19
Članek prispel / Received
17. 8. 2020
Članek sprejet / Accepted
14. 7. 2021
Abstract
Purpose: Henoch–Schönlein purpu-
ra (HSP) is the most common type of 
vasculitis in childhood and has a good 
prognosis. The aim of our study was to 
compare the clinical characteristics of 
children with HSP diagnosed in north–
east Slovenia with existing data from 
other countries.
Methods: We retrospectively reviewed 
the data of patients with HSP in our 
department between 2005 and 2018, 
and assessed their clinical characteri-
stics, laboratory results, and treatment 
at presentation. We also analysed how 
many patients relapsed, the causes of 
this, and the treatment administered. 
These parameters were compared with 
previously published research.
Results: During the study period, 123 
patients with HSP were treated in our 
Izvleček
Namen: Purpura Henoch–Schönlein 
je najpogostejši vaskulitis v otroški dobi 
z dobro prognozo. Z raziskavo primerja-
mo naše izkušnje z boleznijo z epidemi-
ološkega in kliničnega stališča z raziska-
vami v svetovnem merilu.
Metode: Retrospektivno smo pregledali 
podatke o bolnikih s purpuro Henoch–
Schönlein v obdobju med leti 2005 in 
2018. Povzeli smo njihove klinične 
značilnosti, laboratorijske vrednosti ter 
zdravljenje ob prvi prezentaciji. Nadalje 
smo preverili, koliko, zakaj in kako smo 
zdravili bolnike, ki so se zaradi težav 
v našo obravnavo večkrat vračali. Vse 
navedene značilnosti smo primerjali z že 
objavljenimi raziskavami.
Rezultati: V času raziskave smo 
obravnavali 123 bolnikov s purpuro 
Henoch–Schönlein. Mediana starost 
Ključne besede: 
purpura Henoch–Schönlein, 
epidemiologija, klinična slika, 
obravnava, otroci.
Key words: 
Henoch–Schönlein purpura, 
epidemiology, clinical picture, 
management, children.
Henoch–Schönlein purpura – obravnava in 
zdravljenje slovenskih pediatričnih bolnikov 
severovzhodnega dela Slovenije
Henoch–Schönlein purpura: the management 
and treatment of paediatric patients 
in north–east Slovenia
Avtor / Author Mirjam Močnik1, Nataša Marčun Varda1,2
Ustanova / Institute 1Univerzitetni klinični center Maribor, Klinika za pediatrijo, Enota za pediatrično nefrologijo 
in arterijsko hipertenzijo, Maribor, Slovenija; 2Univerza v Mariboru, Medicinska fakulteta, 
Katedra za pediatrijo, Maribor, Slovenija;
1University Medical Centre Maribor, Department of Paediatrics, Paediatric Nephrology and 
Arterial Hypertension Unit, Maribor, Slovenia; 2University of Maribor, Faculty of Medicine, 
Department of Paediatrics, Maribor, Slovenia;
Klinična študija / Clinical study
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INTRODUCTION
Henoch–Schönlein Purpura (HSP) is a systemic 
vasculitis with small vessel inflammation and 
immunoglobulin A (IgA) deposition (1). It is the 
most frequent type of vasculitis in childhood, with an 
incidence of 10−30/100,000 children worldwide (2,3). 
In most cases, HSP is preceded by an upper respiratory 
tract infection, which is a possible trigger. It is assumed 
that HSP is the result of an irregular immune response 
to a pathogen (1,3). 
The classic signs and symptoms of HSP include 
purpura, arthritis or arthralgia, abdominal pain, 
and kidney damage (4,5). Purpura is present in all 
children with HSP. Abdominal pain develops in two–
thirds of patients (2,6). Renal involvement occurs in 
20−55% of patients with haematuria, proteinuria, 
and hypertension. Other less common manifestations 
include cerebral vasculitis, scrotal bleeding, and 
interstitial pulmonary haemorrhage (2).
