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 Clinical manifestations (Tetrad) 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 Male-to-female ratios of 1.2:1 to 1.8:1 Race 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 Immunofluorescence study 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 Laboratory 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 Renal disease 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 이후에도 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.