HENOCH-SCH?NLEIN PURPURA

HENOCH-SCHÖNLEIN PURPURAHENOCH-SCHÖNLEIN PURPURA

Morning Report Morning Report

July 6, 2007July 6, 2007Sima Patel, MDSima Patel, MD

DEFINITIONDEFINITION

Also called “anaphylactoid purpura”Also called “anaphylactoid purpura” HSP is a systemic vasculitic syndrome HSP is a systemic vasculitic syndrome

with:with:– Palpable purpuraPalpable purpura– ArthralgiasArthralgias– GI involvementGI involvement– GlomerulonephritisGlomerulonephritis

BACKGROUNDBACKGROUND

First described in 1801 by William First described in 1801 by William Heberden, a physician in London, who Heberden, a physician in London, who wrote about a case of a 5 year old boy wrote about a case of a 5 year old boy with hematuria, abdominal pain, joint with hematuria, abdominal pain, joint pains and a skin rash.pains and a skin rash.

In 1837, Johann Schönlein and later in In 1837, Johann Schönlein and later in 1874, Edouard Henoch described multiple 1874, Edouard Henoch described multiple case reports of similar cases. They also case reports of similar cases. They also showed an association of an upper showed an association of an upper respiratory infection preceding respiratory infection preceding development of symptoms. development of symptoms.

EPIDEMIOLOGYEPIDEMIOLOGY

90% of cases reported in children90% of cases reported in children– Peak in children aged 4-7Peak in children aged 4-7

Male:Female (1.5:1) Male:Female (1.5:1) 50% follow a URI50% follow a URI Renal disease is more severe in adultsRenal disease is more severe in adults

PATHOGENESISPATHOGENESIS

Likely mechanism thought to be an Likely mechanism thought to be an immune-complex mediated disease with immune-complex mediated disease with deposits in the glomerular capillaries, deposits in the glomerular capillaries, dermal capillaries and GI tract.dermal capillaries and GI tract.

Mesangial deposits of IgA are the same as Mesangial deposits of IgA are the same as those seen in IgA nephropathy those seen in IgA nephropathy

PRECIPITATING ANTIGENSPRECIPITATING ANTIGENS

INFECTIONSINFECTIONS– URIURI– MeaslesMeasles– RubellaRubella– Parvovirus B19Parvovirus B19– MycoplasmaMycoplasma– Coxsackie virusCoxsackie virus– ToxocaraToxocara– AmebiasisAmebiasis– SalmonellaSalmonella

– C.difficile– H.pylori– Adenovirus– Legionella– Tuberculosis– Mumps– Streptococcus– Morganella morganii


DrugsDrugs– VancomycinVancomycin– StreptokinaseStreptokinase– RanitidineRanitidine– CefuroximeCefuroxime– DiclofenacDiclofenac– EnalaprilEnalapril– CaptoprilCaptopril


Other:Other:– Food hypersensitivityFood hypersensitivity– Cold exposureCold exposure– Autosomal recessive Chronic granulomatous Autosomal recessive Chronic granulomatous

diseasedisease– Myelodysplastic syndromeMyelodysplastic syndrome– Small cell lung cancerSmall cell lung cancer– Breast cancerBreast cancer

PATHOLOGIC FEATURESPATHOLOGIC FEATURES

DERMATOLOGIC FINDINGS: DERMATOLOGIC FINDINGS: Leukocytoclastic vasculitis with IgA Leukocytoclastic vasculitis with IgA depositiondeposition

Direct Immunofluorescence of skin biopsy. Granular IgA and C3 staining of cutaneous vasculature.

http://www.medscape.com/viewarticle/459714

H & E stain of skin biopsy showing leukocytoclastic vasculitis with infiltration of neutrophils.

http://www.medscape.com/viewarticle/459714

Skin biopsy: Leukocytoclastic vasculitis with mononuclear and polymorphonuclear cell infiltrates in the perivascular space

www.kjronline.org/abstract/view_articletext.asp?year=2004&page=178www.kjronline.org/abstract/view_articletext.asp?year=2004&page=178

PATHOLOGIC FEATURESPATHOLOGIC FEATURES

RENAL FINDINGS: Granular deposits of IgA, RENAL FINDINGS: Granular deposits of IgA, mesangioproliferative glomerulonephritis mesangioproliferative glomerulonephritis and crescent formation and crescent formation

Renal biopsy: sclerosis and fibrous crescents in the glomerulus.

http://www.ndt-educational.org/nagycase.asp

http://www.ndt-educational.org/nagycase.asp

Immunofluorescence: Glomerular deposits of IgAImmunofluorescence: Glomerular deposits of IgA

CLINICAL FEATURESCLINICAL FEATURES

Tetrad of symptomsTetrad of symptoms– Abdominal painAbdominal pain– Renal diseaseRenal disease– Palpable purpuraPalpable purpura– Arthritis/arthralgias – more common in adults Arthritis/arthralgias – more common in adults

and most common in knees and ankles. and most common in knees and ankles. Generally self-limitingGenerally self-limiting


PALPABLE PURPURA: most commonly PALPABLE PURPURA: most commonly seen on lower extremities and buttocks, seen on lower extremities and buttocks, however can also been seen on the trunk however can also been seen on the trunk and arms. and arms. – Lesions begin as erythematous macules and Lesions begin as erythematous macules and

progress to purpuric, non-blanching, progress to purpuric, non-blanching, nonpruritic lesions that may become confluentnonpruritic lesions that may become confluent

CLINICAL FINDINGSCLINICAL FINDINGS

GI INVOLVEMENT: more common in GI INVOLVEMENT: more common in children. Symptoms include abdominal children. Symptoms include abdominal pain, nausea, vomiting, diarrhea, pain, nausea, vomiting, diarrhea, constipation or bowel intussusception. constipation or bowel intussusception. May present with GI bleeding. May present with GI bleeding.