The diagnosis is made clinically by the presence of 
purpura on the lower limbs and at least one of the 
following: abdominal pain, arthritis or arthralgia, 
histological confirmation of leucocytoclastic vasculitis, 
or proliferative glomerulonephritis with predominant 
deposits of IgA immunoglobulins and kidney 
vzorca bolnikov je bila 6,0 let (IQR 4, 
9) z enakomerno porazdelitvijo med spo-
loma. Pri vseh je bila ob prezentaciji pri-
sotna kožna purpura, pri 52,8 % so bile 
prisotne še otekline sklepov, pri 19,5 % 
bolečine v trebuhu in v do 10 % znaki 
ledvične prizadetosti. Najpogosteje smo 
bolnike zdravili podporno. Pri 61,8 % 
smo vključili nesteroidne antirevmatike, 
pri 13 % pa kortikosteroide. Povratni-
kov je bilo 15,4 %, najpogosteje zaradi 
ponovnih zagonov kožne purpure ter led-
vičnih manifestacij.
Zaklju~ek: V primerjavi z raziska-
vami iz tujine ugotavljamo podoben 
vrstni red prizadetosti posameznih 
organskih sistemov, vendar v nižjem 
odstotku. V primerjavi z ostalimi razis-
kavami se pogosteje odločamo samo za 
podporno terapijo; kortikosteroide up-
orabljamo redkeje. V primerjavi z osta-
limi raziskavami beležimo tudi manjši 
delež povratnikov.
department. The median patient age 
was 6.0 years (interquartile range: 4–9 
years) with a uniform distribution bet-
ween the sexes. Along with purpura, 
52.8% presented with arthropathy, 
19.5% with abdominal pain, and up to 
10% with renal involvement. Patients 
received supportive therapy the most 
frequently. Non–steroidal anti–inflam-
matory drugs were prescribed to 61.8% 
of patients and corticosteroids to 13%. 
Relapse occurred in 15.4% of patients, 
most presenting with skin purpura and 
kidney manifestations.
Conclusion: The basic demographic 
data of our patients were comparable to 
those of other similar published studies. 
The order of frequency of individual organ 
system involvement was also similar, but 
the percentages were lower. Purpura–ac-
companying manifestations were reported 
less often in our study than in previous stu-
dies. Compared to other studies, suppor-
tive therapy alone was prescribed more 
frequently to our patients, and corticoste-
roids were rarely administered. Compared 
to other studies, a smaller proportion of 
patients with relapse was recorded.
Naslov za dopisovanje /  
Correspondence
Asist. Mirjam Močnik, dr. med., 
Univerzitetni klinični center Maribor, 
Klinika za pediatrijo, Enota za 
pediatrično nefrologijo in arterijsko 
hipertenzijo, Ljubljanska 5, 2000 
Maribor, Slovenija
Telefon +386 40323726
E–pošta: mirjammocnik91@gmail.com, 
mirjam.mocnik@ukc–mb.si
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involvement (2,4).
The treatment of HSP is supportive, and 
immunosuppression is rarely needed. Good hydration, 
rest, pain management, and monitoring are essential. 
Patients with arthritis respond well to non–steroidal 
anti–inflammatory drugs (NSAID). The use of 
corticosteroids provides a faster relief of abdominal 
pain and reduces the risk of intussusception (6). There 
is no evidence to suggest that the use of corticosteroids 
prevents the onset of renal disease. Immunosuppressive 
therapy is required in patients with HSP nephritis and 
severe renal involvement. Renal biopsy is recommended 
before commencing immunosuppressant therapy. 
Renin–angiotensin–aldosterone inhibitors are 
administered to patients with persistent proteinuria 
because they have been shown to reduce proteinuria 
and renal fibrosis (2,7).
HSP disease prognosis is generally favourable with 
spontaneous cessation of symptoms and signs (2). The 
long–term prognosis depends primarily on kidney 
damage (8). HSP recurs in approximately a third of 
patients (9).
The aim of our retrospective study was to analyse the 
clinical picture, course of disease, and treatment of 
patients with HSP in our department.
