HSP pediatrics

1. Henoch-schonlein
purpura
Dr.Anita Lamichhane
Dept of 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 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.

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/U
outcome
interventio
n
HSPrenal
incident
study
12M
Hematuria
proteinuria
Prednison
e given for
7-10 days.
Tappered
over next
7-14 days.
32 %
Dudley
2007
12M
RBCs>5HP
F
UP>300m
g/l
15 %
Huber
2004
unclear
Hematuria
proteinuria
0 %
Islek
1999
6M
UP>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/U
outcome
intervention
Inclusion
criteria
study
6Y
Proteinuria
ESRD
death
Cyclophosph
amide for
42D.
Nephrotic
range
proteinure
a renal
biopsy;
ISKDC>III
2004
2.9Y
Remission
UP/UC<200
Cyclosporin
for 12M
ACE
inhibitor
2006

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.

45. Thank you…..