeru-
lonephritis may complicate the picture. Viral
investigations (serology and throat swabs) may
reveal a precipitating cause of HSP and should be
considered in the unwell child but are unlikely to
alter management.
Arch Dis Child Educ Pract Ed 2008;93:1–8. doi:10.1136/adc.2004.066035
Best practice
SPECIFIC ORGAN INVOLVEMENT MAY WARRANT
MORE DETAILED INVESTIGATIONS
Renal
If there is evidence of renal involvement, including
haematuria, proteinuria, renal impairment or
hypertension the pathway suggested in figure 4 is
recommended.
Abdominal ultrasonography may show thicken-
ing of both small and large intestinal walls. As the
most common form of intussusception is within
the small bowel, its frequent inaccessibility to
demonstration by contrast enema means that
ultrasonography is the investigation of choice.
PATHOLOGY
In the skin there is a leucocytoclastic vasculitis
with perinuclear infiltration of polymorphs and
mononuclear cells. There may be necrosis of small
blood vessels and platelet thrombi. IgA is found in
most purpuric lesions and can also be found in
non-affected areas. In practice skin biopsies are
rarely required to make the diagnosis but may be
useful in severe, atypical or recurrent disease when
the diagnosis is in doubt.
The renal lesion of Henoch–Schönlein nephritis
is indistinguishable from that of IgA nephropathy
characteristically showing a focal and segmental
proliferative glomerulonephritis. The proliferation
of extracapillary cells may result in crescent
formation. Findings are graded according to the
classification of the International Study for
Kidney Diseases in Children (ISKDC).22
Immunofluorescence usually reveals mesangial
IgA with IgG, C3 and fibrin. It is the non-renal
clinical features that differentiate IgA nephropathy
from HSPN.
Indications for renal biopsy include acute
nephritic syndrome/acute renal impairment or
nephrotic syndrome/nephrotic range proteinuria
(urine protein:urine creatinine ratio .250 mg/
mmol) for 4–6 weeks (fig 4).
IMMUNOPATHOLOGY
Although the aetiology of HSP is not clear there is
evidence to support immunopathological mechan-
isms. Widespread abnormalities in IgA have been
described including altered levels of IgA, increased
levels of IgA class Ab such as IgA RF, IgA ANCA,
IgA immune complexes and IgA deposition in renal
and skin biopsies. It has been suggested that IgA
ANCA could have a role in the diagnosis of HSP.23
IgA occurs in two isotypes—IgA1 and IgA2.
Sixty per cent of IgA in secretions is IgA2 and
generally polymeric, while serum IgA is predomi-
nantly IgA1 and 90% is monomeric. However in
HSP there is predominantly deposition of poly-
meric IgA1 in skin, gastrointestinal and glomerular
capillaries.
In patients with HSP-associated nephritis there
have been reports of significantly elevated levels of
abnormally glycosylated IgA1 compared with
those without nephritis and controls.24 This
abnormality has also been shown in adults with
3
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Best practice
Figure 4 Detection and
referral of patients with
Henoch–Schönlein purpura
nephritis (adapted from
local guidelines developed
by Dr D Hothi and Bristol
Paediatric Nephrologists).
IgA nephropathy supporting the view of a com-
mon immunogenetic pathway in IgA-mediated
glomerular disease.
Clinically the likelihood of a common pathogen-
esis is supported for example by the cases of
patients with IgA nephropathy developing HSP
and of simultaneous development of IgA nephro-
pathy and HSP within sibships.25
GENETIC POLYMORPHISMS
A number of studies have tried to identify genetic
polymorphisms that may be associated with the
development of HSP or associated with disease
severity or development of HSP nephritis.
Interleukin 1b expression has been found in skin
biopsies of patients with HSP and a polymorphism
of this gene has been associated with severity of
nephropathy and renal sequelae.26 Polymorphisms
of cytokine genes may mediate an abnormal
inflammatory response leading to severe sequelae.