METHODS 
Paediatric patients treated for HSP in the Department of 
Paediatrics, University Medical Centre Maribor, north–
east Slovenia between 2005 and 2018 were included 
in the study.  This department is the second largest 
paediatric department in the country and the only other 
tertiary centre besides the one in Ljubljana. Data were 
collected from the hospital’s database. Patients were 
mostly managed by rheumatologists except in cases of 
severe abdominal pain. Patients who relapsed were seen 
by the same specialists working predominantly in the 
subspecialist field, e.g. rheumatologists, nephrologists, 
and gastroenterologists. The HSP diagnosis was based 
on clinical criteria. 
We retrospectively analysed the available clinical data on 
the clinical picture (purpura, abdominal pain, arthritis 
or arthralgia, renal involvement, upper respiratory tract 
infection, and systolic and diastolic blood pressure) 
and laboratory characteristics (streptococcal test; 
antistreptolysin titre; occult blood in faeces; urinalysis, 
blood count; sedimentation rate; C–reactive protein, 
electrolytes [sodium, potassium, and chloride]; kidney 
function tests and liver damage markers; albumin; 
complement C3 and C4 fractions; immunoglobulins 
IgA, IgG, and IgM; antinuclear antibody, antibodies 
against cytoplasmic antigens of neutrophil granulocytes, 
and anti–DNA antibodies). The prescribed therapy was 
also analysed (supportive, NSAIDs, corticosteroids, and 
renin–angiotensin–aldosterone inhibitors). Finally, we 
assessed the number of patients who returned to our 
department with at least two symptom recurrences. 
Statistical analysis of the data was performed using IBM 
SPSS Statistics Standard version 22 (IBM Corporation, 
Armonk, NY, USA) with basic statistical methods, e.g., 
the frequency of descriptive clinical characteristics, 
and the median with interquartile range (IQR) 
and minimum and maximum values for numerical 
parameters. Additionally, differences between boys and 
girls were evaluated using crosstabs, and the Pearson 
chi–squared text for descriptive parameters and the 
independent samples t–test to compare the numerical 
data. A p–value of <0.05 was considered statistically 
significant.
RESULTS 
The data of 123 children (57 boys (46.3%) and 66 girls 
(53.7%), p=0.417) diagnosed with HSP were reviewed. 
Clinical and laboratory characteristics at disease onset 
are described in Tables 1 and 2. The median age of 
the children was 6.0 (IQR: 4–9). Along with purpura, 
more than half of the patients had signs of an upper 
respiratory tract infection, and a quarter had a positive 
streptococcal test. In more than half of cases, joint 
swelling occurred, while 24 patients had abdominal 
pain, 20 of whom had occult blood in the stools. Signs of 
renal involvement with haematuria or proteinuria were 
detected in up to 10% of patients, with normal renal 
function and blood pressure recorded in most cases. 
No significant immunological deviations were observed 
except for occasionally elevated IgA immunoglobulin 
values according to published normal values for age 
(10). Some of the results, e.g., electrolytes and liver 
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damage markers, are not shown due to their lack of 
relevance for this study. Blood pressure, presented in 
Table 2, was measured on admission. Patients with 
renal involvement also underwent ambulatory blood 
pressure measurement, which was generally within the 
normal limits. 
At first presentation, rest and hydration were 
recommended to all patients, NSAIDs were prescribed 
in 76 children (61.8%), corticosteroids, mostly 
prednisone, were administered intravenously at a dose 
of 1–2 mg/kg or in pulses of 30 mg/kg, in 16 children 
(13%), and angiotensin–converting enzyme (ACE) 
inhibitors were prescribed for three children (2.4%). A 
total of 19 children (15.4%) were treated more than 
once, 16 (84.2%) with recurrent purpura, eight (42.1%) 
with kidney involvement (proteinuria, haematuria, 
elevated blood pressure, and mildly elevated 
creatinine), five with frequent joint swelling, and one 
with symptoms of gastrointestinal tract involvement. In 
68% of relapsing patients, two or more organ systems 
were involved. NSAIDs were prescribed to 61.8% of 
Table 1. Clinical characteristics of the patients.
