Transcript HSP

HENOCH SCHÖNLEIN PURPURA (HSP)
Dr. Mohamed Haseen Basha
Assistant professor ( Pediatrics)
Faculty of Medicine
Al Maarefa College of Science and Technology
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CLASSIFICATION OF CHILDHOOD VASCULITIS
Predominantly large vessel vasculitis
Takayasu arteritis
Predominantly medium-sized vessel vasculitis
Childhood Polyarteritis Nodosa
Cutaneous Polyarteritis
Kawasaki Disease
Predominantly small vessel vasculitis
Granulomatous
Wegener granulomatosis
Churg–Strauss syndrome
Nongranulomatous
Microscopic polyangiitis
HSP
Isolated cutaneous leucocytoclastic vasculitis
Hypocomplementic urticarial vasculitis
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Other vasculitides
Behçet’s disease
Vasculitis secondary to infection (including Hepatitis B–associated
PAN), malignancies and drugs, including hypersensitivity
vasculitis
Vasculitis associated with connective tissue diseases
Isolated vasculitis of the central nervous system
Cogan Syndrome
Unclassified
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HENOCH SCHÖNLEIN PURPURA
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HENOCH SCHÖNLEIN PURPURA (HSP)
• This is the most common vasculitis in children
• Incidence of HSP is estimated at 14-20/100,000 children per year
• It is more common in boys
• Approximately 90% of HSP cases occur in children, usually between the
ages of 3 and 10 yr.
• HSP is more common in the winter and spring
• Many cases of HSP follow a documented upper respiratory infection.
Many organisms have been implicated, including streptococci.
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HENOCH SCHÖNLEIN PURPURA (HSP)
DEFINITION
Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood
and is characterized by leukocytoclastic vasculitis and immunoglobulin (Ig) A
deposition in the small vessels in the skin, joints, gastrointestinal tract, and
kidney.
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HENOCH SCHÖNLEIN PURPURA (HSP)
PATHOLOGY
• Skin biopsies demonstrate vasculitis of the dermal
capillaries and postcapillary venules. The inflammatory
infiltrate includes neutrophils and monocytes.
• Renal
histopathology
typically
shows
endocapillary
proliferative glomerulonephritis, ranging from a focal
segmental process to extensive crescentic involvement.
• Immunofluorescence identifies IgA deposition in walls of
small vessels, accompanied to a lesser extent by
deposition of C3, fibrin, and IgM.
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HENOCH SCHÖNLEIN PURPURA (HSP)
PATHOGENESIS
• The exact pathogenesis of HSP remains unknown.
• Given the seasonality of HSP and the frequency of preceding upper
respiratory infections, infectious triggers such as group A βhemolytic streptococcus, Staphylococcus aureus, mycoplasma, and
adenovirus have been suspected.
• The common finding of deposition of IgA, specifically IgA1, suggests
that HSP is a disease mediated by IgA and IgA immune complexes.
• HSP occasionally clusters in families, suggesting a genetic
component.
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HENOCH SCHÖNLEIN PURPURA (HSP)
• The hallmark of HSP is its rash: palpable purpura starting as
pink macules or wheals and developing into Petechiae,
raised purpura, or larger ecchymosis. Occasionally, bullae
and ulcerations develop.
• The skin lesions are usually symmetric and occur in gravitydependent areas (lower extremities) or on pressure points
(buttocks)
• The skin lesions evolve in groups, typically lasting 3-10 days,
and may recur up to 4 months after initial presentation.
Subcutaneous edema localized to the dorsa of hands and
feet, periorbital area, lips, scrotum, or scalp is also common.
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HENOCH SCHÖNLEIN PURPURA (HSP)
• Musculoskeletal involvement - arthritis and arthralgias (75%).
The arthritis tends to be self-limited and oligoarticular,
predilection for the lower extremities, does not lead to
deformities. The arthritis usually resolves within 2 wk but can
recur.
• Gastrointestinal manifestations of HSP (80%),
include
abdominal pain, vomiting, diarrhea, paralytic ileus and
melena; intussusception, mesenteric ischemia, intestinal
perforation are uncommon.
