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Henoch-Schnlein Purpura

Morning Report

Chris Williams

November 6, 2007

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Clinical Presentation Classic Tetrad:

Palpable purpura without thrombocytopenia or coagulopathy

Arthritis/arthralgia (~50%) Abdominal pain (~50%) +/- hematochezia

Renal Disease (~30-50%)

Other organ systems can be involved including therespiratory tract and CNS. Rare myocardial and scrotalinvolvement.

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Skin Findings

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Epidemiology Peak incidence in children ages 4-7. 10% of cases occur

in adults

14 cases per 100,000 Male predominance with 1.5-2:1 ratio

Spring, Fall & Winter

Caucasian > Asian > African American

Often preceded by URI particularly strep Other triggers include parasitic infections, drugs, toxins,

chronic systemic diseases and malignancy

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Associated Infections/Vaccinations Group A strep

Mycoplasma

Yersinia

Campylobacter

Meningococcus

HSV

Cosackievirus

Adenovirus Parvo B19

VZV

EBV

Hepatitis B

Typhoid

Measles

Cholera

Yellow fever

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Drug & toxin associations Ampicillin

Penicillin

Erythromycin Quinines

Chlorpromazine

Salicylates

Azo dyes

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Pathophysiology Leukoclastic vasculitis with IgA immune complexes mostly

seen in postcapillary venules

Immunofluoresence demonstrates IgA, C3 and fibrindeposition within the walls of involved vessels, endothelialand mesangial cells of the kidney

A proposed mechanism is polymerisation of IgA1 due to

lysis of the sialic acid attached to the AA in the hingeregion between the complement fixing regions byneuraminidase

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Differential Diagnosis ID

Sepsis, meningococcal mengingitis, septic arthritis, toxic synovitis,endocarditis, RMSF

Rheumatologic:

SLE, Wegeners granulomatosis, microscopic polyangitis,cryoglobulinemia, Churg-Strauss syndrome, idiopathicthrombocytopenic purpura, hypersensitivity vasculitis, polyarteritis

nodosa, rheumatic fever, rheumatoid arthritis GI

Acute abdomen including: appendicitis, bowel perforation,intussussception, volvulus, celiac sprue, mesenteric ischemia [Acuteabdomen]

Renal IgA nephropathy (Bergers disease)

Malignancy

leukemia

Drug or toxin reaction

Testicular Torsion

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Diagnostic Testing

Usually a clinical diagnosis

Normal platelets and coags

UA: blood, protein, casts

Serum complement levels normal Antibody negative (ANA, dsDNA, ANCA)

ANCA negative C-ANCA = Wegeners

P-ANCA = microscopic polyangitis or Churg Strausssyndrome

ASO may be elevated

Serum IgA elevated in 50%

Often concomitant leukocytosis and incESR

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Diagnosis: Skin Biopsy

Leukocytoclastic

vasculitis in

postcapillary venules

with IgA deposition is

pathognomonic

Skin lesions should be

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Diagnosis: Skin Biopsy

IgA and C3 positive immunofluoresence.

Qui iI ( r ) r r

r t t i i tur .

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Renal Biopsy

R

enal involvement occurs in about 33% ofchildren and 63% of adults

Segmentary focal glomerulonephritis, alwaysassociated with granulous IgA deposits in themesangium is typical but there is a wide variety

of pathology seen dependent on severity

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Risk of renal progression

Severity of other organ involvement is NOT prognostic forrenal disease

Age and mean proteinuria during follow-up are

independent prognostic predictors Proteinuria, HTN and CRI at baseline is not significantly

related to renal progression

One study demonstrated that lab studies at onset and atrenal biopsy (renal function impairment, hypertension,

nephrotic-range proteinuira) were not significantly relatedwith renal detrimental progression

However, a different study showed that if proteinuria > 1g/d and/or renal failure was present, the risk of developingchronic renal failure was 28% in adults

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Treatment Self Limited

Supportive therapy includes bedrest, adequate hydration, compressionstockings and NSAIDs (used cautiously)

Prednisone in a dose of 1 mg/kg/d for 2 weeks and then tapered over 2

more weeks has been shown to improve gastrointestinal and jointsymptoms. Although this regimen did not decrease the incidence of renaldisease, it did lessen the severity of nephritis in some patients in an RCTof 171 pediatric patients

Immunosuppressive drugs (e.g. cyclophosphamide, azathioprine anddapsone) have been used in more severe cases or for recurrence

Ace-inhibitors may be helpful in decreasing proteinuria in chronic renal dz Plasma exchange and polyclonal immunoglobulin therapy have been used

in life threatening cases

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Prognosis Generally good prognosis

Around 10-30% of adult patients will have some degree of

renal insufficiency on follow-up (CrCl

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Doggie Bag: HSPConsider dx with purpura w/o thrombocytopenia or

coagulopathy

American College of Rheumatology (2 of 4) Age

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References Coppo R, DAmico G. Factors predicting progression of IgA nephropathies. J Nephrol. 2005

Sep-Oct; 18(5):503-12.

Ly M, Breza T. Henoch-Schonlein purpura in an adult. SKINmed: Dermatology for theClinician. 2003;2(4):262-264.

PertuisetE, Liote F, et al. Adult Henoch-Schonlein purpura associated with malignancy.

Semin Arthritis Rheum. 2000 Jun;29(6):360-7.

PilleboutE, ThervetE, et al. Henoch-Schonlein purpura in adults: outcome and prognosticfactors. J Am Soc Nephrol. 2002 May;13(5):1271-8.

Rieu P, Noel LH. Henoch-Schonlein nephritis in children and adults. Morpholgical featuresand clinicopathological correlations. Ann Med Interne. 1999 Feb; 150(2):151-9.

Ronkainen J, Koskimies O, et al. Early prednisone therapy in Henoch-Schonlein purpura: a

randomizd, double-blind, placebo-controlled trial. J Pediatr. 2006 Aug;149(2):241-7. Saulsbury FT. Corticosteroid therapy does not prevent nephritis in Henoch Schonlein

Purpura. Pediatric Nephrol.1993 Feb;7(1):69-71.

Szer IS. Henoch-Schonlein purpura: when and how to treat.JRheumatol. Sep 1996;23(9):1661-5.

UNC Kidney Center. IgA Nephropathy. Internet. Accessed 10/30/07. Available:

http://www.unckidneycenter.org/patients/iga_nephropathy.htm