2. Introduction
Also known as
anaphylactoid purpura ,
leukocytoclastic angiitis.
Small vessel vasculitis
having cutaneous and
systemic manifestations.
Most common cause of
non-thrombocytopenic
purpura in children.
4. History
The syndrome takes its name from 2 German
physicians.
Johan Schönlein first described peliosis
rheumatica or purpura associated with arthritis.
Thirty years later, Henoch described the GI
manifestations.
7. Etiology
Vaccines associated with Henoch-
Schönlein purpura
Typhoid and paratyphoid A and B
Measles
Yellow fever
cholera
8. Pathophysiology
IgA mediated vasculitis of
small vessels.
Deposition of IgA and C3
in skin and renal
glomeruli.
Immune complexes
activates complement
pathway.
9. Pathophysiology
Chemotactic fragments
are activated.
Inflammatory cells are
attracted.
Lysosomal enzymes are
released and destroy
cellular matrix of vessels.
Polymorphonuclear
leukocyte disintegrate to
nuclear dust;
leukocytoclastic angiitis.
10. Clinical manifestations
The prodrome is associated with the
following:
Headache
Anorexia
Fever
After the prodrome, a rash, abdominal pain,
peripheral edema, vomiting and/or arthritis
develop.
11. Cutaneous manifestations
The rash appears in
100% of patients
presenting feature in
50%.
including the lower
trunk, lower extremities,
buttocks and perineum.
The rash typically
appears in crops with
new crops appearing in
waves.
12. Cutaneous manifestations
Rash begin as
maculopapular rash.
initially blanch on
pressure and progress
to purpura.
Palpable purpura;
evolve from red to
purple to rusty brown
and fades away.
13. Cutaneous manifestations
Crops lasts for 3-10 days.
Reappear at interval of 3-4 M.
<10% children may not and until as late as a
year.
Damage to vessels; local angioedema.
14. G.I involvement
85% of patients will have GI symptoms,
including abdominal pain, nausea, and
vomiting.
The most common symptom is colicky
abdominal pain.
Peritoneal exudates,mesenteric lymphadenitis
haemorrhage into bowel.
Intussusception may occur.
15. Arthritis
Joint involvement is present in 75% of
patients
presenting sign in approximately 25%.
The large joints ( knees and ankles) are most
commonly involved, with pain and edema
being the only symptoms.
The arthritis resolves completely over several
days without permanent articular damage.
16. Renal involvement
Renal involvement is
present in 30-50% of
patients.
persist as long as 6
months after the
onset of the rash.
manifests in a range
from mild hematuria
or proteinuria to
oliguria and renal
failure.
17. Diagnostic criteria..
established by the American College of
Rheumatology.
These criteria include:
palpable purpura - hemorrhage (bleeding) into the
skin or mucous membranes and other tissues.
at onset of the disease, the patient is younger than
20 years of age.
18. Diagnostic criteria..
bowel angina, or pain in the abdomen which is
worse after meals, or bowel ischemia which may
result in bloody diarrhea.
presence of certain cells when a tissue sample
from the purpura is examined under the
microscope.
23. TREATMENT…
Symptomatic treatment
Adequate hydration
pain control with acetaminophen
avoidance of competitive activities
avoidance of maintatining lower limbs in
dependent position
24. Treatment
Specific treatment for Henoch-Schönlein
purpura will be determined by
child's overall health and medical history
extent of the condition
child's tolerance for specific medications
expectation for the course of the disease
specific organs that are affected
25. Treatment
Intestinal complications
Oral or I/V corticosteroids (1-2mg/kg/day).
Hydrostatic reduction of intussusception
Surgical resection of intussusception
26. Treatment
Chronic or recurrent HSP;
Pulse I/V methylprednisolone
30mg/kg/day, max. 1G /day for 3 days
followed by 1-2 times wkly and tapered
depending on response.
27. Treatment
HSP nephritis
High dose steroids.
Patients with crescentic glomerulonephritis;
Cyclophosphamide, azathioprine
Dipyridamole and heparin in severe nephritis
Anticardiolipin antibodies present ; start
aspirin.
28. Complications…
Nephrotic syndrome
End stage renal disease
intussuscption
Bowel perforation
Testicular torsion
Chronic hypertension
Seizures, paresis and coma
30. Prognosis
Self limiting vasculitis with excellent
prognosis.
<1% develop persistent renal disease.
<0.1% have serious renal disease
Follow for renal pathology uptill 6 M;
persistent renal disease refer to
nephrologist.
31. Prognosis…
Microscopic hematuria at
presentation
excellent prognosis.
Nephritic or nephrotic syndrome at
presentation
highest risk of developing chronic renal failure
and hypertension.
32. University of Michigan
Department of Pediatrics
Evidence-Based Pediatrics
Steroids Prevent Delayed Renal Disease in
Henoch-Schonlein Purpura
Corticosteroids given for 14 days appear to substantially
reduce the incidence of renal disease in children with
HSP .
children who did not receive steroids, developed renal
disease 2 to 6 weeks after acute episode.
children who received steroids, none developed renal
disease persistent renal insufficiency or ESRD.
34. Objective..
To determine benefits and harms of
therapies used to prevent or treat renal
involvement in H.S.P.
35. Study design
Systemetic review of randomised
controlled trials.
Subjects.
1230 children aged less than 18 Y.
36. Inclusion criteria…
All RCTs of interventions compared with
placebo.
Interventions included;
Corticosteroids
Antiplatelet agents
Immunosuppressive agents
ACE inhibitors
37. Primary outcome..
Children who developed
Persistent renal involvement
Haematuria
proteinuria
Hypertension
Nephrotic syndrome
Nephritis
Need for dialysis or transplant
41. Effect of antiplatelets and heparin
With antiplatelet therapy No significant
difference in risk of renal disease in
children with or without treatment.
Heparin significantly reduced risk of renal
involvement.
42. What is already known…
Controversy remains as to value of
corticosteroids in preventing renal disease.
Randomised controlled trials are limited
regarding efficacy of several treatments in
HSP.
43. What this study adds….
No significant benefit of short course
prednisone administered at presentation
of HSP in preventing renal disease.
No significant benefit in treating severe
HSP nephritis with cyclophosphamide or
cyclosporin.
44. Conclusion…
Data from randomised trials for any
intervention used to improve renal
outcome in HSP are very sparse except
short term steroid , which has not been
shown to be effective.