Investigation of the association of renin-angioten-
sin system gene polymorphisms with susceptibility to
HSP and HSP nephritis (HSN) has produced conflict-
ing results,27 28 with the polymorphisms of the ACE
gene and angiotensinogen (Agt) gene possibly influ-
encing the risk of developing HSP and Agt being
shown to be associated with HSN. However further
studies are needed to investigate this finding.
4 Arch Dis Child Educ Pract Ed 2008;93:1–8. doi:10.1136/adc.2004.066035
DIFFERENTIAL DIAGNOSIS
The diagnosis of HSP is usually obvious due to the
characteristic rash. However, in the event of an
atypical picture other conditions should be con-
sidered. These include other forms of vasculitis,
particularly small vessel vasculitis such as micro-
scopic polyarteritis, Wegener’s granulomatosis and
isolated cutaneous leucocytoplastic vasculitis.
Systemic lupus erythematosus (SLE) may also be
associated with a similar vasculitic rash. Some of
the vasculitides and SLE will be distinguished by
the presence of ANCA or ANA/dsDNA respec-
tively.
Children with HSP are usually systemically well
whereas the child with septicaemia is generally
clinically obvious. However, if there is any doubt
systemic antibiotics should be given pending
results of cultures. Other clotting disorders or
thrombocytopenia should be identified through
appropriate haematological investigations.
Acute haemorrhagic oedema of infancy (AHEI) is
sometimes considered a variant of HSP.29 There are
large purpuric lesions in a target-like pattern with
oedema mainly affecting the face, auricles and
extremities. Although the histological findings are
similar only a third have IgA deposition and it is
generally regarded as a distinct entity which rarely
involves other organs.
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TREATMENT
The natural history of HSP is predominantly
resolution of all symptoms except for the renal
disease which may be associated with long-term
complications. Therefore drug therapy is aimed at
more rapid symptomatic relief, except in the case
of significant renal disease or severe organ
involvement such as cerebral disease when treat-
ment may have a significant impact on long-term
outcome.
Gastrointestinal disease
Steroids are often used for the relief of abdominal
pain although until recently there have been no
randomised controlled studies on which to base
this treatment. In a retrospective analysis children
treated with 2 mg/kg prednisolone were more
likely to have resolution of their abdominal pain
than those on no treatment in the first 24 h (44%
vs 14%) but by 72 h pain had resolved in 75% of
both groups.30 Other retrospective studies and case
series have also suggested a benefit of steroids in
the treatment of abdominal pain.31 In one pro-
spective, randomised, placebo controlled study of
oral prednislolone in 40 children32 there was no
significant difference in the development of gastro-
intestinal complications, although two in the
placebo group developed intussusception compared
with none in the steroid group. There was also a
tendency for abdominal symptoms to be more
prolonged in the prednislolone group. In the most
recently published prospective study, prednisolone
1 mg/kg daily (maximum dose 50 mg) for two
weeks with subsequent weaning over two weeks
was effective in reducing the intensity of abdom-
inal pain and the duration by a mean of 1.2 days.33
In severe gastrointestinal disease associated with
protein-losing enteropathy a combination of intra-
venous methylprednisolone (10 mg/kg (max dose
500 mg) daily for 3 days, followed by 2 mg/kg oral
prednisolone (max dose 80 mg) daily and weaning)
and an elemental diet has been successful (personal
experience).
Severe gastrointestinal haemorrhage has also
been treated with high dose methylprednisolone
(1 g 6 3 days) followed by 40 mg prednisolone
daily for a week in a 15-year-old boy.34 It is
important to exclude gastrointestinal complica-
tions which may be exacerbated by steroids,
before treatment and, in view of the association
of gastrointestinal haemorrhage with non-steroi-
dal anti-inflammatory drugs, it is recommended
that these should not be used in combination
with steroids in HSP.
Abdominal pain usually settles within a few
days with or without treatment. Occasionally
chronic abdominal pain may persist and metho-
trexate has been reported to be a useful steroid
sparing agent in this situation.35 Mycophenolate
has also been used for unresponsive and recurrent
abdominal pain.36 However the potential gastro-
intestinal side effects of Mycophenolate should be
considered.