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HENOCH SCHÖNLEIN PURPURA (HSP)
• Renal involvement (50%), manifesting as microscopic hematuria,
proteinuria, hypertension, frank nephritis, nephrotic syndrome, and
acute or chronic renal failure. Progression to end-stage renal disease is
uncommon in children (1-2%)
• Neurologic manifestations of HSP, caused by hypertension or central
nervous system (CNS) vasculitis, may also occur. They include
intracerebral hemorrhage, seizures, headaches, and behavior changes.
• Other less-common potential manifestations of HSP are orchitis, carditis,
inflammatory eye disease, testicular torsion, and pulmonary hemorrhage.
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HENOCH SCHÖNLEIN PURPURA (HSP)
• The diagnosis of HSP is a clinical one and is often straightforward when
the typical rash is present. However, in at least 25% of cases, the rash
appears after other manifestations, making early diagnosis challenging.
• The differential diagnosis for HSP depends on specific organ involvement
other small vessel vasculitides, infections, acute post streptococcal
glomerulonephritis, hemolytic-uremic syndrome, coagulopathies, and
other acute intraabdominal processes.
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HENOCH SCHÖNLEIN PURPURA (HSP)
CLASSIFICATION CRITERIA FOR HSP
Palpable purpura (in absence of coagulopathy or thrombocytopenia) and 1
or more of the following criteria must be present
• Abdominal pain(acute, diffuse, colicky pain)
• Biopsy of affected tissue demonstrating predominant immunoglobulin A
deposition
• Arthritis or arthralgia
• Renal involvement (proteinuria >3 grams/24 hr), hematuria or red cell
casts
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HENOCH SCHÖNLEIN PURPURA (HSP)
• No laboratory finding is diagnostic of HSP.
• Common but nonspecific findings include leukocytosis, thrombocytosis,
mild anemia, and elevations of erythrocyte sedimentation rate (ESR) and
C-reactive protein involvement with blood pressure, urinalysis, and
serum creatinine is necessary.
• Ultrasound - look for bowel wall edema or intussusception.
• Barium enema - diagnose and treat intussusception.
• biopsies of skin and kidney - particularly in atypical or severe cases, and
characteristically show IgA deposition in affected tissues.
• ANA and ANCA are negative.
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HENOCH SCHÖNLEIN PURPURA (HSP)
TREATMENT
• Mild and self-limited HSP is supportive, adequate hydration, nutrition, and
analgesia.
• Steroids - significant gastrointestinal involvement or other life-threatening
manifestations. Prednisone (1 mg/kg/day for 1-2 wk, followed by taper)
reduces abdominal and joint pain but does not alter overall prognosis nor
prevent renal disease.
• Intravenous immune globulin and plasma exchange are sometimes used in
the setting of severe disease.
• Chronic HSP renal disease is managed with a variety of
immunosuppressants,
including
azathioprine,
cyclophosphamide,
cyclosporine, and mycophenolate mofetil.
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HENOCH SCHÖNLEIN PURPURA (HSP)
COMPLICATIONS
• Acutely, serious gastrointestinal involvement such as intestinal
perforation imparts significant morbidity and mortality.
• Renal disease is the major long-term complication(1-2%).
Renal disease can develop up to 6 mo after diagnosis. It is
recommended that children with HSP undergo serial
monitoring of blood pressure and urinalyses for several
months after diagnosis to monitor for the development of
nephritis.
• An infrequent complication of scrotal edema is testicular
torsion, which is quite painful and must be treated promptly.
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HENOCH SCHÖNLEIN PURPURA (HSP)
PROGNOSIS
• Overall, the prognosis for childhood HSP is excellent, and most children
experience an acute, self-limited course lasting on average 4 wk.
• From 15-60% of children with HSP experience 1 or more recurrences,
typically within 4-6 mo of diagnosis.
• The long-term prognosis usually depends upon the severity and duration
of gastrointestinal or renal involvement.
• Chronic renal disease develops in 1-2% of children with HSP, and
approximately 8% of those with HSP nephritis go on to have end-stage
renal disease.
• Rarely, death may occur during the acute phase of the disease as a result
of bowel infarction, CNS involvement, or renal disease.
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