Henoch scholein purpura

Henoch–Schönlein
purpura(HSP)
Prof. Dr. Saad S Al Ani
Senior Pediatric Consultant
Head of Pediatric Department
Khorfakkan Hospital
Sharjah ,UAE
saadsalani@yahoo.com

Introduction
• Henoch–Schönlein purpura is the most
common systemic vasculitis of childhood
presenting with a tetrad of:
• Purpura
• Arthritis or arthralgia
• Abdominal pain
• Renal disease.
06/01/2019
Henoch-Scholein Purpura
2

Introduction (Cont.)
• Henoch–Schönlein purpura belongs to
the group of non‐granulomatous,
predominantly small vessel vasculitides
06/01/2019
Henoch-Schönlein Purpura
3
Ozen S, Ruperto N, Dillon MJ et al. EULAR/PReS endorsed consensus criteria for the
classification of childhood vasculitides. Ann. Rheum. Dis. 2006; 65: 936–941.

Definition
• Henoch-Schönlein purpura (HSP) is an
acute immunoglobulin A (IgA)–mediated
disorder characterized by a generalized
vasculitis involving the small vessels of the
skin, the gastrointestinal (GI) tract, the
kidneys, the joints, and, rarely, the lungs
and the central nervous system (CNS)
06/01/2019
4
https://emedicine.medscape.com/article/984105-overview

New EULAR/PRINTO/PRES endorsed
classification of childhood vasculitis
New EULAR/PRINTO/PRES endorsed classification of
childhood vasculitis
I Predominantly large vessel vasculitis
• Takayasu arteritis
II Predominantly medium sized vessel vasculitis
• Childhood polyarteritis nodosa
• Cutaneous polyarteritis
• Kawasaki disease
06/01/2019
5

classification of childhood vasculitis (Cont.)
III Predominantly small vessel vasculitis
(A) Granulomatous
• Wegener's granulomatosis
• Churg‐Strauss syndrome
(B) Non-Granulomatous
• Microscopic polyangiitis
• Henoch–Schönlein purpura
• Isolated cutaneous leucocytoclastic vaculitis
• Hypocomplementic urticarial vasculitis
06/01/2019
6

classification of childhood vasculitis (Cont.)
IV Other vasculitides
• Behçet disease
• Vasculitis secondary to infection (including hepatitis B
associated polyarteritis nodosa) malignancies, drugs,
including hypersensitivity vasculitis
• Vasculitis associated with connective tissue diseases
• Isolated vasculitis of the central nervous system
• Cogan syndrome
• Unclassified
06/01/2019
7

Epidemiology
• HSP is the most common childhood
vasculitis
• Annual incidence varies between 10 and
30 cases per 100 000 children < 17 years
(1)
• The mean age of presentation is 6 years
with most cases in children < 10 years of
age,
06/01/2019
8
1.Penny K, Fleming M, Kazmierczak D et al. An epidemiological study of Henoch‐Schönlein purpura. Pediatr.
Nurs. 2010; 22: 30–35.

Epidemiology (Cont.)
• An equal incidence in males and
females (2)
• HSP occurs predominantly in cold
months of the year
• It has been reported worldwide.
06/01/2019
9
2.González LM, Janniger CK, Schwartz RA. Pediatric Henoch‐Schönlein purpura. Int. J. Dermatol. 2009; 48: 1157–
1165.

Aetiology and Pathogenesis
• The majority of HSP cases are preceded
by an upper respiratory tract infection
suggesting a potential infectious trigger.
06/01/2019
10

Aetiology and Pathogenesis (Cont.)
• The most commonly implicated
respiratory pathogens include:
• Streptococcus
• Staphylococcus
• Parainfluenza
Association between virtually all
respiratory pathogens and HSP (1)
06/01/2019
11
(1)Weiss PF, Klink AJ, Luan X, Feudtner C.Temporal association of Streptococcus, Staphylococcus, and parainfluanza
pediatric hospitalizations and hospitalized cases of Henoch-Schönlein purpura..
J. Rheumatol., 2010

• The characteristic pathological feature
of HSP vasculitis is a deposition of IgA-
containing immune complexes in :
 The vessel walls of affected organs
 The kidney mesangium.
06/01/2019
12
(Cont.)