Arch Dis Child Educ Pract Ed 2008;93:1–8. doi:10.1136/adc.2004.066035
Best practice
Joint involvement
Joint pain is often managed with non-steroidal
anti-inflammatory drugs but in severe cases ster-
oids have been used. In one prospective study
prednisolone appeared to reduce the severity of
pain compared with the placebo group.33 Non-
steroidal drugs should, however, be avoided in the
presence of renal disease.
Skin involvement
Skin disease rarely requires specific treatment but
bullous lesions have anecdotally been reported to
respond to steroids.37 Dapsone, an anti-leprotic
drug, has also been shown to be of benefit as a
steroid sparing agent, particularly for skin, but has
no effect on renal disease.38 39 A combination of
aspirin and colchicine has also been used for
chronic skin and joint disease.40
HSP nephritis
Many have looked for prognostic factors which
might help to select those patients who could
benefit from treatment. The natural history of HSP
is that most patients make a good recovery with
the incidence of severe long-term morbidity/
mortality being less than 5%.7 41 42 However, long-
term renal impairment occurs in 19.5% of those
with a history of nephritic or nephrotic syn-
drome.42 In the past HSP nephritis (HSPN) has
been reported to account for up to 15% of children
with end-stage renal failure (ESRF) (now termed
chronic kidney disease (CKD) stage 5).43 More
recent data show that HSPN accounts for 1.8–3%
of children with CKD stage 5,41 44 possibly suggest-
ing that more aggressive treatment may have had a
beneficial impact on the outcome.
The presence of nephrotic/nephritic syndrome
and renal impairment has been shown to be
associated with a poor prognosis. Tarshish reported
the outcome of 79 patients with heavy proteinuria
and ISKDC grade III histopathological changes,
some of whom were treated with cyclophospha-
mide as part of a prospective trial. Only 5/28 with
nephrotic level proteinuria and severe histopathol-
ogy recovered completely and no patient with .50
crescents on renal biopsy recovered completely.45 In
a review of 114 patients with a diagnosis of HSPN
69 patients with normal and 25 with minor urinary
abnormalities were designated to be the favourable
outcome group, and 15 with active renal disease
and five with renal failure the unfavourable group.
Nephrotic syndrome, decreased Factor XIII activ-
ity, hypertension and renal failure at onset were
more frequent in the unfavourable group.46 Other
risk factors for development of renal disease
include older age, abdominal pain and persisting
purpura.47 However, in a follow-up study of 219
adults and children Coppo found, on multivariate
analysis, that persisting proteinuria as opposed to
disease markers at onset is the best predictor of
outcome.48
In a Finnish study of 19 patients with nephrotic
range proteinuria the initial biopsy was not
5
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Best practice
predictive of outcome. Although severe changes on
early biopsy may lead to institution of successful
treatment, a biopsy that is too early may be falsely
reassuring and re-biopsy should be considered if the
clinical condition does not improve.49
The treatment of HSPN remains controversial.
In view of the evidence that severe proteinuria is a
risk for long-term renal damage there have been
attempts to prevent the development of renal
disease with the early treatment with steroids. In a
large study of 164 children with HSP but without
evidence of nephropathy at the onset, patients
were allocated alternately to receive prednislolone
1 mg /kg for two weeks or no treatment.50 None of
the treated group and 11.9% of the untreated
group developed evidence of nephropathy within
six weeks of the onset of HSP. Two others
developed nephropathy at two and six years after
the initial illness. The difference in the two groups
was highly significant suggesting a beneficial effect
of steroids. Another study in support of the use of
steroids was that of Kaku.47 In this retrospective
study of 194 patients without nephritis at onset,
79 were treated with steroids for symptoms of
abdominal pain or joint pain. A multivariate
analysis identified the use of steroids as having a
role in reducing the development of renal disease.
