Henoch-schonlein purpura.ppt

HSP pediatrics

1. Henoch-schonlein
purpura
Dr.AnitaLamichhane
Deptof Pediatrics
Lumbini Medical college

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.

3. Epidemiology
Males affected twice as compared to
females.
Common age 2-8 Y.
Incidence ; 9 /100,000.

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.

5. Etiology
Infectious agents associated with Henoch-
Schönlein purpura
Streptococcus species
Yersinia
Legionella
parvovirus
adenovirus
mycoplasma
ebstein-barr virus
varicella

6. Etiology
Drugs associated with Henoch-
Schönlein purpura
Penicillin
Ampicillin
Erythromycin
Quinidine
Quinine

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 rashtypically
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.

19. Investigations..
CBC
anemia
leukocytosis
thrombocytosis
ESR
Raised

20. Investigations.
Immunoglobulins
IgA IgM raised
Urine R/E
WBCs
RBCs
Casts
Albumin

21. Investigations..
Barium enema
Diagnostic and
therapeutic
Ileoileal
intussusception

22. Investigations..
Skin biopsy
Leukocytoclastic
angiitis.
Renal biopsy
Deposition of
IgA
IgM
C3 and fibrin.

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

29. Complications..
Rare complications are;
Cardiac invovement
Mononeuropathies
Pancreatitis
Pulmonary and intramuscular
haemorrhage

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.

33. Prevention and treatment
of renal disease in
Henoch-Schonlein
Purpura…….

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

38. Results
F/Uoutcomeinterventio
n
HSPrenal
incident
study
12MHematuria
proteinuria
Prednison
e given for
7-10 days.
Tappered
over next
7-14 days.
32 %Dudley
2007
12MRBCs>5HP
F
UP>300m
g/l
15 %Huber
2004
unclearHematuria
proteinuria
0 %Islek
1999
6MUP>200m
g/lRBC>5
19 %2006

39. discussion
No significant difference in number of
children with persistent renal disease at
3,6 and 12 M after short term steroid
therapy.

40. Immunosuppressive therapy..
F/UoutcomeinterventionInclusion
criteria
study
6YProteinuria
ESRD
death
Cyclophosph
amide for
42D.
Nephrotic
range
proteinure
a renal
biopsy;
ISKDC>III
2004
2.9YRemission
UP/UC<200
Cyclosporin
for 12M
ACE
inhibitor
2006

41. Effect of antiplatelets and heparin
With antiplatelet therapyNo 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.

45. Thank you…..