HENOCHHENOCH–SCHONLEIN SCHONLEIN...
Transcript of HENOCHHENOCH–SCHONLEIN SCHONLEIN...
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HENOCHHENOCH––SCHONLEINSCHONLEINPURPURAPURPURA
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M. Sjabaroeddin Loebis, Lily Irsa, Rita Evalina
Allergy Immunology Division
Pediatrics Departement
Medical Faculty Sumatera Utara University
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�� 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
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�� 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
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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
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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
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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
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•• 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(+)
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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
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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
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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
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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)
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1111
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KIDNEY USG : NEPHROPATHY APPEARANCES IN BOTH KIDNEYNORMAL BLADDER
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1313First week Second week
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RECOVEREDRECOVERED
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