HENOCHHENOCH––SCHONLEINSCHONLEINPURPURAPURPURA
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M. Sjabaroeddin Loebis, Lily Irsa, Rita Evalina
Allergy Immunology Division
Pediatrics Departement
Medical Faculty Sumatera Utara University
�� INTRODUCTIONINTRODUCTIONHENOCH SCHONLEIN PURPURA / ANAPHYLACTOID HENOCH SCHONLEIN PURPURA / ANAPHYLACTOID PURPURAE / NON TROMBOSITOPHENIC PURPURAEPURPURAE / NON TROMBOSITOPHENIC PURPURAE
→ VASCULITIS DISEASE OF THE SMALL VESSELS→ VASCULITIS DISEASE OF THE SMALL VESSELS
→ SKIN, JOINTS, GI TRACT AND KIDNEY→ SKIN, JOINTS, GI TRACT AND KIDNEY
→ → PALPABLE PURPURAPALPABLE PURPURA, ARTHRITIS/ARTHRALGIA, , ARTHRITIS/ARTHRALGIA,
DIFFUSE ABDOMINAL PAIN, NEPHRITIS or HEMATURIADIFFUSE ABDOMINAL PAIN, NEPHRITIS or HEMATURIA
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DIFFUSE ABDOMINAL PAIN, NEPHRITIS or HEMATURIADIFFUSE ABDOMINAL PAIN, NEPHRITIS or HEMATURIA
��HISTORYHISTORYWILLAN AND HEBERDEN (1806) WILLAN AND HEBERDEN (1806) �������� PALPABLE PURPURA PALPABLE PURPURA DUE TO VASCULITISDUE TO VASCULITIS
J.SCHONLEIN (1837) J.SCHONLEIN (1837) �������� + JOINTS PAIN+ JOINTS PAIN
E. HENOCH (1874) E. HENOCH (1874) ��������+ NEPHRITIS AND GI BLEEDING+ NEPHRITIS AND GI BLEEDING
�� INCIDENCEINCIDENCE-- USA : 14 USA : 14 –– 15 CASES/100.00015 CASES/100.000
-- ENGLAND : 20,4 CASES/100.000ENGLAND : 20,4 CASES/100.000
-- NORWAY : 3,3 CASES/100.000NORWAY : 3,3 CASES/100.000
-- INDONESIAINDONESIA
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-- INDONESIAINDONESIA
RSCM 1998 RSCM 1998 -- 2003 : 23 CASES2003 : 23 CASES
RSWS MAKASAR 1996RSWS MAKASAR 1996--2000: 4 CASES2000: 4 CASES
�� AFFECTS ALL AGES, 2 AFFECTS ALL AGES, 2 –– 15 YEARS OLD, PEAK : 15 YEARS OLD, PEAK : 44--7 YO, MALE : FEMALE = 1,5 : 17 YO, MALE : FEMALE = 1,5 : 1
ETIOLOGY
� Still unknown
� Genetic factor, UTRI, food,immunization,
medicine
� After treatment by antirheumatic, MTX, � After treatment by antirheumatic, MTX,
anti-TNF
� Increase IgA serum concentration,
immune compleks, and IgA deposit in
vessel walls / mesangial renal �
important role
PATOFISIOLOGY
� Renal / skin biopsi : immune deposit compleks
(contains IgA)
� Complement activation (alternative pathway)
� Mediator inflamation activation (vascular
prostaglandin) � small vascular inflamation in prostaglandin) � small vascular inflamation in
the skin, renal, joint and abdominal � skin
purpura, nephritis, arthritis and GIT bleeding
