الفهرس 
Juvenile Idiopathic ArthritisOnly 14 pages are availabe for public view
 |  | from | 122 |  |  |
| | | from | 122 | | |
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.