henoch-schonlein purpura discussion

8/2/2019 Henoch-Schonlein Purpura Discussion

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HENOCH-SCHONLEIN PURPURA

named after Eduard Heinrich Henoch(18201910), a German pediatrician,and his teacher.. Johann Lukas

Schnlein (17931864), who describedit in the 1860s


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A.K. A.

Anaphylactoid Purpura

Purpura rheumatica

systemic small-vessel vasculitis characterizedby deposition of immune complexes containing the

antibody IgA


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Classification

2006 European League Against Rheumatism(EULAR) and Pediatric Rheumatology Society(PReS) classification:

palpable purpura, together with at least 1 of the ff:

diffuse abdominal pain

predominant IgA deposition (biopsy)

acute arthritis in any joint

renal involvment (hematuria, proteinuria)


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Presentation

Classic of HSP:

purpura (hallmark)

arthritis (80%)abdominal pain (62%)

low grade fever and fatigue (more than ofaffected children)


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Rash

pinkish maculopapules that initiallyblanch on pressure

petechia/purpura

(characterized as palpable purpura that evolve

from red to purple to rusty brown before theyeventually fade)


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Rash

Purpura typically appear on the legs andbuttocks, but may also be seen on the arms,face, and trunk

Lesions tend to occur in crops, last from 3-10days


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Arthritis

Usually localized to the knees and ankles

Non-erosive and hence causes no permanentdeformity


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Abdominal pain

Colicky in character, and may beaccompanied by nausea, vomiting,constipation, or diarrhea

There may be blood or mucus in the stools


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Other manifestations

25-50% of cases have evidence of kidneyinvolvement mainly in the form of hematuria

Rare CNS involvement


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Pathophysiology

Complexes of IgA and C3 are deposited onarterioles, capillaries, and venules


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Pathophysiology

PREFERENTIAL

INVOLVEMENT

SKIN JOINTS GIT

PURPURA JOINT PAIN ABDOMINAL PAIN


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HSP can develop after infections withstreptococci (-haemolytic, Lancefield groupA), hepatitis B, herpes simplex virus,parvovirus B19, Coxsackievirus, adenovirus,Helicobacter pylori,measles, mumps, rubella,mycoplasma and numerous others
http://en.wikipedia.org/wiki/Streptococcushttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Hepatitis_Bhttp://en.wikipedia.org/wiki/Herpes_simplex_virushttp://en.wikipedia.org/wiki/Parvovirus_B19http://en.wikipedia.org/wiki/Coxsackievirushttp://en.wikipedia.org/wiki/Adenovirushttp://en.wikipedia.org/wiki/Helicobacter_pylorihttp://en.wikipedia.org/wiki/Measleshttp://en.wikipedia.org/wiki/Mumpshttp://en.wikipedia.org/wiki/Rubellahttp://en.wikipedia.org/wiki/Mycoplasmahttp://en.wikipedia.org/wiki/Mycoplasmahttp://en.wikipedia.org/wiki/Rubellahttp://en.wikipedia.org/wiki/Mumpshttp://en.wikipedia.org/wiki/Measleshttp://en.wikipedia.org/wiki/Helicobacter_pylorihttp://en.wikipedia.org/wiki/Adenovirushttp://en.wikipedia.org/wiki/Coxsackievirushttp://en.wikipedia.org/wiki/Parvovirus_B19http://en.wikipedia.org/wiki/Herpes_simplex_virushttp://en.wikipedia.org/wiki/Hepatitis_Bhttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus


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Diagnosis

Lab tests neither specific nor diagnostic Blood tests may show:

elevated creatinine and urea levels (in kidneyinvolvement)

raised IgA levels (in about 50%)

and raised C-reactive protein (CRP) orerythrocyte sedimentation rate (ESR) results


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Platelet count may be raised, anddistinguishes it from diseases where lowplatelets are the cause of the purpura

Renal involvement is manifested by RBC,WBC, casts, or albumin in the urine

Diagnosis


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DEFINITIVE DIAGNOSIS of vasculitis:

confirmed by biopsy

Diagnosis


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Management

Symptomatic treatment

adequate hydration, pain control withacetaminophen

Therapy with oral or IV corticosteroids (1-2mg/kg/day)

Alternate-day colchicine (0.6 mg/24hr everyother day) for rheumatoid nodules


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Complications

nephrotic syndrome

bowel perforation


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Prognosis

HSP is a self-limited vasculitic disease with anexcellent overall prognosis (spontaneousrecovery in 94% of children )

In children under 10, the condition recurs inabout 1/3 of cases and usually within 1st

4months after the initial attack


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HSP is systemic small vessel vasculitis

characterized by deposition of immunecomplexes containing the antibody IgA

It occurs mainly in young children and teens

Typical symptoms include palpable purpura,joint pains, and abdominal pain


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Most cases are self-limiting, but the disease

may relapse in a third of cases

The exact cause of HenochSchnlein

purpura is unknown, although it may occurafter certain viral and bacterial infections, aswell as an adverse drug reaction to somemedications


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THANK YOU!

henoch-schonlein purpura discussion

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