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Henoch-Schönlein Purpura in Children: An Updated Review

Article in Current Pediatric Reviews · May 2020

DOI: 10.2174/1573396316666200508104708

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Current Pediatric Reviews, 2020, 16, 265-276

REVIEW ARTICLE
ISSN: 1573-3963
eISSN: 1875-6336

Henoch-Schönlein Purpura in Children: An Updated Review

BENTHAM
SCIENCE

Alexander K.C. Leung1,*, Benjamin Barankin2 and Kin Fon Leong3

1
Department of Pediatrics, The University of Calgary, Alberta Children’s Hospital, Calgary, Alberta, Canada; 2Toronto
Dermatology Centre, Toronto, Ontario, Canada; 3Pediatric Institute, Kuala Lumpur General Hospital, Kuala Lumpur,
Malaysia

Abstract: Background: Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic small-

vessel vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys. It is the
most common form of systemic vasculitis in children.
Objective: The study aimed to familiarize physicians with the etiopathogenesis, clinical manifesta-
tions, evaluation, and management of children with Henoch-Schönlein purpura.
Methods: A PubMed search was conducted in January 2020 in Clinical Queries using the key terms
“Henoch-Schönlein purpura” OR “IgA vasculitis” OR “anaphylactoid purpura”. The search
strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies,
and reviews published within the past 10 years. Only papers published in the English literature
ARTICLE HISTORY
were included in this review. This paper is based on, but not limited to, the search results.
Current Pediatric Reviews

Received: February 20, 2020
Revised: April 14, 2020
Accepted: April 14, 2020
DOI:
10.2174/1573396316666200508104708
Keywords: Abdominal pain, arthralgia, arthritis, hematuria, IgA vasculitis, palpable purpura, proteinuria, vasculitis.
Results: Globally, the incidence of HSP is 10 to 20 cases per 100, 000 children per year. Approxi-
mately 90% of cases occur in children between 2 and 10 years of age, with a peak incidence at 4 to
7 years. The diagnosis should be based on the finding of palpable purpura in the presence of at least
one of the following criteria, namely, diffuse abdominal pain, arthritis or arthralgia, renal involve-
ment (hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition. Most
cases are self-limited. The average duration of the disease is 4 weeks. Long-term complications are
rare and include persistent hypertension and end-stage kidney disease. Therapy consists of general
and supportive measures as well as treatment of the sequelae of the vasculitis. Current evidence
does not support the universal treatment of HSP patients with corticosteroids. Oral corticosteroids
may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemor-
rhage.
Conclusion: Most cases of HSP have an excellent outcome, with renal involvement being the most
important prognostic factor in determining morbidity and mortality. Unfortunately, early steroid
treatment does not reduce the incidence and severity of nephropathy in children with HSP. In HSP
children who have severe nephritis or renal involvement with proteinuria of greater than 3 months,
an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker should be considered in
addition to corticosteroids to prevent and/or limit secondary glomerular injury.

1. INTRODUCTION
Henoch-Schönlein purpura (HSP) (also referred to as IgA
vasculitis or anaphylactoid purpura) is an IgA-mediated sys-
temic small-vessel vasculitis with a predilection for the skin,
kidneys, gastrointestinal tract, and joints [1-3]. HSP is the
most common systemic vasculitis in childhood [1, 4, 5]. The
hallmark is a pressure- or gravity-dependent nonthrombocy-
topenic purpuric or petechial rash [2, 3]. Abdominal pain,
*Address correspondence to this author at the Department of Pediatrics, The
University of Calgary, Alberta Children’s Hospital, #200, 233 - 16th Avenue
NW, Calgary, Alberta, Canada; Tel: (403) 230 3300; Fax: (403) 230-3322;
E-mail: aleung@ucalgary.ca
arthritis, and nephritis are other characteristic features [2, 3].
The condition was first described in 1,802 by William He-
berden who reported a 5-year-old boy with arthralgia, hema-
turia, abdominal pain, melena, and "bloody points" over his
legs [6]. In 1837, Johann Lakas Schönlein described the as-
sociation of purpura and arthralgia [7]. In 1874, his former
student Eduard Heinrich Henoch described purpura, ab-
dominal pain, and melena as a syndrome and in 1895, he
went on to recognize renal involvement in this syndrome [8,
9]. The syndrome now bears the names of both Henoch and
Schönlein.
A PubMed search was conducted in January 2020 in
Clinical Queries using the key terms “Henoch-Schönlein

