Abstract:After observing a child with systemic onset juvenile rheumatoid arthritis (S-JRA) who developed purpura fulminans in association with disseminated intravascular coagulation, with subsequent gangrene and autoamputation, we undertook a prospective study of coagulation parameters in children with JRA. Ten consecutive children with S-JRA, 10 children with rheumatoid factor-negative, polyarticular juvenile rheumatoid arthritis (P-JRA), and 10 age-and sex-matched controls were studied. Routine coagulation screening … Show more
In patients with juvenile dermatomyositis (JDM), small vessel occlusion and thrombosis result in a decrease in the capillary: muscle fiber ratio. We studied 15 patients with JDM. Six of 7 patients with clinically active JDM had elevated levels of C3d. Moreover, concentrations of fibrinopeptide A and factor VIIIrelated antigen were significantly increased in patients with clinically active JDM.Juvenile dermatomyositis (JDM) is a multisystem inflammatory disease that primarily affects skin and muscle (1,2). The hallmark of the pathology within the muscle is endothelial cell disruption, with subsequent small vessel thrombosis and progressive occlusion that results in a decrease in the capillary : muscle fiber ratio. In a number of immunologically mediated disorders, the occurrence and degree of tissue damage depend on the activation of the complement system (3). Subtle abnormalities of coagulation have also been demonstrated in many connective tissue diseases which have an underlying vasculitis in common.
In patients with juvenile dermatomyositis (JDM), small vessel occlusion and thrombosis result in a decrease in the capillary: muscle fiber ratio. We studied 15 patients with JDM. Six of 7 patients with clinically active JDM had elevated levels of C3d. Moreover, concentrations of fibrinopeptide A and factor VIIIrelated antigen were significantly increased in patients with clinically active JDM.Juvenile dermatomyositis (JDM) is a multisystem inflammatory disease that primarily affects skin and muscle (1,2). The hallmark of the pathology within the muscle is endothelial cell disruption, with subsequent small vessel thrombosis and progressive occlusion that results in a decrease in the capillary : muscle fiber ratio. In a number of immunologically mediated disorders, the occurrence and degree of tissue damage depend on the activation of the complement system (3). Subtle abnormalities of coagulation have also been demonstrated in many connective tissue diseases which have an underlying vasculitis in common.
We describe a 16-year-old girl with systemiconset juvenile arthritis who presented with pulmonary hypertension, without evidence of pleural or parenchymal involvement of the lung, pulmonary vasculitis, or immune deposition in the pulmonary vasculature. Pleuropulmonary involvement occurs occasionally in juvenile arthritis, but primary pulmonary hypertension has not, to our knowledge, been previously reported. Histocompatibility typing showed positivity for HLA-DR3 and DRw52, both of which are associated with idiopathic pulmonary hypertension in children, and with pulmonary hypertension among patients with systemic sclerosis. Treatment with cyclosporine and corticosteroids resulted in a marked improvement in the clinical findings and pulmonary function in our patient.Systemic-onset juvenile arthritis (JA) is characterized by high spiking fever, rash, lymphadenopathy, hepatosplenomegaly, serositis, and arthritis, and laboratory features of anemia, leukocytosis, thrombocytosis, and elevated serum levels of acute-phase reactants (1). Pulmonary manifestations in children with
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