X linked agammaglobulinaemia and rheumatoid arthritis

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X linked agammaglobulinaemia and rheumatoid arthritis.

BACKGROUND Much interest has been shown recently in the pathogenic role of B cells in rheumatoid arthritis (RA) owing to the marked clinical responses to anti-CD20 treatment in RA. CASE REPORT A patient with X linked agammaglobulinaemia (XLA) presented with an erosive symmetric polyarthritis with histological features of RA, including formation of a destructive pannus. Furthermore, the patien...

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X-linked agammaglobulinemia and rheumatoid arthritis

The pathogenic role of B cells in rheumatoid arthritis (RA) has recently gained much interest by the marked clinical responses of anti-CD20 therapy in RA. We describe a patient with X-linked agammaglobulinemia (XLA) who presented with an erosive symmetrical polyarthritis with histological features of RA including formation of a destructive pannus. Furthermore, the patient also developed subcuta...

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X linked agammaglobulinaemia with a 'leaky' phenotype.

Typical X linked agammaglobulinaemia (XLA) is characterised by absence of immunoglobulin production and lack of mature B cells. The gene responsible for XLA has recently been identified, and codes for a B cell tyrosine kinase, BTK. A family affected by a B cell immunodeficiency, which is less severe than classical XLA, is described but they had a pedigree suggestive of X linked inheritance. Dem...

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Neutropenia associated with X-linked agammaglobulinaemia.

In a series of six cases of sex-linked agammaglobulinaemia neutropenia occurred as a presenting feature in four and during the presenting illness in the other two. The six patients all had low antibody titres and absent or low immunoglobulin concentrations with normal concentrations of T cells and absent B cells. The patients were all first seen with severe, acute infection, including septic ab...

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Mortality and morbidity in patients with X-linked agammaglobulinaemia.

BACKGROUND X-linked agammaglobulinaemia (XLA) is a genetic disorder characterised by a defect in the generation of mature B cells, lack of antibodies production, and susceptibility to recurrent bacterial infections. Understanding of the risk factors responsible for morbidity and mortality in these patients can help in a better management of this disorder. However, there is a lack of specific st...

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ژورنال

عنوان ژورنال: Annals of the Rheumatic Diseases

سال: 2005

ISSN: 0003-4967

DOI: 10.1136/ard.2004.030049