1875-6336/20 $65.00+.00 © 2020 Bentham Science Publishers

266 Current Pediatric Reviews, 2020, Vol. 16, No. 4
purpura” OR “IgA vasculitis” OR “anaphylactoid purpura”.
The search strategy included meta-analyses, randomized
controlled trials, clinical trials, observational studies, and
reviews published within the past 10 years. Only papers
published in the English literature were included in this
review. This paper is based on, but not limited to, the
search results.
2. EPIDEMIOLOGY
HSP is the most common form of systemic vasculitis in
children [10]. Globally, the incidence is 10 to 20 cases per
100, 000 children per year [11-13]. The condition is more
common among Asians than Caucasians [11, 14]. Blacks are
the least frequently affected [5, 15]. Approximately 90% of
cases occur in children between 2 and 10 years of age, with a
peak incidence at 4 to 7 years [11, 12, 14, 16]. The occur-
rence of HSP at the extremes of age is rare [14, 17, 18]. HSP
is slightly more prevalent in boys than in girls [5, 13, 14].
HSP occurs throughout the year, but least frequently in the
summer [10, 19].
3. ETIOPATHOGENESIS
HSP is the result of a leukocytoclastic vasculitis mediat-
ed by an antigen-stimulated increase in the levels of IgA,
subsequent deposition of IgA-containing immune complexes
in the vasculature (usually capillaries) of involved organs,
and activation of complement pathways [10, 13, 20]. Activa-
tion of complement pathways leads to neutrophil accumula-
tion resulting in inflammation and vasculitis of small vessels
without a granulomatous reaction [10, 13, 20]. Elevated se-
rum levels of galactose-deficient IgA1 are seen in patients
with HSP [14]. The IgA-mediated vasculitis might result
from the interactions of multiple genes and environmental
factors, such as infections, medications, and vaccinations
[11].
There is a genetic predisposition to HSP [21]. Certain
human leukocyte antigens (HLA) such as HLA-DRB1*01,
HLA-DRB1*11, HLA-DQA1*0301, HLA-DRB1*014,
HLA-B*7, HLA-B*15, HLA-B*35, HLA-B*4102, HLA-
B*52, HLA-A*2, HLA-A*11, and HLA-A*26 that code for
inflammatory pathways within the blood vessels themselves
and those coding within the kidney may contribute to the
susceptibility [10, 11, 14, 21, 22]. Genetic variants in genes
that may influence how the vascular system responds to an
insult such as the angiotensinogen-converting enzyme
(ACE), endothelial nitric oxide synthetase (eNOS), trans-
forming growth factor (TGF), interleukin 18 (IL18), and
chemokine monocyte protein chemoattractant protein (MCP)
are other predisposing factors [14]. In addition, mutations in
the Mediterranean fever gene (MEFV), located on chromo-
some 16p13.3, which encodes pyrin, could be a contributing
factor [11, 22, 23]. HSP patients with homozygous exon 10
MEFV mutations tend to have the more severe disease than
those carrying other mutations [24].
Between 50 and 75% of patients with HSP have a history
of preceding upper respiratory tract infection [2, 3, 19].
Streptococcus pyogenes is the most common infecting or-
ganism [25-27]. Other reported precipitating infectious
agents include parvovirus B19, parainfluenza virus, adenovi-
rus, hepatitis A, B, C, and E viruses, Epstein-Barr virus, ro-
Leung et al.
tavirus, coxsackievirus, varicella-zoster virus, Mycoplasma
pneumoniae, Staphylococcus aureus, Haemophilus parain-
fluenzae, Yersinia, Shigella, Salmonella, Campylobacter, and
Helicobacter pylori [28-37]. Drugs such as clarithromycin,
erythromycin, ampicillin, vancomycin, angiotensin-
converting enzyme inhibitors, angiotensin II receptor antag-
onists, thiazides, nonsteroidal anti-inflammatory drugs, qui-
nine, quinidine, losartan, adalimumab, rituximab, secuki-
numab, infliximab, trifluridine, tipiracil, rivaroxaban, and
cytarabine have also been implicated [21, 38-44]. Other pre-
cipitating factors that have been implicated include insect
bites, horse serum, exposure to cold, trampoline jumping,
and α-1 antitrypsin deficiency [34, 45-47]. Vaccinations
(influenza, hepatitis A, hepatitis B, pneumococcus, cholera,
typhoid, mumps-measles-rubella, and yellow fever) have
also been implicated [48], but are not a major etiologic fac-
tor as the absolute risk is low [49, 50]. The Italian Multi-
center Study Group for Drug and Vaccine Safety in Chil-
dren found that among vaccines, only the mumps-measles-
rubella vaccine showed an increased risk of HSP (odds ra-
tio: 3.4; 95% confidence interval: 1.2 to 10.0) [49]. A Eu-
ropean, multicenter (5 pediatric departments) case-
crossover study involving 167 children showed that the
odds ratio for HSP occurring within 3 months of vaccina-
tion was 1.6 (95% confidence interval: 0.8 to 3.0), and thus
was not significant [51].
4. HISTOPATHOLOGY
In patients with HSP, the skin biopsy reveals classical
leukocytoclastic vasculitis most prominent in superficial
postcapillary venules with a predominance of IgA deposition
[5, 52]. Specific histopathologic findings in renal biopsies
include diffuse hypercellularity; mononuclear cell infiltra-
tion; mesangial proliferation; focal and segmental prolifera-
tion; minimal change to severe crescentic glomerulonephri-
tis; segmental sclerosis fibrosis; mesangial, subendothelial,
and subepithelial deposits; IgA deposits in the mesangium;
and diffuse glomerular deposits of IgA, IgG, IgM, C3, fibrin,
and properdin [52].
5. CLINICAL MANIFESTATIONS
The typical prodrome of HSP includes anorexia, head-
ache, and fever [17]. The clinical features of HSP may de-
velop days to weeks later [5]. HSP is characterized by the
classic tetrad of palpable purpura without thrombocytopenia
or coagulopathy, arthralgia/arthritis, colicky abdominal
pain/gastrointestinal bleeding, and nephropathy [5, 12, 53].
The clinical features of HSP are diverse according to in-
volved organs and may be atypical at the extremes of age
[10]. The severity tends to be milder in children under 2
years of age and worse in adults [10, 16, 25].
5.1. Dermatologic Manifestations
A purpuric or petechial rash in pressure- or gravity-
dependent distribution is present in almost all patients with
HSP (Fig. 1) [2, 25, 46]. The characteristic distribution of the
rash is over the ventral aspect of the feet, ankles, and lower
legs in older children and buttocks, back, and upper thighs in
young children [4, 25].
Henoch-Schönlein Purpura Current Pediatric Reviews, 2020, Vol. 16, No. 4 267

