الفهرس | Only 14 pages are availabe for public view |
Abstract Juvenile Idiopathic Arthritis is the most frequent chronic rheumatic disease in childhood. MAS is a complication of JIA with elevated morbidity and mortality. MAS appears to occur with varying degrees of severity, ranging from the highly morbid child with persistent high grade fever, hepatosplenomegaly, bleeding tendency, and CNS manifestations. Laboratory parameters showing pancytopenia, coagulopathy, hepatic, and renal disorders to the unwell child with persistent fever, no organomegaly, a relative DROP in blood cell counting, and mild coagulopathy. Early recognition, differentiation from activity of the JIA itself and the rapid introduction of aggressive treatment contribute to better prognosis. The progress in understanding the pathophysiology behind MAS and identification of the pathways associated with the early stages of this syndrome can develop new biomarkers and treatment of MAS. The aim of this study is to investigate the presence of macrophage activation syndrome among JIA patients to assess the extent of the problem in those patients. We included in our study one hundred JIA patients registered in pediatric departments over the previous 2 years. Diagnosis of MAS was done according to preliminary diagnostic guidelines for MAS complicating JIA. We excluded from the study JIA patients who had history of recent infection, history of hepatitis or other liver disease, history of malignancy and history of pancytopenia for any cause. The hospital registry for JIA patients was scanned for medical history with special concerns for records of history suggestive of MAS, thorough clinical examination, Laboratory investigations and radiological data. The present study revealed: Among the 100 JIA patients, 14% of patients fulfilled the criteria and were diagnosed as MAS patients. Mortality rate of MAS syndrome represented 2% of all JIA patients and 14% of JIA cases with MAS. Continuous Fever, CNS and hemorrhagic manifestations were present only in MAS patients not in uncomplicated JIA patients. Ferritin level was elevated in all MAS patients. Renal affection was present in the majority of MAS patients, which was in contrast to uncomplicated JIA where there was no significant renal involvement. Coagulation abnormalities were prominent in majority of patients in form of thrombocytopenia and hypofibrinogenemia. |