• Deposited immune complexes activate
the alternative complement pathway
(with deposition of C3) and recruit
inflammatory cells causing
glomerulonephritis (1,2)
06/01/2019
13
(Cont.)
(1) Sanders JT, Wyatt RJ. IgA nephropathy and Henoch Schönlein purpura nephritis Curr.
Opin. Pediatr., 2008
(2) Novak J, Julian BA, Tomana M. IgA glycosylation and IgA immune complexes in
the pathogenesis of IgA nephropathy.Semin. Nephrol., 2008

• Deposition of IgA1-containing immune
complexes in other sites (skin, gut,
joints) leads to organ-specific clinical
manifestations of HSP.
06/01/2019
14
(Cont.)

Clinical Manifestations
• HSP is a systemic vasculitis with
multiorgan involvement.
• The classic tetrad of signs and
symptoms includes:
1/ Palpable purpura
2/ Arthritis or arthralgia
3/ Abdominal pain
4/ Renal disease
06/01/2019
15

Typical prodrome of HSP
• The typical prodrome of HSP includes
the following:
Headache
Anorexia
Fever
06/01/2019
16

The Most common
symptoms
• Rash (95-100% of cases)
especially involving the legs; this is the hallmark
of the disease
• Abdominal pain and vomiting (35-85%)
• Joint pain (60-84%)
especially involving the knees and ankles
• Subcutaneous edema (20-50%)
• Scrotal edema (2-35%)
• Bloody stools
06/01/2019
17

Palpable purpura
• Skin involvement is present in all children with
HSP (1)
• Petechiae and palpable purpura are the most
common
• Other skin rashs:
Erythematous
Macular
Urticarial
Bullous
have also been observed.
06/01/2019
18
(1) González LM, Janniger CK, Schwartz RA. Pediatric Henoch-Schönlein purpura.Int. J.
Dermatol., 2009

Palpable purpura (Cont.)
• Purpura is characteristically distributed
symmetrically over :
Extensor surfaces of the lower limbs,
Buttocks
Forearms
06/01/2019
19

06/01/2019
20
Mayo Clinic

06/01/2019
21
Obgyn Key

Palpable purpura (Cont.)
• Recurrence of purpura, which might be
associated with more severe renal
involvement, is observed in
of children with HSP
06/01/2019
22

Arthritis/arthralgia
• Arthritis/arthralgia is present in three
quarters of children with HSP (1)
• Joint involvement is usually
oligoarticular with large joints of the
lower extremities (knee, ankle, hip)
most commonly affected
06/01/2019
23
Trapani S, Micheli A, Grisolia F et al. Henoch Schonlein purpura in childhood: epidemiological and clinical
analysis of 150 cases over a 5‐year period and review of literature. Semin. Arthritis Rheum. 2005; 35: 143–153.

Arthritis/arthralgia (Cont.)
• There is usually prominent periarticular
Swelling, Tenderness and Pain
• Erythema and joint Effusion are rare
• Arthritis is non‐deforming and heals
without chronic damage within a few
weeks.
06/01/2019
24

Abdominal pain
• Approximately two thirds of children
with HSP develop abdominal pain (1)
• Usually diffuse, increasing after meals,
and sometimes associated with nausea
and vomiting
06/01/2019
25
(1) Choong CK, Beasley SW. Intra‐abdominal manifestations of Henoch‐Schönlein purpura. J.
Paediatr. Child Health1998; 34: 405–409.

Abdominal pain (Cont.)
• The most severe gastrointestinal
complication is intussusception, affecting
3–4% patients with HSP
• In 60% of these cases, it is limited to small
bowel
• Clinical presentation of intussusception is
characterised by severe abdominal pain,
often colicky in nature and vomiting.
06/01/2019
26

Abdominal pain (Cont.)
• Other significant, though less common
gastrointestinal complications are:
 Gangrene of the bowel
 Bowel perforation
 Massive haemorrhage
06/01/2019
27

Renal involvement
• Renal involvement is reported in 20–55%
of children with HSP (1,2)
 Isolated microscopic haematuria (most
common )
 Proteinuria of variable degree
 Nephrotic syndrome ( in severe cases)
06/01/2019
28
(2)Jauhola O, Ronkainen J, Koskimies Oet al. Renal manifestations of Henoch‐Schönlein purpura in a 6‐month
prospective study of 223 children. Arch. Dis. Child. 2010; 95: 877–882.
(1)Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch‐Schonlein
purpura with normal or minimal urinary findings: a systematic review. Arch. Dis. Child. 2005; 90: 916–920.