Fig. (1). HSP rash symmetrically distributed over the ventral

aspect of the feet, ankles, and lower legs. (A higher resolution /
colour version of this figure is available in the electronic copy of
the article).

The face, trunk, palms, soles, and mucous membranes are

usually spared, except in infants in whom facial involvement
is not uncommon [15, 45, 54]. The eruption commonly be- Fig. (3). HSP rash coalescing to form large ecchymotic areas. (A
gins as erythematous macules that evolve into petechiae or higher resolution / colour version of this figure is available in the
purpura in 12 to 24 hours [2]. The petechial or purpuric rash electronic copy of the article).
is often symmetrically distributed and is the presenting sign
in 50 to 75% of patients [2, 10, 21, 25]. The HSP rash is typ-
ically palpable and does not blanch (Fig. 2) [10, 21, 25]. The
rash may coalesce to form large plaques that resemble ec-
chymoses (Fig. 3) [25]. Typically, the rash is not itchy or
painful [25]. Koebner phenomenon has rarely been reported
[55-57]. The eruption in children appears in crops over an
average of three weeks and is characterized by its polymor-
phism in contrast to the eruption seen in adults, which is of-
ten monomorphic [21, 25]. Typically, the color of purpura
progresses from red to purple, and then to rust-colored or
brown as the rash fades [13]. These color changes occur over
approximately 10 days [13]. There is no associated thrombo-
cytopenia or coagulopathy. Some patients have target-like
lesions, with each lesion consisting of a central punctate
hemorrhage surrounded by circumferential regions of pallor
and then hemorrhage [3, 25]. Blisters and bullous lesions are Fig. (4). Bullous lesions in a child with HSP. (A higher resolution /
uncommon (Fig. 4) [58-62]. Hemorrhagic bullae (Fig. 5) and colour version of this figure is available in the electronic copy of
skin necrosis leading to ulcers are rare [62-65]. the article).
Subcutaneous edema is reported in 35 to 70% of patients
[3]. The edema is nonpitting and usually noted on the dorsal
surface of the hands and feet but may also occur on the
scalp, ears, periorbital area, and abdominal wall [15, 66].
Some authors proposed to include subcutaneous edema as a
diagnostic criterion of HSP [67]. Rarely, subcutaneous nod-
ules have been reported [68].

