Transcript Henoch_Scholein_Purpura

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Summary
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Epidemiology
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Etiology and Pathogenesis
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Clinical Manifestations
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Diagnosis
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Treatment
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Most common systemic vasculitis in children
 90% of cases occur in the pediatric group
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Etiology: Unknown
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Pathogenesis: End organ IgA immune complex (IC)
deposition
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Complications: Renal
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Clinical Diagnosis:
 Palpable purpura without thrombocytopenia
 Abdominal pain
 Renal disease
 Arthritis
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Treatment: Disease usually self limited and treatment is
usually symptomatic
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Disease of early childhood ages 3-15
 20 per 100,000 in UK children < 17 years-old
 70 per 100,000 in UK children 4-6 years-old
 No comparable data in adults, less common
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Male : Female ratio 1.2- 1.8: 1
Arthritis Rheum 1997 May;40(5):859-64
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Less common in African American children
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More severe course in adults
 More frequent and severe renal disease 
requirement for more aggressive treatment
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Seasonal variance occurs in the fall, winter
and spring and is rare in summer
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Unknown etiology!
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Precipitating antigen may be infectious
 Many cases follow upper respiratory infection (URI)
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Example: identical twins following adenovirus
infection:
 HSP in one, IgA nephropathy in other
J Pediatr 1985 Jan;106(1):27-32.
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Immune-complex (IC) mediated disease associated with
Immunoglobin A (IgA) deposition
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Characteristic findings is leukocytoclastic vasculitis
(LCV)of post capillary venules
 Accompanied by IgA deposition within affected organs
 IC of IgA1 is the ONLY subtype
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Immune complexes activate complement (alternative)
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Hinge region O-linked glycans of IgA1 are
deficient in galactose and/or sialic acid
content
 Renal mesangial cells bind galactose/sialic acid deficient
hinge regions
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Berger’s disease (IgA nephropathy) also involves
IgA1 exclusively
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Classic Tetrad: May develop over days to weeks
 Rash (100%)
 Arthralgias (82%)
 Abdominal pain (63%)
▪ GI bleeding (33%)
 Renal disease (40%)
Medicine (Baltimore) 1999 Nov;78(6):395-409.
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Presenting feature by %
 Rash 74%
 Arthralgias 15%
 Abdominal pain 12%
ACR 1990
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Palpable Purpura
Age at onset < 20 years
Acute abdominal pain
Biopsy:
EULAR / PRES 2005
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Palpable purpura without
coagulopathy or
decreased platelets
AND
 Granulocytes in walls of
small arterioles / venules
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> or = 2 criteria
 90% Sensitivity/Specificity
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Diffuse abdominal pain
Arthritis or arthralgia
Bopsy with IgA deposition
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Erythematous, macular or urticarial wheals 
petechia/palpable purpura
 NORMAL clotting studies and platelets
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Appears in crops
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Symmetric distribution
 Gravity/pressure dependent areas such as lower
extremities
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Localized subcutaneous edema in dependent and
periorbital areas seen in children < 3 years old
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Differential diagnosis of palpable purpura:
 Mixed Cryoglobulinemia
 Hypersensitivity vasculitis
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Occur in up to 84% of patients
 Uncommon sole presenting symptom at presentation
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Transient, migratory oligoarthritis (1-4 joints)
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Lower extremities > upper extremity joints
 More common in hips, knees and ankles
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Non-destructive arthritis (no chronic damage)
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Prominent periarticular swelling with no synovitis
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Significant pain + limited range of motion
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Differential diagnosis (DDx):
 Juvenile Idiopathic arthritis (JIA)
 Rheumatoid arthritis (RA)
 Systemic Lupus Erytematous (SLE)
MILD SYMPTOMS
SEVERE SYMPTOMS
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Nausea / vomiting
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GI bleed
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Abdominal pain
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Bowel ischemia and
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Transient paralytic leus
necrosis
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Intussusception
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Bowel perforation
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Develops typically within 8 days of the rash
 GI symptoms precede the rash in 15-35% of cases
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Abdominal pain = submucosal hemorrhage + edema
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Guaiac + stool is seen in 56% of patient
 Massive GI hemorrhage is rare
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Purpuric lesions may be seen on endoscopy
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Most common gastrointestinal complication of HSP
 Incidence of 3.5%
 Edema and hemorrhage contributes to development
 Limited to small bowel in 60% of cases
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Initial screening test: Ultrasound
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Differential diagnosis: Appendicitis
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Occurs in 20-54 % of children (more prevalent in adults)
 2 days to 4 weeks after onset of systemic symptoms
