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Adult onset Still’s disease: response to Enbrel
  1. R A Asherson1,
  2. L Pascoe1
  1. 1Rheumatic Diseases Unit, Department of Medicine, The Groote Schuur Hospital and the University of Cape Town School of Medicine, Cape Town, South Africa and The Rosebank Clinic, Johannesburg, South Africa
  1. Correspondence to:
    Dr R A Asherson, Johannesburg Consulting Offices, Rosebank Clinic (Suite 21), Sturdee Avenue, Johannesburg 2196, South Africa;

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Over the past year several publications have appeared recording the use of tumour necrosis factor (TNF) blockers, particularly infliximab (Revellex) in the treatment of adult onset Still’s disease (AOSD), a condition often resistant to a variety of treatments.

We wish to report a favourable response to etanercept (Enbrel) in a single patient who has proved resistant to a wide variety of agents over a 10 year period and who has had several, very serious complications of this condition, including the development of cardiac amyloidosis.

The patient, a 35 year old nursing sister, was initially diagnosed with AOSD at the age of 23 and was treated with non-steroidal anti-inflammatory drugs, salicylates, methotrexate, antimalarial drugs (chloroquine), and d-penicillamine. On this regimen she developed frequent flares and side effects to most of the disease modifying antirheumatic drugs to which she had been exposed—for example, antimalarial drugs resulted in the loss of peripheral vision in her right eye. Intramuscular gold injections (Myocrisin) were then given but also to no avail. High dose steroids (3g daily over five days intravenously (IV)) given at 6–8 weekly intervals caused weight gain and Cushing’s syndrome. Attacks of myalgic pain affecting the neck, shoulders, and mid-back areas were common, and were present throughout her illness. Eventually this was diagnosed as fibromyalgia.

At the age of 29 she was admitted to hospital with severe dyspnoea and chest pain. She tested positive for cytomegalovirus and coxsackievirus B. Steroids were ineffective and an emergency tracheotomy was performed in 1996. She was admitted to the intensive care unit with severe surgical emphysema, bilateral pneumothoraces, pleural effusions, and a pericardial effusion. She was kept sedated for most of her admission. The tracheotomy tube was removed but had to be reinserted owing to the collapse of both arytenoid cartilages. Eventually a Montgomery stent (permanent …

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