Fig. (5). Hemorrhagic bullous lesions in a child with HSP. (A high-

Fig. (2). Nonblanchable HSP rash. (A higher resolution / colour er resolution / colour version of this figure is available in the elec-
version of this figure is available in the electronic copy of the arti- tronic copy of the article).
cle).
268 Current Pediatric Reviews, 2020, Vol. 16, No. 4
5.2. Musculoskeletal Manifestations
Arthralgia or arthritis develops in 65 to 85% of affected
patients and is the presenting symptom or sign in 17 to 25%
of cases [2, 3]. In one study of 186 children (97 boys and 89
girls), female sex, soft tissue swelling, and low mean platelet
volume levels were risk factors for joint involvement [1].
The frequency of joint involvement is inversely correlated
with the patient's age; younger children tend to be more fre-
quently affected [21]. Large joints in the lower extremities
(e.g., the knees and ankles), are the most commonly in-
volved, while smaller joints (e.g., the fingers) and spine are
less commonly affected [10, 20, 62]. The joint involvement
is usually symmetrical in distribution and non-migratory [17,
25, 34, 69]. Typically, only a few joints (less than four) are
affected [14]. Periarticular pain and swelling are common,
while erythema, warmth and joint effusion are rare [70]. Af-
fected patients may have a limitation of movement of the
affected joints [12, 54]. Arthralgia or arthritis is usually tran-
sient and does not lead to permanent disability [14, 71]. My-
ositis is rare but has been reported [72].
5.3. Gastrointestinal Manifestations
Gastrointestinal involvement occurs in 50 to 75% of
children with HSP and may precede the onset of purpura in
10 to 20% of patients [10, 62]. Abdominal pain is the most
common symptom, which may be dull aching or, more
commonly, colicky [2, 62, 73, 74]. The pain is usually dif-
fuse but may be localized to the periumbilical or epigastric
area [15, 21]. Nausea and vomiting are common [25]. Hema-
temesis and melena may occur [75]. Gross blood in stools is
more commonly seen if the vasculitis involves the colon
rather than the small bowel [76]. The gastrointestinal symp-
toms can be attributed to mesenteric vasculitis with resultant
visceral or peritoneal purpura and extravasation of blood and
interstitial fluid into the bowel wall and intestinal lumen as
well as infarction of the bowel [21, 34]. Intussusception is
the most common surgical complication, with an overall
incidence of approximately 3.5% [2, 13, 75, 77, 78 ]. The
intussusception is confined to the ileum in approximately
60% of cases; this is in contrast to idiopathic intussusception,
which is typically ileocolic [5]. Protein-losing enteropathy
occurs in approximately 3% of cases [79]. Other gastrointes-
tinal complications, though rare, include intestinal perfora-
tion, hemorrhagic ascites, acute acalculous cholecystitis, and
acute pancreatitis [80-87].
5.4. Renal Manifestations
Renal manifestations develop in 40 to 50% of patients
[12, 13, 25]. Approximately 97% of these patients have renal
manifestations within 3 months of disease onset [10, 12, 13,
25, 54]. Renal involvement varies widely, with glomerulo-
nephritis being the most common [54]. Hematuria (micro-
scopic or, less commonly, macroscopic) is the most common
renal manifestation and is usually asymptomatic [12]. Active
screening for hematuria is therefore necessary. Other find-
ings include proteinuria, nephrotic syndrome, and acute ne-
phritis with hypertension [3, 13, 25, 74, 88]. Rarely, renal
colic has been reported in patients with HSP [74]. Persistent
hematuria and proteinuria predict the development of end-
stage kidney disease [25, 34].
Leung et al.
Risk factors for renal involvement include severe ab-
dominal pain with gastrointestinal hemorrhage, age more
than 5 years at the onset, persistent purpura for more than a
month, scrotal involvement, obesity, elevated serum IgA,
elevated C-reactive protein (CRP) levels, and decreased se-
rum C3 levels [1, 89-92]. Nephropathy is generally mild in
young children but tends to be more severe in older children
(> 6 years of age) and in adults [3, 25, 93]. In one study of 2,
731 children with HSP, renal involvement occurred in 844
children and severe kidney disease occurred in 104 children
[94]. The authors found that being more than 6 years of age
at onset, interval of more than 8 days between the onset of
symptoms and diagnosis, angioedema, central nervous in-
volvement, and recurrence were highly associated with se-
vere kidney disease [94]. A 2016 meta-analysis of 13 studies
involving 2,398 children with HSP showed that the follow-
ing risk factors were associated with renal involvement: male
gender; age > 10 years at onset; severe abdominal symptoms
(including abdominal pain, gastrointestinal bleeding, bowel
angina); arthralgia/arthritis; persistent purpura or relapse;
white blood cell count > 15 x 109/L; platelet count > 500 x
109/L; elevated serum antistreptolysin O (ASO) titer; and
low serum C3 [95].
5.5. Genital Manifestations
Orchitis is reported in 10 to 20% of boys with HSP [10].
Clinical findings, which include pain, tenderness, and swell-
ing of the involved testicle or scrotum, can mimic testicular
torsion [25, 96]. Scrotal involvement occurs in approximate-
ly 20% of boys with HSP [97]. Penile edema, priapism, epi-
didymitis, hematoma of the spermatic cord, testicular infarc-
tion, thrombosis of the spermatic veins, scrotal hematoma,
and labial edema have also been reported [98-105].
5.6. Neurologic Manifestations
Patients with HSP may develop cerebral vasculitis [106].
Headache, dizziness, and behavioral changes (e.g., irritabil-
ity, hyperactivity, apathy, mood liability) develop in up to
30% of patients [2, 3, 16]. Seizures, visual abnormalities,
verbal disability, confusion, ataxia, and focal neurologic def-
icits are reported in only 2 to 8% of patients [3, 107]. Rarely,
peripheral neuropathy, facial nerve palsy, myelopathy, intra-
cranial hemorrhage, subarachnoid hemorrhage, subdural
hematoma, cerebral venous thrombosis, Guillain-Barré syn-
drome, encephalopathy, and posterior reversible encephalo-
pathy syndrome have been reported [106-114].
5.7. Pulmonary Manifestations
Pulmonary complications include diffuse alveolar hemor-
rhage, interstitial pneumonia, interstitial fibrosis, and pleural
effusion [16, 115-117]. In general, pulmonary complications
are rare [16].
5.8. Cardiac Manifestations
Myocarditis and myocardial necrosis/infarction have
rarely been reported in patients with HSP, which can be at-
tributed to the vasculitis involving the myocardium [118-
120].
Henoch-Schönlein Purpura Current Pediatric Reviews, 2020, Vol. 16, No. 4 269