� Histologis : vasculitis leukocitoclastic
CLINICAL MANIFESTATIONCLINICAL MANIFESTATION�� PALPABLE PURPURAPALPABLE PURPURA (>75% OF CASES), (>75% OF CASES),
ARTHRALGIA OR ARTHRITIS, DIFFUSEARTHRALGIA OR ARTHRITIS, DIFFUSE
ABDOMINAL PAIN, GI BLEEDINGABDOMINAL PAIN, GI BLEEDING
�� KIDNEY DAMAGE → VARIED IN KIDNEY DAMAGE → VARIED IN
INCIDENCE AND SEVERITY, 20 INCIDENCE AND SEVERITY, 20 –– 80 %80 %
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INCIDENCE AND SEVERITY, 20 INCIDENCE AND SEVERITY, 20 –– 80 %80 %
�� LESION IN OTHER ORGANS : TESTIS, LESION IN OTHER ORGANS : TESTIS,
PANCREAS, PAROTID GLAND, MUSCLES, PANCREAS, PAROTID GLAND, MUSCLES,
CNS, AND LUNGS CNS, AND LUNGS
•• ARTHRALGIA & ARTHRITIS ARTHRALGIA & ARTHRITIS �������� 6868--75 % OF CASES 75 % OF CASES ��������SWOLLENSWOLLEN, PAINFUL, BLEEDING & EFFUSION (, PAINFUL, BLEEDING & EFFUSION (--))
•• AcRA CRITERIA : AcRA CRITERIA :
PALPABLE PURPURAPALPABLE PURPURA
AGE OF ONSET ≤ 20 YEARS OLDAGE OF ONSET ≤ 20 YEARS OLD
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BOWEL ANGINABOWEL ANGINA
SKIN BI0PSY : GRANULOSIT (+)SKIN BI0PSY : GRANULOSIT (+)
DIAGNOSIS : 2 OF 4 SYMPTOMS→ HSP(+)DIAGNOSIS : 2 OF 4 SYMPTOMS→ HSP(+)
Examination
� Lab finding : no specifik
� GIT bleeding : moderate lekocytosis,
normochromic anemia
� Eosinophylia
� ESR : increase
� IgA serum : sometime increase
� Skin biopsy : lekocitoclastik vasculitis
� Immonoflerence : deposit IgA and
complemen on the vessel walls
DIFFERENTIAL DIAGNOSIS OF HSP ACUTE ABDOMINAL PAINACUTE ABDOMINAL PAIN
RHEUMATOID ARTHRITISRHEUMATOID ARTHRITIS
RHEUMATOID FEVERRHEUMATOID FEVER
SPESIFIC LAB TEST FOR HSP (SPESIFIC LAB TEST FOR HSP (--))
THERAPYTHERAPY→ STEROID 1→ STEROID 1--2 mg/kgBW2 mg/kgBW
99
THERAPYTHERAPY→ STEROID 1→ STEROID 1--2 mg/kgBW2 mg/kgBW
PROGNOSIS :PROGNOSIS :DEPENDS ON THE SEVERITY AND WIDTH OF KIDNEY DEPENDS ON THE SEVERITY AND WIDTH OF KIDNEY DAMAGE DAMAGE
SIDE EFFECTS OF STEROIDSIDE EFFECTS OF STEROID
--HYPERTENSION HYPERTENSION
--HYPERGLYCEMIAHYPERGLYCEMIA
--HIRSUTISMHIRSUTISM
--MIOPATHYMIOPATHY
--OBESITYOBESITY
--MOON FACEMOON FACE
--OSTEOPOROSISOSTEOPOROSIS
--ACNEACNE
--PSEUDOMOTOR CEREBRIPSEUDOMOTOR CEREBRI
--CATARACTCATARACT
--GLAUCOMAGLAUCOMA
--MENTAL RETARDATIONMENTAL RETARDATION
(Am J. (Am J. DisChildDisChild 78:132; 80678:132; 806--10)10)
1111
1212
KIDNEY USG : NEPHROPATHY APPEARANCES IN BOTH KIDNEYNORMAL BLADDER
1313First week Second week
RECOVEREDRECOVERED
1414
1515