Henoch–Schönlein purpura (HSP) is a type of vasculitis that causes small vessel inflammation, especially in the skin, digestive tract, and kidneys. It is more common in children ages 4-7 and is often triggered by infections. The skin presents with purpuric rashes and joint pain. Gastrointestinal involvement can cause abdominal pain, bleeding, or intussusception. Kidney involvement results in proteinuria and potentially renal failure. Treatment involves steroids, immunosuppressants, and addressing complications like gastrointestinal bleeding. Prognosis is generally good but long term kidney issues can occasionally occur.

1. Name : Low Wei Quan
Group: 88
Henoch–Schönlein purpura

2. What is HSP ?
-Is called haemorrhagic vasculitis or ANCA negative
vasculitis
- Small vessel vasculitis involving vessels of
microcirculation which are venules , capillaries
and small arteries and characterised by
palpable purpura.
- HSP is a systemic disease and it can target any
tissue or any organ for example skin ,
Gastrointestinal Tract ( GI ) , Kidneys , joints ,
lung and Central nervous system ( CNS).

3. Incidence
 prevalence of HSP peaks in children aged 4-7
years, but the condition is also seen in adults
 male-to-female ratio is 1.5:1.

4. Etiology
 Infections:
-Mononucleosis
-Group A streptococcal Infection (most common)
-Hepatitis
-Mycoplasma infection
-Campylobacter enteritis
-Hepatitis C–related liver cirrhosis
-Subacute bacterial endocarditis
- Helicobacter pylori infection (specifically noted in
China )
-Yersinia infection
-Shigella infection
-Salmonella infection
-Brucellosis

5.  Vaccination
- Typhoid and paratyphoid A and B
-Measles
-Yellow fever
-Cholera
 Environmental exposure to the following may
precede the development of HSP:
-Drugs (eg, ampicillin, erythromycin, penicillin,
quinidine, quinine, losartan, and cytarabine )
-Foods
-Horse serum
-Cold exposure
-Insect bites

6. Pathogenesis
 HSP is an acute immunoglobulin A (IgA)–
mediated disorder.
 IgA aggregates or IgA complexes with
complement deposited in target organs, resulting
in activation of inflammatory mediators such as
tumor necrotic factor β , IL-1, IL-6 prostacyclin,
may play a central role in the pathogenesis of
HSP vasculitis.
 A subpopulation of human lymphocytes bears
surface Fc and/or C3 receptors (complement
receptor lymphocytes), which can bind circulating
immune complexes or C3 generated by activation
of the alternative complement pathway.

7.  As a result , IgA aggregates or IgA complexes
with complement deposited in target organs.

8. Clinical Manifestation
 Constitutional symptom :
-Fever
-anorexia
-Headache
-arthralgia.

10.  Target organs sign and symptoms
-Skin
Bilateral palpable purpura at the lower extremities
and the buttock area . It can be located in
anywhere of the body trunk .
*The higher the location of the purpura in the body
the greater the suspicion of a secondary origin of
vasculitis .
But if the purpura is not palpable it does not mean
that it is not HSP .
Skin pigmentation can also be found in HSP. Why?
Deposition of hemosiderin in the skin .

12. *Joint
- Location : lower extremities .
- Pain and swelling of the joint but no deformities and
non-destructive.
*GI tract
- Colicky abdominal pain especially after meal
- Upper GI will present with melenic stool
- Lower GI will present with hematochezia
- Hematemesis
- massive GI hemorrhage
- In children , one of the dangerous complication is
intussuception as this is a fatal complication that lead to
mechanical obstruction and eventually peritonitis. This
will require surgical intervention .

14. *Renal system
- Glomerulonephritis ( inflammation of glomeruli due to
deposition of immune complex ).
- In one series, acute glomerular lesions, including
mesangial hypercellularity, endocapillary
proliferation, necrosis, cellular crescents, and
leukocyte infiltration,
- In 80% of patients, renal involvement becomes
apparent within the first 4 weeks of illness. Overall,
2-5% of patients progress to end-stage renal failure
(ESRD)
*Lung- Vasculitis may lead to fatal complication which
is pulmonary hemorrhage .
*Heart – involved myocardium

15.  Investigations:
Laboratory investigation :
Full blood count : Leukocytosis , normal platelet count
, occasionally eosinophilia ,.
Serum complement : C3 and C4 will be in normal
level .
Immunofluorescence test :Ig A will be elevated .
ANCA and cryoglobulin can be tested but mostly for
differential diagnosis .
Urinalysis : proteinuria(< 2 g/day) and hematuria
(microscopic) if there is involvement of kidney
Skin biopsy , renal biopsy and instrumental
investigation (CT and MRI) can be done to check
the progression fo the disease.
* Diagnosis of HSP is mainly based on clinical signs
and symptoms.