In contrast Saulsbury found no benefit in the use
of steroids in the prevention of nephritis. However,
in this report steroids were used to treat patients
with abdominal pain and as these are at higher risk
of developing renal disease it may have obscured
the benefit of steroids.51
The first randomised placebo controlled study of
prednislolone in early HSP, which included 40
children, did not show a benefit in the use of
steroids on the risk of renal involvement at one
year.32 Ronkainen’s larger randomised, placebo
controlled study of 171 patients showed no
significant benefit of steroids in the development
of renal symptoms overall.33 However, there was a
slightly more rapid resolution of renal symptoms in
the steroid treated group and there appeared to be
a specific benefit in a small subgroup of children
over 6 years who had or developed renal symptoms
in the first month. A recent systematic review of
the use of corticosteroids in HSP concluded that
based on the prospective studies from Ronkainen,33
Huber32 and Mollica50 there was a benefit of
steroids on reducing the persistence of renal
disease.52 However the study by Mollica only
included those without renal disease at presenta-
tion and the follow up in the Ronkainen study was
only 6 months. In addition, in the latter study the
definition of renal disease included those with
microscopic haematuria alone and therefore renal
disease of less significance in terms of long term
outcome. The authors of this review suggested that
larger prospective studies should be performed.
The largest prospective study to date which has
been undertaken in SW England and Wales
randomised 353 children to receive either predni-
solone or placebo for two weeks. The preliminary
results of this study have not shown any benefit of
6 Arch Dis Child Educ Pract Ed 2008;93:1–8. doi:10.1136/adc.2004.066035
early steroids in the prevention of renal disease at
one year.53 However, subgroup analysis is still
awaited (Dudley et al, unpublished data). Data
from this study was not available at the time of the
systematic review.
For those with established renal disease there is
again a lack of clear evidence of the benefits of
treatment. This is in part due to the fact that
relatively few patients have severe renal disease at
onset and therefore randomised controlled trials
are difficult to perform. However there are a
number of uncontrolled case series showing some
benefit of immunosuppressive therapy.
In a study of nine Japanese children with severe
proteinuric HSPN associated with ISKDC Grade III
or IV histological changes, early treatment with
oral prednisolone and cyclophosphamide resulted
in a favourable outcome in all patients.54 However,
this is an uncontrolled small study with a relatively
short follow-up period. Foster also demonstrated a
benefit of steroids and azathiaprine in patients, all
of whom had significant proteinuria or nephrotic
syndrome and three of whom had renal impair-
ment at onset.55 There has also been anecdotal
evidence in support of the use of cyclosporin.56
The evidence for the efficacy of treatment in the
patients with the most severe disease is limited but
the significant long-term consequences in some
patients make this an important area to consider.
There is again some evidence for the benefits of
intensive immunosuppression. Niaudet treated 38
children with severe HSPN (nephrotic syndrome
and/or 50% crescentic nephritis) with methylpred-
nisolone; seven also had cyclophosphamide with or
without plasma exchange. Four of these 38 com-
pared with 11/29 historical untreated controls
developed end stage renal failure.41 A good outcome
was found in 11 of 12 patients with rapidly
progressive glomerulonephritis, nine of whom had
60–90% crescents on biopsy, following aggressive
treatment with methylprednisolone, cyclophospha-
mide, oral prednisolone and dypiridamole.57 Plasma
exchange also appears to have a possible benefit
when used in conjunction with steroids and
cyclophosphamide if treatment is started early in
the disease,58 as has a combination of methylpredni-
solone and urokinase with or without cyclopho-
sphamide.59 In contrast a randomised study of 56
patients with severe nephritis showed no benefit of
cyclophosphamide on renal outcome.60
In this severe group there is some, albeit
uncontrolled, evidence to suggest that aggressive
therapy may be useful. In view of the potential
long-term consequences, treatment is often insti-
tuted in these patients and therefore timely
discussion/referral to a paediatric nephrologist is
essential (fig 4). Intensive treatment including
plasma exchange may also have a role in non-renal
involvement such as neurological disease.58
The antihypertensive and renoprotective effects
of angiotensin-converting enzyme inhibitors
(ACEI) are well established in adults with hyper-
tension and/or chronic renal failure. However, the
experience in children is less well documented. One
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study has shown a benefit of ACEI in the
treatment of hypertension and reducing protei-
nuria in 397 children with mild to severe chronic
renal failure.61 In addition a study of 66 children
and adults with IgA nephropathy but not HSPN
demonstrated a benefit of ACEI on the progression
of IgA nephropathy.62 A small case series showed a
stabilisation of renal function in children with
HSPN treated with fish oil with or without ACEI
therapy.63 Therefore by extrapolation there may be
a benefit in treating patients with evidence of
chronic disease following HSP, particularly with
significant proteinuria/hypertension, with an
ACEI. This should be discussed with a paediatric
nephrologist.
