henoch-schönlein purpura (hsp)

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.

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