5.9. Ophthalmic Manifestations 6. DIFFERENTIAL DIAGNOSIS

Engorgement of episcleral vessels, uveitis, and keratitis As HSP can affect a lot of organs, the differential diagno-
have rarely been reported in patients with HSP [121, 122]. ses are extensive. The differential diagnoses of some com-
mon manifestations of HSP are listed in Table 1.

Table 1. Differential diagnoses of some common manifestations of HSP.

Manifestations Differential Diagnoses

Purpura Immune thrombocytopenic purpura [123]

Thrombocytopenia secondary to drugs, viral infections, or bone marrow infiltration [123]
Drug reaction [124]
Child abuse [123]
Kasabach-Meritt syndrome [123]
Cryoglobulinemia [93]
Hypersensitivity vasculitis [93]
Hemolytic uremic syndrome [125]
Leukemia [126]
Disseminated intravascular coagulopathy [123]
Deficiency of coagulation factors [123]
Acute hemorrhagic edema of infancy [126]
Meningococcemia
Disseminated gonococcemia [124]
Rickettsial diseases
Rocky Mountain spotted fever [124]
Hypersensitivity vasculitis [126]
Systemic lupus erythematosus [126]
Wegener granulomatosis [93, 127]
Churg-Strauss syndrome [126]
Gardner-Diamond syndrome [123]
Arthralgia/arthritis Toxic synovitis [126]
Juvenile rheumatoid arthritis [126]
Septic arthritis [126]
Rheumatic fever [126]
Systemic lupus erythematosus [126]
Serum sickness [93]
Psoriasis
Trauma to the joint