RENAL INVOLVEMENT: RENAL INVOLVEMENT: – in up to 50% of patientsin up to 50% of patients– Usually more rapidly progressive in adults. Usually more rapidly progressive in adults.

Rare in childrenRare in children– May present with hematuriaMay present with hematuria– Can have mild glomerulonephritis leading to Can have mild glomerulonephritis leading to

microscopic hematuria and can lead to a microscopic hematuria and can lead to a rapidly progressive glomerulonephritis with rapidly progressive glomerulonephritis with RBC castsRBC casts

– Usually resolve spontaneously. Usually resolve spontaneously.

DIAGNOSTIC EVALUATIONDIAGNOSTIC EVALUATION

May have mild leukocytosisMay have mild leukocytosis Normal platelet countNormal platelet count Normal serum complement levelsNormal serum complement levels Elevated IgA in 50%Elevated IgA in 50%

DIAGNOSISDIAGNOSIS

Generally a clinical diagnosisGenerally a clinical diagnosis Skin Biopsy: can be helpful and used to Skin Biopsy: can be helpful and used to

confirm IgA and C3 deposits and confirm IgA and C3 deposits and leukocytoclastic vasculitis. leukocytoclastic vasculitis.

Renal Biopsy: not usually needed for Renal Biopsy: not usually needed for diagnosis. Will show mesangial IgA diagnosis. Will show mesangial IgA deposits and segmental glomerulonephritisdeposits and segmental glomerulonephritis

MANAGEMENTMANAGEMENT

Usually self-limiting (1-6 weeks)Usually self-limiting (1-6 weeks) Steroids: Steroids:

– may decrease tissue edema, may aid in may decrease tissue edema, may aid in arthralgias and some abdominal painarthralgias and some abdominal pain

– Has not been shown to be beneficial in kidney Has not been shown to be beneficial in kidney disease or dermal manifestationsdisease or dermal manifestations

– Does not lessen chance of recurrenceDoes not lessen chance of recurrence– Does not shorten duration of diseaseDoes not shorten duration of disease

MANAGEMENTMANAGEMENT

if rapidly progressive glomerulonephritisif rapidly progressive glomerulonephritis– Multidrug regimens with cytotoxic drugs Multidrug regimens with cytotoxic drugs

however not many reports with treatment in however not many reports with treatment in adults.adults.

– PlasmaphoresisPlasmaphoresis– IVIGIVIG

Symptomatic management of GI Symptomatic management of GI symptoms and surgical intervention if symptoms and surgical intervention if warranted. warranted.

PROGNOSISPROGNOSIS

Prognostic factors: Prognostic factors: – generally a milder course in children with generally a milder course in children with

shorter duration and fewer recurrences shorter duration and fewer recurrences – Proteinuria >1gm/day with worse prognosis if Proteinuria >1gm/day with worse prognosis if

develop nephrotic syndromedevelop nephrotic syndrome 1-5% children progress to ESRD1-5% children progress to ESRD Recurrence in up to 40% of patientsRecurrence in up to 40% of patients

REFERENCESREFERENCES

Kasper, Dennis, and Eugene Braunwald, 16th edition, Kasper, Dennis, and Eugene Braunwald, 16th edition, eds. Harrison’s Principles of Internal Medicine. New eds. Harrison’s Principles of Internal Medicine. New York: McGraw-Hill, 2005.York: McGraw-Hill, 2005.

Tierney Jr, Lawrence, and Stephen McPhee, 45th Tierney Jr, Lawrence, and Stephen McPhee, 45th edition, eds. Current Medical Diagnosis and Treatment. edition, eds. Current Medical Diagnosis and Treatment. New York: McGraw-Hill, 2006.New York: McGraw-Hill, 2006.

Uptodate: Clinical manifestations and diagnosis of Uptodate: Clinical manifestations and diagnosis of Henoch-Schonlein PurpuraHenoch-Schonlein Purpura

Anup Rai, et al., Henoch-Schonlein Purpura Nephritis. Anup Rai, et al., Henoch-Schonlein Purpura Nephritis. American Society of Nephrology. Volume 10, pages American Society of Nephrology. Volume 10, pages 2637-2644, 19992637-2644, 1999

Espositio et al., Henoch-Schonlein Purpura in Chronic Espositio et al., Henoch-Schonlein Purpura in Chronic Hemodialys patients. Journal of Nephrology. Volume 12: Hemodialys patients. Journal of Nephrology. Volume 12: 197-200, 1999197-200, 1999

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HENOCH-SCH?NLEIN PURPURA