henochhenoch–schonlein schonlein...

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

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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

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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KIDNEY USG : NEPHROPATHY APPEARANCES IN BOTH KIDNEYNORMAL BLADDER

1313First week Second week

RECOVEREDRECOVERED

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