Acute abdominal pain Abdominal trauma [128]

Gastroenteritis [128]
Constipation [128]
Appendicitis [128]
Mesenteric lymphadenitis [128]
Intestinal obstruction [128]
Inflammatory bowel disease [128]
Pelvic inflammatory disease [128]
Threatened abortion [128]
Ectopic pregnancy [128]
Ovarian/testicular torsion [128]
Sickle cell crisis [128]
Pancreatitis [128]
(Table 1) Contd…
270 Current Pediatric Reviews, 2020, Vol. 16, No. 4
Manifestations
Hematuria
Benign familial hematuria (thin basement membrane disease)
Malignancies (e.g., Wilms tumor, carcinoma of the bladder)
7. LABORATORY EVALUATION
There are no laboratory studies that definitively confirm
the diagnosis of HSP, although an elevated serum IgA level
is suggestive [3, 132]. Approximately 50 to 70% of patients
with HSP have elevated serum IgA levels, but serum IgA
levels do not correlate with disease severity [5, 15].
Renal involvement should be investigated with urinalysis
and estimated glomerular filtration rate [133]. When renal
involvement is present, urinalysis may reveal dysmorphic
red blood cells, white blood cells, cellular casts, and/or pro-
tein [134]. Elevated levels of serum creatinine or blood urea
nitrogen suggest renal insufficiency associated with HSP
nephritis. A decrease in the levels of serum total protein and
albumin associated with proteinuria greater than 1 g/m2/day
suggests nephrotic syndrome [88, 135-137].
Laboratory studies such as complete blood cell count and
coagulation studies might be necessary to rule out other
causes of vasculitis and potential causes of similar symp-
toms. The hemoglobin is usually normal unless severe gas-
trointestinal, renal, or pulmonary bleeding occurs, in which
case normochromic anemia may be noted [54, 134]. Leuko-
cytosis is common. A normal platelet count differentiates
HSP from thrombocytopenic purpura and normal results of
coagulation studies (prothrombin time, partial thromboplas-
tin time, bleeding time) distinguish HSP from primary hem-
orrhagic disorders. In a study of 214 children with HSP, ele-
vated neutrophil count and elevated neutrophil to lympho-
cyte ratio were associated with gastrointestinal involvement
and nephritis [138]. On the other hand, lower platelet count
and higher mean platelet volume were associated with HSP
recurrence [138]. In a study of 141 patients with HSP, neu-
trophil to lymphocyte ratio was significantly higher (p =
0.001) and platelet to lymphocyte ratio was significantly
lower (p = 0.032) in patients with gastrointestinal bleeding
Leung et al.
Differential Diagnoses
Post-streptococcal glomerulonephritis [129]
IgA nephropathy
Urinary tract infection [130, 131]
Urinary tract trauma
Nutcracker syndrome
Interstitial nephritis
Hypercalciuria
Renal/ureteric stone
Hemolytic uremic syndrome [125]
Acute tubular necrosis
Polycystic kidney
Renal tuberculosis
Bleeding diathesis
Sickle cell disease
Hemorrhagic cystitis
Alport syndrome [132]
than in those without gastrointestinal bleeding [139]. These
findings were confirmed by another similar study [140]. In a
study of 112 patients with HSP, leukocyte count, neutrophil
count, monocyte count, neutrophil to lymphocyte ratio, and
CRP were significantly higher in HSP patients with internal
organ involvement than in those without internal organ in-
volvement [141]. CRP and/or erythrocyte sedimentation rate
(ESR) may be included as screening tests, but they are nei-
ther specific nor sensitive for HSP [76]. D-dimer and fibrin
degradation products may be elevated, reflecting the disease
activity of HSP [52, 142]. Normal serum antinuclear anti-
body, double-stranded DNA, and C3 and C4 levels help to
distinguish HSP from other causes of vasculitis, such as sys-
temic lupus erythematosus [15]. Anti-neutrophil cytoplasmic
antibody (ANCA) titers are normal in HSP but elevated in
Wegener granulomatosis [127].
Further laboratory tests should be ordered, only when
indicated by the history and physical examination. Stool
guaiac test to detect occult blood may be considered for pa-
tients with gastrointestinal symptomatology. Abdominal ul-
trasonography should be performed in patients with severe
abdominal pain to exclude intestinal intussusception [133]. A
skin biopsy is usually not necessary but should be considered
if the clinical manifestations are incomplete or atypical (e.g.,
no or atypical rash) [5, 133]. A renal biopsy is indicated for
HSP patients with severe proteinuria (>250 mg/mmol for at