Clinical manifestation YESN (%)
NO
N (%)
NO DATA
N (%)
Purpura 123 (100) 0 0
Signs of upper respiratory tract infection 67 (54.4) 33 (26.8) 23 (18.7)
Positive streptococcal test 30 (24.3) 43 (35) 50 (40.7)
Joint swelling 65 (52.8) 57 (46.3) 1 (0.8)
Abdominal pain 24 (19.5) 98 (79.7) 1 (0.8)
Laboratory data YESN (%) 
NO
N (%)
NO DATA 
N (%)
Faecal occult blood 21 (17.1) 97 (78.9) 5 (4.1)
Haematuria 11 (8.9) 111 (90.2) 1 (0.8)
Proteinuria 9 (7.3) 112 (91.1) 2 (1.6)
Positive ANA 9 (7.3) 56 (45.5) 58 (47.2)
Positive ANCA 12 (9.8) 46 (37.4) 65 (52.8)
Positive anti–DNA 0 20 (16.3) 103 (83.7)
ANA, anti–nuclear antibody; ANCA, anti–neutrophil cytoplasmic antibodies; anti–DNA, anti–DNA antibodies.
patients, and corticosteroids to 13%. In addition to 
corticosteroids, corticosteroid pulses were administered 
in one patient and ACE inhibitors were used in five 
patients for a prolonged period (from six months to a 
year, or even longer).
Renal biopsy was performed in three patients. A renal 
biopsy was performed when laboratory and clinical 
data predicted the possibility of significant nephritis 
and the need for additional treatment, especially 
immunosuppressive therapy. The histopathology in all 
three patients showed IgA glomerulonephritis grade 
III according to Haas, grade III according to Lee, and 
M1E1S0T0 according to the Oxford classification 
(11). One of the patients, an active athlete, refused 
renal biopsy and immunosuppressive treatment. All 
children with kidney involvement were followed–
up. At presentation, mostly low–grade haematuria 
and proteinuria were present. Six patients had both 
haematuria and proteinuria, and three had only 
proteinuria (0.5–1 g/day). Among the patients that 
relapsed, at the time of the study, only one adolescent 
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Table 1. Laboratory characteristics of the patients.
Laboratory characteristic Number of patients Median (IQR)(minimum–maximum) Reference value/range
Number of patients out of 
range
ASLO (IU) 63 251 (IQR 499)(10–3450) < 200 Above: 36 (57%)
Systolic pressure (mmHg) 34 113 (IQR 20)(93–189) Age–dependent Above: 13 (38%)
Diastolic pressure (mmHg) 34 74 (IQR 11)(62–110) Age–dependent Above: 14 (41%)
Leucocyte count (x 10^9) 122 10.3 (IQR 5.0)(3.32–22.57) Age–dependent
Above: 43 (35%)
Below: 3 (2.5%)
Haemoglobin (g/L) 122 128.5 (IQR 12)(101–160) Age–dependent Below: 31 (25%)
Sedimentation rate (mm/h) 49 25 (IQR  27)(2–79) > 15 Above: 33 (67%)
CRP (mg/L) 114 9 (IQR  16)(0–204) <3
>50: 8 (7%)
>3 and <50: 70 (61%)
<3: 26 (23%)
Urea (mmol/L) 98 3.9 (IQR 1.3)(1.6–6.0) Age–dependent Below: 10 (10%)
Creatinine (µmol/L) 100 40 (IQR  19)(14–102) Age–dependent
Above: 2 (2%)
Below: 5 (5%)
Albumin (g/L) 7 34 (IQR 10)(27–44) 32–55 Below: 2 (28%)
C3 (g/L) 55 1.35 (IQR 0.32)(0.92–1.86) 0.9–1.8 Above: 1 (1.8%)
C4 (g/L) 55 0.27 (IQR 0.10)(0.11–1.43) 0.1–0.4 Above: 3 (5.4%)
Immunoglobulin IgM (g/L) 85 0.92 (IQR 0.54)(0.33–2.06) Age–dependent Below: 1 (1.1%)
Immunoglobulin IgG (g/L) 85 10.9 (IQR 3.8)(2.15–23.3) Age–dependent
Above: 4 (4.7%)
Below: 9 (11%)
Immunoglobulin IgA (g/L) 84 1.84 (IQR 1.21)(0.14–15) Age–dependent
Above: 2 (2.4%)
Below: 6 (7.1%)
ASLO, antistreptolysin titre; CRP, C–reactive protein; AST, aspartate aminotransferase; ALT, alanine aminotransferase; GGT, gamma–glutamyl transpep-
tidase; C3, complement fraction C3; C4, complement fraction C4.
had significant proteinuria (2.7 g/day)..
