A case of macrophage activation syndrome successfully treated with anakinra.
BACKGROUND
A 13-year-old girl presented with features typical of systemic juvenile idiopathic arthritis, including fever, rash and arthritis. Her past medical history was unremarkable.


METHODS
Routine investigations, including CBC, serological tests for urea and electrolyte levels, tests of liver function and clotting, measurement of erythrocyte sedimentation rate, cultures of blood, urine and throat swabs, abdominal ultrasonography, echocardiography, bone marrow aspiration, and determination of laboratory parameters, including presence of anti-streptolysin O antibody and levels of C-reactive protein, lactate dehydrogenase, serum ferritin, D-dimer, fibrinogen and ciclosporin. Specialized investigations included measurement of serum levels of interleukin (IL)-1, IL-6, IL-10, tumor necrosis factor and soluble CD25.


METHODS
Systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome.


RESULTS
The disease remained active following treatment with intravenous steroid, immunoglobulin and ciclosporin. The patient's disease was successfully controlled following the introduction of anakinra.
Kelly
Alison
A
Paediatric Rheumatology at the Bristol Royal Hospital for Children, Bristol, UK.
Ramanan
Athimalaipet V
AV
D000293:Adolescent; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001171:Arthritis, Juvenile / Q000150:complications*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D055501:Macrophage Activation Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology
D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein