Download Clinical manifestations

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)
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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
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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
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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
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Upper skin involvement
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Renal involvement
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Persist hematuria, proteinuria
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Chronic renal failure
# Initial renal insufficienty
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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.