1 HENOCH–SCHONLEIN PURPURA M. Sjabaroeddin Loebis, Lily Irsa, Rita Evalina Allergy Immunology Division Pediatrics Departement Medical Faculty Sumatera Utara University

2  INTRODUCTION HENOCH SCHONLEIN PURPURA / ANAPHYLACTOID PURPURAE / NON TROMBOSITOPHENIC PURPURAE HENOCH SCHONLEIN PURPURA / ANAPHYLACTOID PURPURAE / NON TROMBOSITOPHENIC PURPURAE → VASCULITIS DISEASE OF THE SMALL VESSELS → SKIN, JOINTS, GI TRACT AND KIDNEY → PALPABLE PURPURA, ARTHRITIS/ARTHRALGIA, DIFFUSE ABDOMINAL PAIN, NEPHRITIS or HEMATURIA DIFFUSE ABDOMINAL PAIN, NEPHRITIS or HEMATURIA  HISTORY WILLAN AND HEBERDEN (1806)  PALPABLE PURPURA DUE TO VASCULITIS WILLAN AND HEBERDEN (1806)  PALPABLE PURPURA DUE TO VASCULITIS J.SCHONLEIN (1837)  + JOINTS PAIN E. HENOCH (1874)  + NEPHRITIS AND GI BLEEDING

3  INCIDENCE - USA : 14 – 15 CASES/ USA : 14 – 15 CASES/ ENGLAND : 20,4 CASES/ ENGLAND : 20,4 CASES/ NORWAY : 3,3 CASES/ NORWAY : 3,3 CASES/ INDONESIA - INDONESIA RSCM : 23 CASES RSCM : 23 CASES RSWS MAKASAR : 4 CASES RSWS MAKASAR : 4 CASES  AFFECTS ALL AGES, 2 – 15 YEARS OLD, PEAK : 4-7 YO, MALE : FEMALE = 1,5 : 1

ETIOLOGY  Still unknown  Genetic factor, UTRI, food,immunization, medicine  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 the skin, renal, joint and abdominal  skin purpura, nephritis, arthritis and GIT bleeding  Histologis : vasculitis leukocitoclastic

6 CLINICAL MANIFESTATION CLINICAL MANIFESTATION  PALPABLE PURPURA (>75% OF CASES), ARTHRALGIA OR ARTHRITIS, DIFFUSE ARTHRALGIA OR ARTHRITIS, DIFFUSE ABDOMINAL PAIN, GI BLEEDING ABDOMINAL PAIN, GI BLEEDING  KIDNEY DAMAGE → VARIED IN INCIDENCE AND SEVERITY, 20 – 80 % INCIDENCE AND SEVERITY, 20 – 80 %  LESION IN OTHER ORGANS : TESTIS, PANCREAS, PAROTID GLAND, MUSCLES, PANCREAS, PAROTID GLAND, MUSCLES, CNS, AND LUNGS CNS, AND LUNGS

7 ARTHRALGIA & ARTHRITIS  % OF CASES  SWOLLEN, PAINFUL, BLEEDING & EFFUSION (-) ARTHRALGIA & ARTHRITIS  % OF CASES  SWOLLEN, PAINFUL, BLEEDING & EFFUSION (-) AcRA CRITERIA : AcRA CRITERIA : PALPABLE PURPURA PALPABLE PURPURA AGE OF ONSET ≤ 20 YEARS OLD AGE OF ONSET ≤ 20 YEARS OLD BOWEL ANGINA BOWEL 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

9 DIFFERENTIAL DIAGNOSIS OF HSP ACUTE ABDOMINAL PAIN RHEUMATOID ARTHRITIS RHEUMATOID FEVER SPESIFIC LAB TEST FOR HSP (-) THERAPY→ STEROID 1-2 mg/kgBW PROGNOSIS : DEPENDS ON THE SEVERITY AND WIDTH OF KIDNEY DAMAGE

SIDE EFFECTS OF STEROID - HYPERTENSION - HYPERGLYCEMIA - OBESITY - MOON FACE - OSTEOPOROSIS - ACNE - HIRSUTISM - MIOPATHY - PSEUDOMOTOR CEREBRI - CATARACT - GLAUCOMA - MENTAL RETARDATION (Am J. DisChild 78:132; )

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12 KIDNEY USG : NEPHROPATHY APPEARANCES IN BOTH KIDNEY NORMAL BLADDER

13 First week Second week

14 RECOVERED RECOVERED

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