There are no convincing data to date to support
the use of fish oil in HSPN.
PROGNOSIS
Henoch–Schönlein purpura is generally a self-
limiting disease but about 33% have recurrence of
symptoms. Saulsbury reported 1–6 recurrences per
child occurring from two weeks to 18 months after
initial resolution of symptoms. Two per cent had
recurrences after four months and patients with
renal disease were more likely to have recurrences.8
The long-term outlook for children with HSP is
predominantly related to the renal disease. In two
unselected series chronic renal disease developed in
up to 1.4% of children at about eight years from
diagnosis.7 11 Mortality is also very unusual occur-
ring in ,1% in one series.7 Although no feature is
absolutely predictive of renal outcome many
studies confirm that features including nephrotic
syndrome, renal impairment, hypertension and
decreased Factor XIII at presentation are associated
with a poorer prognosis.46
In a follow-up study of 78 children who had had
HSPN the severity of the clinical presentation and
the renal biopsy were related to final outcome but
nothing reliably predicted individual outcome.
Forty four per cent of those with nephritis or
nephrotic syndrome at presentation followed for a
mean of 23.4 years had hypertension or impaired
renal function at follow-up whereas 82% of those
with minimal involvement initially were normal at
follow-up. However some patients who had
apparently recovered initially deteriorated in the
longer term follow-up.64 More optimistically about
50% of patients with severe disease at presentation
had a favourable prognosis.64
In this study 16/44 pregnancies were compli-
cated with proteinuria and/or hypertension. This
finding was confirmed in another long-term
follow-up study of 52 children with HSP in which
16/23 (70%) of subsequent pregnancies were
similarly complicated and five women had poor
renal outcome.65
In view of the potential late deterioration of
renal disease it is recommended that all patients
with a history of HSPN should have lifelong
follow-up. In contrast, those with normal urina-
lysis and blood pressure at six months are unlikely
to develop significant nephritis. A systematic
Arch Dis Child Educ Pract Ed 2008;93:1–8. doi:10.1136/adc.2004.066035
Best practice
review of 12 studies including 1133 children
concluded that of the children developing renal
involvement 97% did so within six months of
presentation.42 No long-term renal impairment
occurred after normal urinalysis. Therefore those
with no evidence of renal disease can be safely
discharged after 6–12 months. In cases where there
is recurrence of non-renal symptoms (for example,
rash with or without abdominal pain or arthritis)
surveillance should continue until six months from
the start of the last relapse.
CONCLUSION
Henoch–Schönlein purpura is the most common
vasculitis of childhood which is self-limiting in the
majority of cases. In the absence of either renal
disease or ongoing extra-renal symptoms, children
may be discharged at six months from diagnosis. In
contrast, in those with a history of nephritis the
risk of long-term renal damage warrants long-term
surveillance with particular vigilance in women
during pregnancy. Steroids appear to have a role in
managing the symptoms of abdominal pain but are
probably not indicated to prevent nephropathy. In
view of the small number of patients with severe
renal disease multicentre/international studies are
required to establish the role of immunosuppres-
sive therapy. On the current available evidence
patients with significant renal or extra-renal
disease should be assessed and considered for
treatment with steroids with or without immuno-
suppressive therapy.
Competing interests: None.
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