Henoch Schonlein Purpura

Dairion Gatot,

Hematology -Onkology Medic Division

Internal Departement of Medical Faculty of North Sumatera University /
Haji Adam Malik General Hospital,
Medan 2018
 DEFINITION
• Also called “anaphylactoid purpura”
• HSP is a systemic vasculitic syndrome with:
– Palpable purpura
– Arthralgias
– GI involvement
– Glomerulonephritis
 EPIDEMIOLOGY
• 90% of cases reported in children
– Peak in children aged 4-7
• Male:Female (1.5:1)
• 50% follow a URI
• Renal disease is more severe in adults
 PATHOGENESIS
• Likely mechanism thought to be an immune-
complex mediated disease with deposits in
the glomerular capillaries, dermal capillaries
and GI tract.
• Mesangial deposits of IgA are the same as
those seen in IgA nephropathy
 PRECIPITATING ANTIGENS
• INFECTIONS
– URI – C.difficile
– Measles – H.pylori
– Rubella – Adenovirus
– Parvovirus B19 – Legionella
– Mycoplasma – Tuberculosis
– Coxsackie virus – Mumps
– Toxocara – Streptococcus
– Amebiasis – Morganella morganii
– Salmonella
 PRECIPITATING ANTIGENS
• Drugs
– Vancomycin
– Streptokinase
– Ranitidine
– Cefuroxime
– Diclofenac
– Enalapril
– Captopril
 PRECIPITATING ANTIGENS
• Other:
– Food hypersensitivity
– Cold exposure
– Autosomal recessive Chronic granulomatous
disease
– Myelodysplastic syndrome
– Small cell lung cancer
– Breast cancer
 PATHOLOGIC FEATURES

DERMATOLOGIC FINDINGS: Leukocytoclastic

vasculitis with IgA deposition
 CLINICAL FEATURES
• Tetrad of symptoms
– Abdominal pain
– Renal disease
– Palpable purpura
– Arthritis/arthralgias – more common in adults and
most common in knees and ankles. Generally self-
limiting
 CLINICAL FEATURES
• PALPABLE PURPURA: most commonly seen on
lower extremities and buttocks, however can
also been seen on the trunk and arms.
– Lesions begin as erythematous macules and
progress to purpuric, non-blanching, nonpruritic
lesions that may become confluent
 CLINICAL FINDINGS
• GI INVOLVEMENT: more common in children.
Symptoms include abdominal pain, nausea,
vomiting, diarrhea, constipation or bowel
intussusception. May present with GI
bleeding.
 CLINICAL FEATURES
• RENAL INVOLVEMENT:
– in up to 50% of patients
– Usually more rapidly progressive in adults. Rare in
children
– May present with hematuria
– Can have mild glomerulonephritis leading to
microscopic hematuria and can lead to a rapidly
progressive glomerulonephritis with RBC casts
– Usually resolve spontaneously.
 DIAGNOSTIC EVALUATION
• May have mild leukocytosis
• Normal platelet count
• Normal serum complement levels
• Elevated IgA in 50%
 DIAGNOSIS
• Generally a clinical diagnosis
• Skin Biopsy: can be helpful and used to
confirm IgA and C3 deposits and
leukocytoclastic vasculitis.
• Renal Biopsy: not usually needed for
diagnosis. Will show mesangial IgA deposits
and segmental glomerulonephritis
 MANAGEMENT
• Usually self-limiting (1-6 weeks)
• Steroids:
– may decrease tissue edema, may aid in arthralgias
and some abdominal pain
– Has not been shown to be beneficial in kidney
disease or dermal manifestations
– Does not lessen chance of recurrence
– Does not shorten duration of disease
 MANAGEMENT
• if rapidly progressive glomerulonephritis
– Multidrug regimens with cytotoxic drugs however
not many reports with treatment in adults.
– Plasmaphoresis
– IVIG
• Symptomatic management of GI symptoms
and surgical intervention if warranted.
 PROGNOSIS
• Prognostic factors:
– generally a milder course in children with shorter
duration and fewer recurrences
– Proteinuria >1gm/day with worse prognosis if
develop nephrotic syndrome
• 1-5% children progress to ESRD
• Recurrence in up to 40% of patients
Terima Kasih