Juvenile Dermatomyositis with a Rare and Severe Complication: Macrophage Activation Syndrome

Macrophage activation syndrome as a complication of juvenile rheumatoid arthritis.

OBJECTIVE Juvenile rheumatoid arthritis (JRA), also known as juvenile idiopathic arthritis (JIA), is a rare autoimmune joint disorder of children. The concrete causes for the prevalence of the above pathological state are still unknown. In other words, it is an arthritis affecting mainly children and adolescents. Clinically, it has 3 different clinical subtypes. JRA patients are often noticed w...

Severe vocal cord dysfunction: an unusual complication of juvenile dermatomyositis.

Efficacy of thalidomide in a girl with inflammatory calcinosis, a severe complication of juvenile dermatomyositis

We report a 14-year-old girl with juvenile dermatomyositis (JDM) complicated by severe inflammatory calcinosis successfully treated with thalidomide. She was diagnosed as JDM when she was 4 years old after a few months of increasing lethargy, muscle pain, muscle weakness, and rash. During three months, clinical manifestations and abnormal laboratory findings were effectively treated with oral p...

Macrophage Activation Syndrome as the First Presentation of Juvenile Idiopathic Arthritis

Macrophage activation syndrome (MAS) is a rare feature of rheumatic disorders in children and adolescence and its presentation as the first symptom of rheumatic disorders is very infrequent. A 9-year-old girl, in whom MAS developed, was admitted to our Hospital in Tehran, Iran. She suffered from high grade fever and rash followed by multiple joint swelling months afterwards. Bone marrow aspira...

Macrophage activation syndrome: an under-recognised complication in juvenile systemic lupus erythematosus

Macrophage activation syndrome (MAS) is a life-threatening complication in juvenile systemic lupus erythematosus(J SLE). We report three cases of MAS in Juvenile SLE which occurred over the last 6 months. Interestingly, in all the 3 cases, MAS occurred acutely at the time of first presentation of lupus. All of them had fever at presentation. Two of them had lymphadenopathy and splenomegaly and ...