Epidemiology and Clinical Manifestations of Henoch-Schönlein Purpura in Children

Kim, Se-Hun; Lee, Chong-Guk

J Korean Soc Pediatr Nephrol > Volume 7(2); 2003 > Article

J Korean Soc Pediatr Nephrol 2003;7(2): 166-173.

소아 Henoch-Schönlein 자반증의 역학 및 임상양상

김세훈, 이종국

¹인제대학교 의과대학 일산백병원 소아과
²인제대학교 의과대학 일산백병원 소아과

Epidemiology and Clinical Manifestations of Henoch-Schönlein Purpura in Children

Se-Hun Kim, Chong-Guk Lee

¹Department of Pediatrics, College of Medicine, Inje University, Ilsan Paik Hospital
²Department of Pediatrics, College of Medicine, Inje University, Ilsan Paik Hospital

ABSTRACT

Purpose : The cause and pathogenesis of $Henoch-Sch""{o}nlein$ purpura has been studied for many years but the results are disappointing. Recently the hypothesis that abnormalities involving the glycosylation of the hinge region of immunoglobulin Al(IgAl) may have an important role in the pathogenesis of $Henoch-Sch""{o}nlein$ purpura is being approved. $Henoch-Sch""{o}nlein$ purpura is the most common vasculitis Ihat affects children and the prognosis is good. But if kidney invovement occurs, the course may be chronic and troublesome. So we evaluated children with $Henoch-Sch""{o}nlein$ purpura especially from the point of epidemiology and clinical manifestations.
Methods : Investigation of 124 children who were diagnosed with $Henoch-Sch""{o}nlein$ purpura at Inje University Ilsan Paik Hospital from December 1999 to July 2003 was performed retrospectively through chart review. Efforts were made to get informations about the profile, epidemiology, clinical manifestations, progress of the disease and recurrence rate of patients.
Results : The patients were 69 boys and 55 girls, with a mean age of $6.1{pm}2.7$ years at the time of data collection. The male to female ratio was 1.25 : 1. The occurrence rate was much higher in autumn(from September to November, 31.5%) and winter(from December to February, 28.2%) than in spring and summer, with a peak in November. Joint involvement was shown in 66.9% of patients mostly on the foot/ankle(75.9%), knee(39.8%). Seventy(56.5%) out of 124 patients had abdominal pain and 10 patients(8.1%) showed bloody stools. Renal involvement was observed in 24 patients(19.4%) after 21.1 days on the average. IgA was elevated in 10 of 21 patients(47.6%). $C_3$ and $C_4$ levels were normal in 40 of 49 patients (81.7%) and 47 of 48 patients(97.9%), respectively Antistreptolysin-O(ASO) titer was elevated over 250 Todd units in 29 of 62 Patients(46.8%). Mycoplasma antibody titer was elevated in 21 of 49 patients(42.9%) equal or greater than 1:80. Radiologic studies were peformed in 23 patients. Seven patients(30.4%) showed bowel wall thickening and one of them received intestinal resection and anastomosis operation due to terminal ileum necrosis. Eighty four patients took steroid 1.4 mg/kg/day in average. Recurrence rate was 2.5 in 37 patients(29.8%).
Conclusion : $Henoch-Sch""{o}nlein$ purpura in childhood appears most in about 6 years of age. The occurrence rate is much higher in autumn and winter relatively. Diagnosis can be made through the perspective history taking and the inspection of clinical manifestations, but the laboratory findings are not of great help. A small portion of the patients might show abdominal pain or arthritis before purpura develops, therfore various diagnosis can be made. Radiologic evaluation should be performed to avoid surgical complications in cases accompanying abdominal pain, and long term follow up should be needed especially in patients suffering from kidney involvement. In about 30% of the patients $Henoch-Sch""{o}nlein$ purpura would recur. Steroid can be used safely without side effects.

Key words: $Henoch-Sch\""{o}nlein$ purpura | Nephritis | Epidemiology | Clinical manifestations