Complement deficiency and disease: An update.

Anders Sjöholm, Göran Jönsson, Jean Henrik Braconier, Gunnar Sturfelt, Lennart Truedsson

Forskningsoutput: TidskriftsbidragÖversiktsartikelPeer review

Sammanfattning

Complement deficiencies are probably vastly under-diagnosed within clinical medicine. Judging from a Swedish study of C2 deficiency, a deficiency with an estimated prevalence of about 1/20,000 in Western countries, less than 10% of the deficiencies of the classical and alternative pathways and the late complement components are identified in Sweden. C1 inhibitor deficiency and deficiencies of MBL and MASP-2 were not included in the assessment. The introduction of new screening methods should facilitate detection of complement deficiencies in clinical practice. In our study of C2 deficiency (n = 40), 57% of the patients had a history of invasive infection with encapsulated bacteria, mainly Streptococcus pneumoniae. This emphasizes the importance of the classical and/or the lectin pathway in defence against severe infection. Rheumatological disease, mainly systemic lupus erythematosus was present in 43% of the patients. In addition, a significant association was found between C2 deficiency and atherosclerosis. Complement-dependent disease mechanisms are discussed together with the potential importance of non-complement genes for disease expression in complement deficiencies. Analysis of larger patient groups is required in order to establish guidelines for investigation and treatment of patients with complement deficiency.
Originalspråkengelska
Sidor (från-till)78-85
TidskriftMolecular Immunology
Volym43
Nummer1-2
DOI
StatusPublished - 2006

Bibliografisk information

The information about affiliations in this record was updated in December 2015.
The record was previously connected to the following departments: Division of Microbiology, Immunology and Glycobiology - MIG (013025200), Department of Rheumatology (013036000), Division of Infection Medicine (SUS) (013008000)

Ämnesklassifikation (UKÄ)

  • Immunologi inom det medicinska området

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