There were no significant differences in clinical 
presentation recorded between boys and girls (data not 
presented). The recurrence rate was slightly higher in boys 
than in girls (7/57 [12.3%] vs. 12/ 66 [18%]).
DISCUSSION 
The basic demographic characteristics of our patients were 
similar to the available global data, with a balanced gender 
distribution (2). The median patient age was 6.0 years, 
which is similar to that reported in the literature (2,12).
Along with purpura, other organ involvement occurred 
at the same frequency as that reported in the literature 
(2). Almost 10% of patients had signs of renal vasculitis. 
Blood pressure, monitored by sporadic measurements 
and ambulatory blood pressure monitoring, was normal 
in most patients. Otherwise, the initiation of medication 
was considered.
There are no specific laboratory markers for the diagnosis of 
HSP. Anaemia may be present as a result of gastrointestinal 
bleeding. Leucocytosis with elevated inflammatory 
parameters, due to an active respiratory infection or part 
of the immune response, is also common (13). The blood 
count of most of our patients was within the normal 
range. Patients showed normal kidney function tests and 
liver damage markers. The albumin value was very rarely 
checked and we did not notice any major deviations from 
the norm because only a few patients presented with 
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severe renal involvement, reflected by severe protein loss 
or signs of compromised renal function, and even fewer 
presented with severe gastrointestinal manifestations. The 
latter can be a common cause of hypalbuminaemia in the 
absence of renal involvement (14). Research has shown 
the potential role of activation of the alternative pathway 
of the complement system in the pathogenesis of HSP 
(15). In practice, most patients have normal complement 
values, as observed in our patient group. Rarely, there is 
a transient reduction in values, which is not associated 
with specific complications or a worse prognosis (16). IgA 
immunoglobulins can be elevated in HSP, as recorded 
in our patients, but they are non–specific (5). Testing for 
antinuclear antibody, neutrophil granulocytes, and anti–
DNA antibodies was performed in less than half of cases 
because there is no evidence in the literature that they are 
relevant to the pathogenesis nor do they have any clinical 
relevance; however, testing is occasionally performed for 
differential diagnostic dilemmas (5,17). 
Treatment of HSP is mainly supportive (7). Therefore, 
NSAIDs were often prescribed with rest, hydration, and 
monitoring. Corticosteroids were rarely prescribed. Their 
use is still a matter for discussion. Some studies have 
shown their effectiveness primarily in the early stages 
of the disease (18), while others have emphasised their 
effectiveness in the treatment of gastrointestinal, joint, 
and skin symptoms (19). An increasing number of studies 
show that corticosteroids do not play a therapeutic role in 
patients with kidney manifestations, nor do they prevent 
the development of HSP nephritis (20).
Very rarely, in cases of renal involvement, ACE inhibitors 
were prescribed at the first presentation. However, ACE 
inhibitors play a greater role in the long–term treatment 
of HSP nephritis. Relapses of HSP usually occur in 
approximately one–third of cases, mostly with cutaneous 
manifestations (9,21). A smaller number of relapses was 
observed in our sample, which may be because patients 
are always alert to the possibility of relapsing purpura, 
which is inherently innocuous and is always followed–up 
by a paediatrician in primary care. Patients with relapsing 
purpura can usually be followed–up in primary care 
without referral back to the specialist care. In primary care, 
regular urine testing and blood pressure measurements are 
performed, and the development of kidney manifestations 
is the main indication for referral back to specialist care. 
The lower number of relapses may also be due to the 
smaller number of patients treated with severe clinical 
outcomes compared to other European studies (9,21,23). 
