1. Henoch Schonlein Purpura UpToDate® N Engl J Med 2013; 369:1843 J Kor Med Sci 2014 Feb;29(2):
순천향대학교 서울병원
신장내과 R3 김윤석

2. 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

3. 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.)

4. 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

5. Pathogenesis

6. 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

7. Clinical manifestations
Purpura
Mostly intial symptom of HSP
Begins with erythematous, macular, urticarial wheals
Evolve into the ecchymoses, petechiae, palpable purpura

8. 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

9. 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

10. 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

11. 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

12. 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

13. 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

14. 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.