Clinical Study on Strategy for Refractory Henoch-Schönlein Purpura

IgA vasculitis is relatively common in children,especially in Asian countries. Abdominal manifestation could be severe, including bleeding, pancreatitis,appendicitis and intestinal intussusception. Delayed diagnosis could be fatal and cause severe complications.Nowadays no guidelines for those with severe abdominal manifestations in China.However, the most used treatment is steroid. For those severe forms are methylprednisolone pulse, IVIG, immunosuppressants and blood purification. Given the fact that different strategies lead to different endings which varies in cost, adverse effect and clinical outcomes in different medical centers, it is necessary to give birth to a useful and feasible strategy. This clinical trial is a muti-center, randomized,controlled prospective study.Patients with gastrointestinal disease will be recruited in three children's medical centres in Shanghai and will be randomized to two groups: MP group and IVIG group. Cost effect and clinical outcomes will be evaluated. Blood purification will be evaluated as a remedy when MP and IVIG fail to cure.

In this group patients will be given Methylprednisolone pulse(15-30mg/kg/d) and continued with oral prednisolone (2mg/kg/d)

1.methylprednisolone pulse (15-30mg/kg/d) for 3 days with continued oral prednisolone(2mg/kg/d) 2.if the patients still complain abdominal pain and feel abdominal tenderness on the third day after intervention , patients would be given IVIG(2g/kg/d)as a change

1. IVIG 2g/kg and oral prednisolone 2mg/kg/d 2.if the patients still complain abdominal pain and feel abdominal tenderness on the third day after intervention , patients would be given methylprednisolone pulse (15-30mg/kg/d) for 3 days

Omeprazole 0.8mg/kg.d.The maximum dose is less than 40mg per day Low molecular weight heparin calcium 50IU/kg.d

3 days after treatment part of the patients may fail the first assigned intervention and has to accept the alternative intervention and abdominal pain complaint and abdominal tenderness will be checked at 7 days after treatment

other treatment such as blood purification, the second methylprednisolone pulse, and immunosuppressant will be documented if the two interventions all failed

systolic or diastolic blood pressure greater than or equal to 95% for the age, sex, and height of the patient is considered to have 1.hypertension otherwise 0. Blood pressure will be measured with mercury blood pressure monitor.

1.ocular hypertension 0. no ocular hypertension. Ocular hypertension will be defined as intraocular pressure of either eye is greater greater or equal to 21mmHg

1. with steroid-related diabetes 0. no steroid-related diabetes . Steroid-related diabetes will be defined as random blood sugar is greater than 11.1 mmol/l

Inclusion Criteria: both genders age between 2-16 years old IgA vasculitis with gastrointestinal involvement course of disease less than 2 months refractory to ordinary dosage of prednisolone (less than 2mg/kg/d） Exclusion Criteria: patients with severe sepsis patients with central nervous system infection, patients with severe pneumonia patients with chronic infection (such as EBV, CMV, Tuberculosis) patients complicated by CKD who need renal replacement therapy patients suffering from severe central nervous system complications as intracranial hemorrhage or neuropathy