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Henoch Schonlein Purpura 2014 UpToDate® N Engl J Med 2013; 369:1843 J Kor Med Sci 2014 Feb;29(2):198-203 순천향대학교 서울병원 신장내과 R3 김윤석 Introduction • Definition – IgA vasculitis (IgAV) – Most common form of systemic vasculitis in children (90%) • Clinical manifestations (Tetrad) – – – – Palpable purpura without thrombocytopenia, coagulopathy Arthritis/arthralgia Abdominal pain Renal disease Epidermiology • Incidence – In UK: 20 per 100,000 in children < 17 yo – In Czech: 10 per 100,000 in children < 17 yo • Male-to-female ratios of 1.2:1 to 1.8:1 • Race – Asian, white > Black • Seasonal variation – Fall, Winter, Spiring > Summer – About Half cases preceded by an URI(especially Strep.) Pathogenesis • Leukoclastic vasculitis – By IgA immune complexes – Inflammatory infilatrations by neutrophils, monocytes • Immunofluorescence study – IgA, C3, Fibrin deposition within the walls of involved vessels Pathogenesis Clinical manifestations • Purpura (Almost all) • Arthralgia/Arthritis (2nd most common) • Abdominal pain – Coliky pain: about 50% patients – GI bleeding: about 20 to 30% patients • Renal disease (21-54%) • Other minor organs – Scrotum, CNS, Lung, Eye Clinical manifestations • Purpura – Mostly intial symptom of HSP – Begins with erythematous, macular, urticarial wheals – Evolve into the ecchymoses, petechiae, palpable purpura Clinical manifestations • Arthralgia/Arthritis – – – – – Usually transient or migratory Oligoarticular (1 to 4 joints) Nondeforming Usually affects the lower extemity large joints(hip, knee, ankle) Precede of purpura, usually last no more than one or two days Clinical manifestations • Abdominal pain – Mild : Nausea, vomiting, abdominal pain, transient paralytic ileus – Significant : Bleeding, bowel ischemia, necrosis, intussusception, perforation – Typically develop within 8 days of the rash N Engl J Med 2013; 369:1843 Clinical manifestations • Renal disease – Can develop at any time over several days to one month – Mild(asymptomatic hematuria) Bx: focal mesangial proliferation – Severe(marked proteinuria) Bx: Crescent formation Diagnosis • Symptoms • Biopsy – Leukocytoclastic vasculitis, IgA deposition • Laboratory – – – – Serum IgA (50 to 70%, higher level in renal involvement) Leukocytosis, elevated ESR (d/t preceding URI) CBC: IDA (d/t GI bleeding), normal PLT & coagulation (for DDx) Routine urinalysis with microscopy : Red or white cells, Cellular casts, Proteinuria Managements • Symptomatic treatment – Hydration – Pain control(joint, abdominal pain) • Renal disease – Only in advanced patient(Crescentic nephritis, AKI) – Steroid pulse therapy • Pulse IV methylprednisolone 250 to 1000 mg per day for 3 days • Oral prednisone 1mg/kg per day for 3 months – Cyclophosphamide (no benefit), Cyclosporine(May be beneficial) – Plasmapheresis – IVIG Adult HSP # 10yr retrospective study Adult > Children - Upper skin involvement - Renal involvement - Persist hematuria, proteinuria - Chronic renal failure # Initial renal insufficienty - R/F for progression to CKD Case • 33세 남환 1달전 편도 농양으로 입원 치료 받은 과거력 있는 환자로 내원 4일전부터 양쪽 다리에 petechiae 소견 있다가 내원 2일전부터 복통있어 ER 통해 MR dept 입원함. • 11/25 lower leg 의 petechia에 대하여 Punch Bx 시행, Leukocytoclastic vasculitis, consistent with HSP 확인됨. • 입원 당시 Proteinuria +1 이었고 이후 +3 로 악화됨. Steroid 치료 이 후에도 Persistant proteinuria 로 본과 협진, 12/12 renal Bx 시행함. – LM: crescents in 55% of glomeruli – IF: (+) mesangial and paramesangial IF staining of IgG, IgA, C3 – EM: mesangial electron dense deposit • Steroid 치료에 반응 없어 12/18 부터 Cyclophosphamide 치료 시작.