Nonetheless, as reported elsewhere globally (21,23), 
purpura relapses were most commonly recorded in our 
patients because of parents' concerns. This was followed by 
kidney manifestation, joint swelling, and gastrointestinal 
problem relapses, which differs from the reports in the 
literature, where relapses with cutaneous presentations 
(present in 88.7%) were followed by gastrointestinal 
(27.1%), renal (24.8%), and joint (16.5%) manifestations 
(9,21). The majority of patients who relapsed and required 
treatment returned because of renal manifestations, 
and the most commonly prescribed therapy was ACE 
inhibitor. These act as renoprotective agents, mostly due to 
their protein–lowering effect, although the also exert other 
positive effects (22). Patients with kidney manifestations 
are also followed–up for the longest period since the long–
term prognosis of HSP depends on renal function (2).
A study similar to ours was carried out in Spain, where they 
reported a similar median patient age of 7.5 years. In 38% of 
patients, a prior respiratory infection was detected, whereas 
this was recorded in more than half of cases in our study, 
with positive streptococcal tests in approximately one–
quarter of patients. Along with purpura, the most frequent 
symptoms in the Spanish study were gastrointestinal 
(64.5%), followed by joint impairment (63.1%) and kidney 
involvement (41.2%). Similar to our findings, leucocytosis 
was reported occasionally, along with the elevation of 
other inflammatory factors. However, in contrast to the 
Spanish study, we did not record any cases of anaemia, and 
we rarely recorded elevated immunoglobulin IgA values, 
whereas elevated serum IgA levels were observed in 31.7% 
of patients. Compared to our study, corticosteroids were 
more commonly administered in the Spanish study (35% 
vs. 13%), while NSAIDs were more often prescribed in 
our study (61.8% vs. 14%) (21).
In an Italian study, a similar average age at presentation was 
also reported (6.1 years). Purpura was accompanied by joint 
involvement in 74% of patients, kidney manifestations 
in 54%, and gastrointestinal manifestations in 51%. 
Scrotal oedema was also reported in 13% of patients. The 
most frequent laboratory abnormalities were an elevated 
sedimentation rate, elevated IgA immunoglobulins, 
and proteinuria. HSP relapse was reported in 35% of 
patients, and more frequently in patients with elevated 
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sedimentation rates and when corticosteroid therapy was 
required (23).
Other published studies on different populations with a 
similar age at presentation showed a relatively different 
clinical picture and treatment. An epidemiological and 
clinical study performed in South Korea reported a similar 
average age (6.93 years), but more frequently reported 
gastrointestinal tract manifestations (75%), joint (69.8%), 
and kidney involvement (26.9%) (24).
A similar Turkish study found a higher incidence of HSP 
in boys than in girls. Skin purpura was accompanied by 
joint involvement in 66% of patients, gastrointestinal 
system manifestations in 56%, and kidney damage in 30%, 
which is the same order as in our study, but with higher 
frequencies. Corticosteroids were administered to 44% of 
patients with abdominal pain (25), which is slightly higher 
than in our study, where seven out of 24 (29%) patients 
with abdominal pain received corticosteroids.
Our research could be further expanded by comparing 
the clinical picture between the paediatric and adult 
population, as performed by Kang et al. (26). Upper limb 
purpura, diarrhoea, anaemia, elevated C–reactive protein, 
and IgA immunoglobulins with more severe renal 
involvement were found to be more common in adults 
than in children.
The main limitation of the study is the lack of some data 
due to its retrospective nature. 
In conclusion, the paediatric patients with HSP in our 
study showed similar characteristics to those reported from 
other countries. Purpura–accompanying manifestations 
appeared in a similar order of frequency to those reported 
in some previous studies, but were generally less commonly 
observed. Corticosteroid therapy was rarely administered. 
ACE inhibitors were more commonly prescribed in 
patients with kidney involvement. Those with kidney 
manifestations were followed–up for the longest period 
because the prognosis of HSP depends on the degree of 
renal damage.
CONFLICT OF INTEREST STATEMENT AND 
FUNDING 
The authors have no conflicts of interest or funding to 
declare.
AUTHOR CONTRIBUTIONS
NMV: study design; patient data collection, follow–up, 
and treatment; supervised and guided the study; data 
revision; and manuscript revision.
MM: collected, analysed, and interpreted the data; drafted 
the manuscript. 
All authors read and approved the final manuscript.
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