Renal involvement (cont.)
Hypertension might develop (at the
onset or during recovery).
Renal function is usually normal
The occasional patient might present
with a progressive glomerulonephritis
with significant renal impairment.
06/01/2019
29

Other less common clinical
manifestations of HSP
• Cerebral vasculitis
• Scrotal or testicular haemorrhage
• Interstitial pulmonary haemorrhage (1).
• Distal ureteric vasculitis resulting in
ureteric stenosis
06/01/2019
30
(1)Ha TS, Lee JS. Scrotal involvement in childhood Henoch‐Schönlein purpura.
Acta Paediatr. 2007; 96: 552–555.

Diagnosis of HSP
• Is based on the presence of purpura
(palpable) or petechiae (without
thrombocytopenia) with lower limb
predominance (mandatory criterion)
plus at least one of the flowing four
features:
06/01/2019
31
Ozen S, Pistorio A, Iusan SM et al. EULAR/PRINTO/PRES criteria for Henoch‐Schönlein purpura, childhood polyarteritis
nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II.Final classicication criteria. Ann. Rheum.
Dis.2010; 69: 798–806.

Diagnosis of HSP (Cont.)
(1) Abdominal pain
(2) Arthritis or Arthralgia
(3) Leukocytoclastic vasculitis or
Proliferative glomerulonephritis with
predominant deposition of IgA histologically
(4) Renal involvement
(haematuria, red blood cell casts or proteinuria)
06/01/2019
32
Ozen S, Pistorio A, Iusan SM et al. EULAR/PRINTO/PRES criteria for Henoch‐Schönlein purpura, childhood polyarteritis
nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II.Final classicication
criteria. Ann. Rheum. Dis.2010; 69: 798–806.

Laboratory tests
• Laboratory tests are complementary in
assessing renal involvement
 Urinalysis
 Urine microscopy
 Serum creatinine
06/01/2019
33

Imaging studies
• Imaging studies are helpful in the:
 Evaluation of abdominal involvement
 Potential complications (intussusception).
06/01/2019
34

Ultrasound examination of the abdomen in a child with HSP
06/01/2019
35
http://www.ultrasoundcases.info/Monthly-Case.aspx?month=147&show=1

Histopathology
• In children with incomplete or unusual
presentation, biopsy of the affected organ
(skin, kidney) confirms the diagnosis.
06/01/2019
36

Histopathology (cont.)
06/01/2019
37
Glomerular arteriole showing a vasculitis with fibrinoid necrosis of the vessel
wall (arrow) and swelling of the endothelial cells (E). Surrounding the vessel
there is granulomatous inflammation (G) (haematoxylin and eosin, ×400).
https://bjo.bmj.com/content/89/9/1221.2.ful
l

Management of HSP
• Management of HSP includes:
 Supportive care
 Symptomatic therapy
 Immunosuppressive treatment
(in some cases)
06/01/2019
38

Supportive care
• The basic principles of supportive care
consist of:
 Maintenance of good hydration
 Symptomatic pain relief
 Monitoring for the development of
complications
06/01/2019
39

Symptomatic therapy
• Non‐Steroidal Anti‐Inflammatory Drugs
(NSAIDs) can be used for arthritis/arthralgia
• Early glucocorticosteroids( GCS) treatment :
 shortened duration of abdominal pain
 decreased risk of intussusception
 decreased risk of surgical intervention (1)
• GCS treatment cannot be recommended in
all patients with HSP since the majority will
improve spontaneously
06/01/2019
40
(1)Weiss PF, Klink AJ, Localio R et al. Corticosteroids may improve clinical outcomes during
hospitalization for Henoch‐Schönlein purpura. Pediatrics2010; 126: 674–681.

Glucocorticosteroids (Cont.)
• Pulse intravenous methylprednisolone
followed by 3 to 6‐month course of oral
steroids is most commonly used in
patients with rapidly progressive
glomerulonephritis or nephrotic
syndrome (usually accompanied by crescents on kidney biopsy),
06/01/2019
41
Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of
Schönlein‐Henoch purpura nephritis. Pediatr. Nephrol. 1998; 12: 238–243.