least 4 weeks; although the shorter duration of severe pro-
teinuria is also a relative indication), persistent moderate
(100 to 250 mg/mmol) proteinuria or impaired glomerular
filtration rate [133].
8. DIAGNOSIS
According to the European League Against Rheumatism
(EULAR), the Paediatric Rheumatology International Trials
Henoch-Schönlein Purpura
Organisation (PRINTO), and the Paediatric Rheumatology
European Society (PRES), the diagnosis of HSP should be
based on the finding of palpable purpura (mandatory criteri-
on) in the presence of at least one of the four following crite-
ria, namely, acute onset of diffuse abdominal pain, arthritis
or arthralgia, renal involvement (hematuria and/or pro-
teinuria), and a biopsy showing predominant IgA deposition
[143]. The above criteria have a sensitivity of 93% and a
specificity of 89% [12, 54]. Clinically, children presenting
with nonthrombocytopenic palpable purpura with multisys-
tem (gastrointestinal tract, kidneys, joints) involvement may
be diagnosed to have HSP until it is proven otherwise.
9. PROGNOSIS
Most cases of HSP have an excellent outcome, with renal
involvement being the most important prognostic factor in
determining morbidity and mortality [33, 54, 131, 144]. The
average duration of the disease is 4 weeks, except for those
with persistent proteinuria or hematuria [10, 13]. Recurrenc-
es are reported in up to one-third of patients, especially
among patients with renal involvement or received steroid
treatment for more than 10 days [10, 54, 145]. Recurrences
usually occur within one year of presentation but may pre-
sent several years later, with or without any relation to an
upper respiratory infection [15, 54]. Long-term complica-
tions are rare and include persistent hypertension and end-
stage kidney disease [10]. A 2019 meta-analysis of 9 case-
control studies involving 969 patients with HSP nephritis
showed that older age at onset (weighted mean difference
[WMD]: 1.77; 95% confidence interval [CI]: 0.35 to 3.18; p
= 0.014), lower glomerular filtration rate (WMD: -23.93;
95% CI: -33.78 to -14.09; p < 0.0001), initial renal manifes-
tations with nephrotic syndrome (odds ratio [OR]: 1.74; 95%
CI: 1.12 to 2.7; p = 0.013), with nephritic-nephrotic syn-
drome (OR: 4.55; 95% CI: 2.89 to 7.15; p < 0.0001), and
renal biopsy with crescentic nephritis (International Study of
Kidney Disease in Children grades III to IV) (OR: 3.85; 95%
CI: 2.37 to 6.28, p < 0.0001) were significant risk factors
with poor outcomes [146]. On the other hand, initial clinical
features with hematuria (OR: 0.33; 95% CI: 0.16 to 0.69; p =
0.003) and mild proteinuria with or without hematuria (OR:
0.46; 95% CI: 0.28 to 0.75; p < 0.0001) were associated with
good outcomes [146].
10. MANAGEMENT
Most cases of HSP in children are self-limiting and do
not require any specific treatment apart from general and
supportive measures as well as treatment of the sequelae of
the vasculitis. Quiet activities (e.g. leg rest), optimal nutri-
tion, and adequate hydration are helpful. Acetaminophen and
nonsteroidal anti-inflammatory drugs such as ibuprofen and
naproxen can be used to relieve joint and soft tissue discom-
fort/pain. Nonsteroidal anti-inflammatory drugs should be
avoided in patients with active gastrointestinal hemorrhage
or renal insufficiency because of their effects on platelets and
renal perfusion, respectively [126]. The use of aspirin is con-
traindicated in children because of the possibility of Reye
syndrome.
Although the majority of patients with HSP develop renal
involvement within 3 months of disease onset, some patients
Current Pediatric Reviews, 2020, Vol. 16, No. 4 271
may not have renal involvement until later on [34]. As such,
patients with HSP should be followed with weekly or bi-
weekly urinalysis and blood pressure for the first few months
after presentation and less frequently thereafter [25]. Once
the disease is subsiding, urinalysis and blood pressure moni-
toring should be scheduled every month initially and then
every other month for at least one year [126]. Children with
hematuria with or without proteinuria for more than 6
months should have periodic monitoring of serum creatinine
[93]. In children with renal involvement, a low-salt diet
helps minimize the possibility of hypertension.
The use of corticosteroids in patients with HSP is contro-
