© 2002 by Annals of the Rheumatic Diseases
LESSON OF THE MONTH
Recurrent fevers in the presence of multiple autoimmune diseases and antibody deficiency
Series editor: Anthony D Woolf
Clinical Immunology Unit, Queens Medical Centre, Nottingham NG7 2UH, UK
Correspondence to:
Correspondence to:
Dr R J Powell
Accepted 4 March 2002
Keywords: fever; autoimmune diseases; antibody deficiency
Abbreviations: ITP, idiopathic thrombocytopenic purpura; PAN, polyarteritis nodosa
| The first 150 words of the full text of this article appear below. |
A 37 year old white South African woman presented with a two week history of fevers, rigors, and watery diarrhoea up to six times a day.
She had a complex past medical history, developing insulin dependent diabetes mellitus aged 2 years and hypothyroidism aged 11 years. At 13 years she was diagnosed with idiopathic thrombocytopenic purpura (ITP) and two years later underwent splenectomy, with a partial response. At 25 years, she developed haemolytic anaemia. Initial treatment with azathioprine was withdrawn owing to thrombocytopenia and she was subsequently treated for three years with oral cyclophosphamide. On moving to the United Kingdom, treatment was changed to a combination of danazol, hydroxychloroquine, and 10–30 mg oral prednisolone a day.
At the age of 33 she was diagnosed with primary antibody deficiency on the basis of recurrent pneumonia, autoimmune disease, borderline low and falling total IgG, low IgG2 subclass, and failure to produce a
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