16.  Complication:
-Myocardial infarction
-Pulmonary hemorrhage
-Pleural effusion
-Intussusception
-GI bleeding
-Bowel infarction
-Renal failure
-Hematuria
-Proteinuria
-Seizures
-CNS bleeding
-Mononeuropathies
-Recurrence of symptoms, specifically those of renal
impairment (rare)

17. Differential diagnosis
-Acute abdomen
-Bowel infarction or perforation
-Drug reactions
-Elevated renal function test results (blood urea nitrogen
[BUN]creatinine)
-Essential mixed cryoglobulinemia
-Global organ involvement
-Leukocytoclastic vasculitis
-Primary antiphospholipid syndrome
-Idiopathic Thrombocytopenic Purpura
-IgA Nephropathy
-Wegener granulomatosis
-Systemic Lupus Erythematosus

18. Treatment
*Induction of remisssion
-Induction with 250-750 mg of intravenous (IV)
methylprednisolone daily for 3-7 days plus
cyclophosphamide 100-200 mg/d administered orally
(PO)
*Maintanence
Maintenance with prednisone 100-200 mg PO every
other day plus cyclophosphamide 100-200 mg/d PO
30-75 days
*Tapering of prednisone by approximately 25 mg/month
(with the cyclophosphamide dose remaining constant)
Discontinuance of treatment after at least 6 months by
abruptly discontinuing cyclophosphamide and
tapering prednisone completely

19. *Other treatment regimens have included IV or oral
steroids with or without any of the following:
 Azathioprine
 Cyclosporine
 Dipyridamole
 High-dose IV immunoglobulin G (IVIg)
 Dapsone ( for skin and joint 100mg /day)
 mycophenolate mofetil,
Plasmapheresis has been useful in treating rapidly
progressive HSP
nephritis.
If there is massive GI haemorrhage or Pulmonary
haemorrhage pulse therapy with glucocorticosteroid
should be initiated immediately.
If intussusception developed , surgical intervention
should be consider.

22. What is the roles of Von Willebrand factor (vWF)?
1. Facilitates the platelet adhesion to damaged
endothelium
2. Acts as the carrier protein for FVIII : C , protecting
it from inactivation and clearance

23.  vWD is divided into three major categories:
 (1) partial quantitative deficiency (type I),
 (2) qualitative deficiency (type II), and type II is
further divided into four variants (IIA, IIB, IIN, IIM),
 (3) total deficiency (type III). vWD

24.  Pathogenesis
Results from either a quantitative or qualitative
deficiency of von Willebrand Factor (vWF). This
causes defective platelet plug formation
Since vWF is a carrier protein for FVIII:C . Patient with
vWD laso are deficient in FVIII : C
There are many different mutations in the vWF gene
and many different genes types of vWD .
Autosomal dominant .
Commonest subtype is type 1 (60-80%) usually fairly
mild and is often not diagnosed until puberty or
adulthood .

26. Clinical manisfestation
-Bruising
-Excessive , prolonged bleeding after surgery
-Mucosal bleeding such as epistaxis and
menorrhagia .
In contrast to haemophilia , spontaneous soft tissue
bleeding such as large haematomas and
haemarthroses are uncommon .

27. Investigation
 Coagulation profile :
-Prothrombin time (PT)
-Activated partial thromboplastin time (aPTT) –
mildly prolonged
-Factor VIII coagulant activity -low level
-Ristocetin cofactor (RCoF) activity
-Concentration of vWF antigen (vWF:Ag)

28. Differential diagnosis
 Factor X
 Factor XI Deficiency
 Hemophilia A

29. Treatment
Desmopressin ( DDAVP) and transfusion therapy .
Type 1 vWD can usually be treated with DDAVP ,
which causes secretion of both FVIII and vWF into
plasma.
DDAVP should be used with caution in children below
1 year of age because it can cause hyponatremia
due to water retention and may cause seizures .
More severe types of vWD are treat with plasma
derived FVIII ( Fresh Frozen Plasma)
Cryoprecipitate is no longer used to treat vWD as it
has not undergone viral inactivation .
IM injection of aspirin and other NSAIDS should be
avoided .