Usefulness of glycosylated ferritin in atypical presentations of adult onset Still's disease

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Abstract
An 80 year old woman presented with 4 weeks’ fever, myalgias, polyarthritis, and sore throat. Clinical examination disclosed a fixed generalised maculopapular rash, arthritis of wrists and ankles, and spleen enlargement. C reactive protein was 150 mg/l, haemoglobin level 80 g/l, white blood count 30×109/l with 90% polymorphonuclear neutrophils, 10% lymphocytes, and 600×109/l platelets. Transaminases were twice the normal value (2N). Ferritinaemia was 40 000 μg/l (normal 50–300). Extensive investigations for infections, antinuclear and antineutrophil cytoplasmic antibodies, and rheumatoid factor were negative. Echocardiography, thoracic and abdominal computed tomographic scan, bone marrow biopsy, lumbar puncture were normal, except for splenomegaly. Skin biopsy disclosed unspecific urticarial lesions. The outcome was marked by high spikes of fever, general malaise with low blood pressure, hypoxaemia, suggesting systemic inflammatory response syndrome, and necessitating admission to an intensive care unit.