الفهرس 
IntroductionOnly 14 pages are availabe for public view
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Abstract
The study included 70 patients with pediatric vasculitis, 20 of them were studied initially before the onset of therapy and 50 children with pediatric vasculitis were studied on treatment to see the effect of the drugs used in treatment. Study also included 20 apparently healthy children, of matched age and sex to the patients, studied as control. The children on study were 40 males and 50 females. Their age ranged from 2 to 16 years. It was observed that SLE was the most frequently encountered cause of vasculitis followed by SJIA then HSP and lastly post-infectious vasculitis and others. All cases and control have the following investigations done: CBC, ESR, CRP, renal function test, liver function test, C3, C4 and C5a levels measured in the serum, as well as ANCA level in serum. A chest x-ray, abdominal U/S as well as urine analysis and A/C ratio in urine were done. Advanced investigations were needed in some cases as CT abdomen/brain, immunological studies and infectious diseases panel. Although cases in this series differ significantly from control in the studied parameters, yet there was no difference between cases and controls in the mean ± SD of serum C5a levels. Regarding the effect of the drugs used in treatment of pediatric vasculitis in this study, namely; Corticosteroids only (used in 58% of cases), Corticosteroids + Methotrexate (used in 26% of cases) & Corticosteroids + Cyclophosphamide (used in 16% of cases); it was observed that it was observed that cases on combined Methotrexate and Steroid therapy scored best regarding the lowering of C5a level in serum. It is worthwhile to mention that regarding C5a level studied in this series, no significant difference was found between cases and controls; a finding that could be explained by the fact that most of our studied cases were of the immune-complex deposition diseases which have no effect on C5a level. Further research on this point is needed to decide whether such cases could benefit from the use of C5a inhibitors or not. This is unlike the ANCA-associated positive diseases such as Wagener Granulomatosis (Granulomatosis with polyangitis), where C5a level is markedly raised and these cases would benefit from C5a inhibitors. Kids are not just little people as regards types, classifications and causes of vasculitis. So much so that, starting from classification on the basis of blood vessel size, clinical findings and symptoms as well as being immune-complex diseases in most pediatric vasculitides unlike adult vasculitides that are classified on the basis of ANCA-associated positive vasculitis and ANCA negative vasculitis. According to the response to treatment of vasculitis in kids, kids vasculitides can be divided into: Acute self-limited course of vasculitis commonly due to medications such as NSAIDs or common infections responding to simple antibiotics such as Streptococcal infections. Relapsing and remitting course of vasculitis as in HSP and in the setting of connective tissue diseases as SJIA. chronic course On the other hand, vasculitis is most commonly idiopathic; with much smaller percentages caused by medications, autoimmune conditions, infections and malignancy. The importance of genetic factor in IgA immune-complex vasculitis in children such as HSP, SJIA and post-infectious vasculitis, is to be studied further in kids in future researches as it still controversial. Regarding the effect of the drugs used in treatment of pediatric vasculitis in this study, it was observed that cases on combined Methotrexate and Steroid therapy scored best regarding the lowering of C5a level in serum, however, in our cases as a whole, the level of C5a didn’t differ significantly from its level in control. It is worthwhile to mention that although the 3 arms of therapy used in this study had good effect on C3 & C4 levels, yet their effect on C5a level was not significant from control. This finding could be explained by the fact that most of our studied cases were of the immune-complex deposition diseases type (which have no effect on C5a) and were ANCA negative. Therefore, C5a inhibitor drugs probably will not be suitable in treatment of most common causes of pediatric vasculitis encountered in this series. Perhaps, this type of therapy might be used in ANCA-Positive associated vasculitides, e.g. Wegener’s Granulomatosis. Further research with bigger number of ANCA positive cases is needed to decide whether such cases could benefit from the use of C5a inhibitors or not.