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Hematuria is most common presentation
 With or without red blood cell (RBC) casts
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Nephrotic range proteinuria, ↑ creatinine and or
hypertension  ↑ risk of progressive disease (adults)
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% of glomeruli with crescents has prognostic significance
 > 50%
 37% progressed to end stage renal disease (ESRD)
 18% with chronic renal insufficiency (CRI)
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Differential diagnosis:
 Berger’s Disease
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Correlation between disease severity and biopsy
findings
 Asymptomatic hematuria: focal mesangial proliferation
 Proteinuria: cellular proliferation
 Nephrotic range proteinuria: crescents
J Am Soc Nephrol 1999 Dec;10(12):2637-44
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2-38% involvement
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Presentation mimics testicular torsion
 Pain, tenderness and swelling
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Evaluate with ultrasound or radionuclide scanning
 Testicular torsion will show ↓ blood flow versus normal
blood flow in HSP
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Similar manifestations as in children
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Two main differences between adults and children:
 Intussusception is rare in adults
 Adults have ↑ risk of developing significant renal
involvement including end-stage renal disease
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Clinical manifestations (know the Tetrad)
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Classification criteria
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Gold Standard is biopsy
 Demonstration of LCV with IgA deposition = HSP
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Biopsy obtained in children:
 Unusual presentation or significant renal disease
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Biopsy in adults:
 Due to lower incidence, confirmation by biopsy is
more important
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Superficial dermis biopsy sufficient
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Goal to obtain skin lesions less than 24 hour-old
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Leukocytoclastic vasculitis in post capillary venules
with IgA deposition = pathognomonic of HSP
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Obtain in severe renal involvement or uncertain
diagnosis
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Characterized by IgA deposition in mesangium
 Immunoflorence studies  IgG, fibrin, C3
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Mesangial proliferation to crescentic
glomerulonephritis (GN)
 Biopsy generally parallels clinical disease severity
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No lab test is diagnostic for HSP
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Labs obtained tend to be non-specific
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Normal coagulation studies and platelets
 This differentiates HSP from other diseases
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Need to obtain renal function and urinalysis at a
diagnosis
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Obtain in patients with significant abdominal
symptoms
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Plain abdominal X-ray:
 May demonstrate dilated bowel loops
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Ultrasound:
 ↑ bowel wall thickness, hematomas and intussusception
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Complete recovery 94% children, 89% of adults
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Supportive treatment in vast majority
 Rest
 Hydration
 NSAIDS
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Hospitalization indicated:
 Inability to maintain adequate hydration with oral intake
 Severe abdominal pain
 Significant gastrointestinal bleeding
 Changes in mental status
 Severe joint involvement limiting ambulation
 Renal insufficiency (↑ Creatinine), HTN, nephrotic syndrome
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Use is controversial
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Benefits include:
 Shortened duration of abdominal pain
 ↓ risk of intussusception
 ↓ risk of recurrence
 ↓ risk of renal involvement
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Results of benefit mixed in studies
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Absence of definitive evidence of long term benefits
 Only used in patients with severe symptoms requiring
hospitalization
 Prednisone 1-2 mg/kg/day, max dose of 60-80 mg/day
 Inflammation will be improved but the pathophysiology of
the disease does not appear to be affected
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Gastrointestinal
 Corticosteroids not proven to decrease risk of
intussusception
 Using corticosteroids after intussusception has occurred
may obscure the signs of compromised bowel viability
Medicine (Baltimore) 1999 Nov;78(6):395-409.
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Should only be considered in patients with:
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Marked proteinuria and/or impaired renal function
Therapies for cresentic nephritis:
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High dose methylprednisolone  oral prednisone for 3
months has shown benefit
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Azathioprine and corticosteroids
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Cyclophosphamide
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Recurrence occurs in 1/3rd of children
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Occurs within 4 months of initial episode
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Usually milder and briefer and occur in patients:
 Nephritis
 Evidence of acute inflammation
 Patients who received corticosteroids
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Most common systemic vasculitis in children
 90% of cases occur in the pediatric group

Etiology: Unknown
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Pathogenesis: End organ IgA immune complex (IC)
deposition

Complications: Renal, GI
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Clinical Diagnosis:
 Palpable purpura without thrombocytopenia
 Abdominal pain
 Renal disease
 Arthritis
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Treatment: Typically symptomatic and self limited
 Unclear role of corticosteroids in TX
 No specific agent proven efficacious for persistent renal disease
Niaudet P, et al. Renal manifestations of HenochSchönlein purpura. Uptodate 2012.
 Dedeoglu F, et al. Clinical manifestations and
diagnosis of Henoch-Schönlein purpura. Uptodate
2012.
 Dedeoglu F, et al. Management of Henoch-Schönlein
purpura. Uptodate 2012.
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