Indication for GCS usage
• Persistent nephrotic syndrome
• Crescents in more than 50% of glomeruli
• Severe abdominal pain
• Substantial GI hemorrhage
• Severe soft tissue edema
• Severe scrotal edema
• Neurologic system involvement
• Intrapulmonary hemorrhage
06/01/2019
42

Immunosuppressive
treatment
• Immunosuppressive treatment of HSP
nephritis is used in patients with
severe kidney involvement (nephrotic
range proteinuria and/or progressive
renal impairment)
06/01/2019
43

Immunosuppressive
treatment (cont.)
• A current KDIGO guideline
suggests adding cyclophosphamide
to steroid treatment for crescentic
glomerulonephritis
06/01/2019
44
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO
Clinical Practice Guideline for Glomerulonephritis. Kidney Int. 2012; 2: 139–247.

Prognosis of HSP
• In the majority of children, the outcome
of HSP is excellent with spontaneous
resolution of symptoms and signs.
• HSP recurs in approximately one third of
patients, typically within 4 months of the
initial presentation.
06/01/2019
45

Prognosis of HSP (Cont.)
• Recurrent purpura can be occasionally
associated with joint complaints and
episodes of gross haematuria although
each subsequent episode is generally
milder and shorter.
• The long‐term morbidity of HSP is
related to the degree of HSP nephritis.
06/01/2019
46

Initial follow-up for kidney
involvement
• The aim of the initial follow‐up is to
identify patients with worsening kidney
involvement and is based on:
 serial urinalyses
 blood pressure measurement
 blood tests to assess kidney function and
exclusion of other causes of glomerulonephritis
06/01/2019
47

• HSP is :
 Common childhood vasculitis
 Good outcome in the majority
of affected children
 Small subgroup of children
who will develop significant
renal impairment
 Some of them will eventually
progress to ESKD and require
kidney transplantation.
06/01/2019
48

• Patients with severe abdominal
pain need prompt evaluation and
investigations to exclude
intussusception
• In cases with sudden change of
mental status, intracranial
haemorrhage should be excluded
with appropriate imaging
06/01/2019
49

• Patients with compromised renal
function taking NSAIDs need close
monitoring of their:
 fluid status
 blood pressure
 renal function.
• Mild renal involvement (microscopic
haematuria or mild proteinuria)
does not require biopsy or
immunosuppressive treatment, but
these children need close follow‐up.
06/01/2019
50

Reference
• https://onlinelibrary.wiley.com/doi/full/10.1111/jpc.12403
• Ozen S, Ruperto N, Dillon MJ et al. EULAR/ PReS endorsed consensus criteria for the classification of
childhood vasculitides. Ann. Rheum. Dis. 2006; 65: 936–941.
• Penny K, Fleming M, Kazmierczak D et al. An epidemiological study of Henoch‐Schönlein
purpura. Pediatr. Nurs. 2010; 22: 30–35
• Weiss PF, Klink AJ, Luan X, Feudtner C.Temporal association of Streptococcus, Staphylococcus, and
parainfluanza pediatric hospitalizations and hospitalized cases of Henoch-Schönlein purpura.. J.
Rheumatol., 2010
• Sanders JT, Wyatt RJ. IgA nephropathy and Henoch Schönlein purpura nephritis Curr. Opin. Pediatr.,
2008
• Novak J, Julian BA, Tomana M. IgA glycosylation and IgA immune complexes in the pathogenesis of IgA
nephropathy.Semin. Nephrol., 2008
• González LM, Janniger CK, Schwartz RA. Pediatric Henoch-Schönlein purpura. Int. J. Dermatol., 2009
• Trapani S, Micheli A, Grisolia F et al. Henoch Schonlein purpura in childhood: epidemiological and
clinical analysis of 150 cases over a 5‐year period and review of literature. Semin. Arthritis
Rheum. 2005; 35: 143–153.
• Jauhola O, Ronkainen J, Koskimies O et al. Renal manifestations of Henoch‐Schönlein purpura in a
6‐month prospective study of 223 children. Arch. Dis. Child. 2010; 95: 877–882.
06/01/2019
51

06/01/2019
52
Julie Blais Comeau

Henoch scholein purpura

Recommended

Recommended

More Related Content

What's hot

What's hot (20)

Similar to Henoch scholein purpura

Similar to Henoch scholein purpura (20)

More from Dr. Saad Saleh Al Ani

More from Dr. Saad Saleh Al Ani (20)

Recently uploaded

Recently uploaded (20)

Henoch scholein purpura