versial [10, 20]. Current evidence does not support universal
treatment of HSP patients with corticosteroids [69, 147]. On
the other hand, some children may require corticosteroids for
selective indications [133]. It has been shown that cortico-
steroids may shorten the duration of abdominal pain and may
prevent major complications such as gastrointestinal hemor-
rhage and intussusception [20, 69]. As such, oral corticoster-
oids may be considered for HSP patients with severe gastro-
intestinal involvement, such as severe abdominal pain and
gastrointestinal hemorrhage [21, 148, 149]. In addition, oral
steroids may be considered for HSP patients with intrap-
ulmonary hemorrhage, persistent nephrotic syndrome, severe
scrotal edema, orchitis, and cerebral vasculitis [133]. How-
ever, the use of systemic steroids must be balanced against
the potential side effects of steroids [150]. According to the
European SHARE (Single Hub and Access point for paediat-
ric Rheumatology in Europe), the use of systemic steroids in
conjunction with cytotoxic immunosuppressants (e.g., aza-
thioprine, mycophenolate mofetil, cyclophosphamide)
should be considered for those with organ- or life-
threatening involvement [133].
Generally, early administration of oral corticosteroids in
patients with HSP does not prevent or slow the progression
of renal disease [26, 69, 93, 151]. A large scale (n = 352),
randomized, double-blind, placebo-controlled trial found that
early corticosteroid treatment does not reduce the incidence
and severity of nephropathy in patients with HSP [152, 153].
A 2015 Cochrane systematic review of 13 studies (n = 1403)
found that there is no evidence to support the use of cortico-
steroids to prevent persistent kidney disease in children with
HSP [154]. However, in HSP children who have severe ne-
phritis or renal involvement with proteinuria of greater than
3 months, the SHARE recommends that an angiotensin-
converting enzyme inhibitor or angiotensin receptor blocker
should be considered in addition to corticosteroids to prevent
and/or limit secondary glomerular injury [134]. There may
be a role for immunosuppression in HSP patients with per-
sistent /increasing proteinuria [70, 155, 156]. In a study of 84
Chinese patients diagnosed with HSP nephritis by renal bi-
opsy who had proteinuria, patients who received combined
steroid and immunosuppressant therapy were more likely to
recover than those treated with steroid alone and had pro-
teinuria <3 g/24 hours (p = 0.02) or 1 g/24 hours (p = 0.03)
[157]. In addition, combined steroid and immunosuppressant
therapy was significantly associated with remission of HSP
nephritis [157].
Hospitalization should be considered for HSP patients
with severe gastrointestinal bleeding, severe abdominal pain,
272 Current Pediatric Reviews, 2020, Vol. 16, No. 4
or renal failure. Surgical intervention is indicated for patients
with intestinal perforation or mesenteric infarction. Indica-
tions for a renal biopsy include acute renal impair-
ment/nephritic syndrome at presentation, persistent proteinu-
ria, and rapidly deteriorating renal function [2, 141].
CONCLUSION
HSP is the most common systemic vasculitis in child-
hood. The diagnosis can usually be made based on the find-
ing of palpable purpura in the presence of at least one of the
following criteria, namely, diffuse abdominal pain, arthritis
or arthralgia, renal involvement (hematuria and/or proteinu-
ria), and a biopsy showing predominant IgA deposition.
Most cases of HSP are benign and self-limited. Recurrences
are reported in up to one-third of patients, especially among
patients with renal involvement. Long-term complications
are rare and include persistent hypertension and end-stage
kidney disease.
CONSENT FOR PUBLICATION
Not applicable.
FUNDING
None.
CONFLICT OF INTEREST
Professor Alexander K.C. Leung is the section editor of
Current Pediatric Reviews. This paper was sent out for inde-
pendent peer review by the editor-in-chief. Professor
Alexander K.C. Leung, Dr. Benjamin Barankin, and Dr. Kin
Fon Leong confirm that this article has no conflicts of inter-
est.
ACKNOWLEDGEMENTS
Professor Alexander K.C. Leung is the principal author.
Dr. Benjamin Barankin, and Dr. Kin Fon Leong are
coauthors. All the authors contributed to the drafting and
revision of the manuscript and approved the final version
submitted for publication.
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