Henoch-Schönlein purpura (HSP)
Ahmed Abdul Ghany
BACKGROUND1st described in 1801 by William Heberden, a physician in london, who wrote about a case of a 5 year old boy with hematuria, abdominal pain, joint pains and skin rash.
EPIDEMIOLOGYHSP (IgAV) is a systemic vasculitic syndrome seen primarily in children.Male –to- female ratio: 1.8: 1
PATHOGENESISImmunoglobulin A deposition
CLINICAL MANIFESTATIONS
Joints
Abdominal painRenal
Palpable Purpura
Palpable Purpura:
SymmetricalDependent areas
Arthralgia/ arthritis:
2nd most common presentation 84%Usually transient or migratoryOligoarticularNondeformingLower extremity large joints
Abdominal Pain:
50% of patients complain of colicky pain typically develop within 8 days of the appearance of rash.GI bleeding in 20 – 30 %Inussusception is a common complication in children.
Renal disease:
Ranges from 21-54 %Hematuria with or without red cell cast.Proteinuria ranges from mild to nephrotic range.Elevated creatinine and/ or HTN.
Other organs:
CNS including intracerebral hemorrhage.Pulmonary hemorrhageKeratits and uveitis
DIAGNOSIS
Lab. Serum IgA(50-70%)
Abdominal U/S
Biopsy .
Renal biopsy is reserved for patients in whom the diagnosis is uncertain or evidence of sever renal impairmentSkin biopsy including small blood vessels of superficial dermis
Differential diagnosisDD
Purpura
Hypersenstivity vacsulitis
Other small vsvasculitis
SLE
infections
Arthritis
Autoimmune
Septic arthritis
Renal Abdominal Pain
ManagementAdmission is warranted for the following:• Sever abdominal pain• GI bleeding• Elevated creatinine, HTN, and/ or nephrotic • Sever joint involvement• Changes in mental status
Supportive care:
• Includes adequate hydration, rest and pain relief.
Symptomatic therapy:
NSAIDs:• Naproxen 10 – 20 mg/kg• Ibuprofin and other NSAIDs are equally
effective
Glucocorticoids• Their use in patients with HSP is controversial• Prednisone 1- 2 mg /kg daily (max 80 mg)• To be used only in patients with symptoms
sever enough to affect oral intake or daily activities.
Disease modifying agents:
• Targeted toward preventing or ameliorating GI and renal complications.
• Limited data suggest that cyclophosphamide and cyclosporine may be beneficial.
• Plasmapharesis has been used in patients with crescentic disease and rapidly progressive renal failure.
THANK YOU
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