HSP presentation

1. HENOCH-SCHÖNLEIN
PURPURA (HSP)
Prepared by
Dr.Jafar Al-Saggaf

2. Background
 Henoch-Schönlein purpura (HSP) or anaphylactoid purpura: 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).
 The dominant clinical features of HSP include cutaneous purpura,
arthritis, abdominal pain, GI bleeding, orchitis, and nephritis.
 In most of children, an upper respiratory tract infection (URTI)
precedes the clinical onset of HSP by 1-3 weeks.
 HSP is typically an acute, self-limited illness, and treatment is
primarily supportive. However, one third of patients have 1 or more
recurrences.

3. Epidemiology
 The prevalence of HSP peaks in children aged 3-10
years.
 Prevalence of HSP is approximately 15 cases per
100,000 population.
 The disease occurs mostly between November and
January.
 The male-to-female ratio is 3:2
 Whites are affected more often than blacks.

4. Pathophysiology
 IgA aggregates or IgA complexes with complement deposited in
target organs, resulting in elaboration of inflammatory mediators,
including vascular prostaglandins such as prostacyclin, may play a
central role in the pathogenesis of HSP vasculitis.
 Some have speculated that an antigen stimulates the production of
IgA, which, in turn, causes the vasculitis. Allergens, such as foods,
horse serum, insect bites, exposure to cold, and drugs (eg,
ampicillin, erythromycin, penicillin, quinidine, and quinine), may
precipitate the illness.
 The etiology of HSP remains to be clearly defined but is thought to
be multifactorial, with genetic, environmental, and antigenic
components. More than 75% of patients report antecedent URTI,
pharyngeal infection, or GI infection. Multiple bacterial and viral
infectious agents have been associated with the development of
HSP, and cases also have been reported after drug ingestions and
vaccinations

5. Diagnosis - History
 Rash (almost 100%), especially involving the
legs, buttocks and upper thighs.
 GI symptoms (75%): colicky Abdominal pain,
vomiting and bloody diarrhea
 Joint pain and edema(50%), especially involving
the knees and ankles.
 Renal involvement(25%): Hematuria
 Scrotal edema
 Subcutaneous edema (in infants)
 Others: headache, Hemoptysis

6. Diagnosis – PE
 Skin findings: erythematous macular or urticarial lesions 
blanching papules  palpable purpura.
 Site: ankles and lower legs in older children and adults. back,
buttocks, upper extremities, and upper thighs in young
children.
 edema of the scalp, periorbital area, hands, and feet in
infants (AHEI)
 RENAL: proteinuria and hypertension.
 MSK: Joints may be swollen, tender, and painful. Warmth,
erythema, and effusions are not typically associated with
HSP.
 GI: tenderness or rigidity if complicated

7. Investigations - Lab
 CBC (Leukocytosis with eosinophilia)
 CRP & ESR
 RENAL FUNCTION
 URINE ANALYSIS
 STOOL FOR OCCULT BLOOD
 ANA & RF - Absent

8. Investigations - Radiology
 ABDOMINAL ULTRASOUND (if GI symptoms
are present. Findings include thickening of the
bowel wall, free fluid, and intussusception.)
 MRI and CT (are useful for assessing
neurologic findings)

9. Renal Biopsy
 It should be performed when nephrotic
syndrome persists and when renal function
deteriorates.
 biopsy often shows circumferential crescents
in most of the glomeruli. The extent of the
crescents is of prognostic importance.

10. Complications
 Intussusception (usually ileoileal)
 GI bleeding
 Pulmonary hemorrhage
 Pleural effusion
 Bowel infarction and perforation
 Renal failure
 Hematuria
 Proteinuria
 Seizures
 CNS bleeding
 Mononeuropathies
 Recurrence of symptoms, specifically those of renal impairment (rare)
 Acute Pancreatitis
 Acute appendicitis
 Myocardial infarction

11. Treatment
 For minor complaints of arthritis, edema, fever
or malaise symptomatic treatment.
 Hospitalization should be strongly considered
for monitoring for abdominal and renal
complications in case of:
1. severe abdominal pain or vomiting
2. significant GI bleeding
3. marked renal insufficiency

12.  Analgesics: NSAIDs or acetaminophen are effective for joint and
soft tissue discomfort but used cautiously in patients with renal
insufficiency.
 Corticosteroids: Some authors recommend steroids; others do
not. Nevertheless, corticosteroids may be considered in the
following situations:
 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

13.  Prednisone in a dosage of 1 mg/kg/day for 2
weeks and then tapered over 2 more weeks
 Antihypertensives may be indicated with
renal involvement.
 Plasmapheresis has been useful in treating
rapidly progressive HSP nephritis.[75]

14. consultation
 Nephrologist (particularly for assistance in
determining if dialysis is indicated)

15. Prognosis
 HSP is generally a benign disease with an excellent
prognosis.
 Most patients experience complete resolution of
symptoms within 8 weeks, and probably fewer than 5%
experience chronic symptoms, although, Serious GI and
renal complications may occur
 Recurrences occur in as many as 25-50% of patients
within 6 weeks but can happen as late as 7 years after
the initial disease. The higher the number of
recurrences, the higher the likelihood of permanent renal
damage.

16.  Generally, the arthritis resolves completely over
several days without permanent articular damage.
 Kidney damage related to HSP is the primary cause of
morbidity and mortality. As many as 15% of patients
may have long-term renal insufficiency, but no more
than 1-2% will have ESRD.
 Good prognosis factors:
1. Mild kidney involvement
2. No neurologic complications
3. Disease that lasts less than 4-6 weeks initially

17.  Predictors of serious nephropathy or ESRD include the following:
1. Bloody stools
2. Rash persistence
3. Hematuria and proteinuria (patients with only hematuria do not develop
ESRD, but about 15% of patients with hematuria and proteinuria do
develop ESRD)
4. Signs of nephritis or nephrotic syndrome (50% of patients progress to
ESRD within 10 years)
5. Renal biopsy with extensive glomerular crescents

18. Follow up
 In all patients, urinalysis and blood pressure monitoring to
evaluate for renal involvement should be continued for up to
6 months after presentation, even if initial urinalysis results
were normal
 When terminal renal failure develops, long-term hemodialysis
should be instituted until a kidney is available for
transplantation.
 Children who have demonstrated renal manifestations in the
acute phase and continue to have hematuria or proteinuria
should be examined every 3-6 months because renal failure
or hypertension can develop up to 10 years after disease
onset.

19. refference
 Uptodate
 medicine

20. THANKS