REVIEW

Pathogenesis of systemic lupus erythematosus

C C Mok¹, C S Lau²

¹ Department of Medicine and Geriatrics, Tuen Mun Hospital, Tsing Chung Koon Road, New Territories, Hong Kong
² Department of Medicine, Queen Mary Hospital, Hong Kong, SAR, China

Correspondence to:
Correspondence to:
Dr C C Mok, Department of Medicine and Geriatrics, Tuen Mun Hospital, Tsing Chung Koon Road, New Territories, Hong Kong;
ccmok{at}netvigator.com

The exact patho-aetiology of systemic lupus erythematosus (SLE) remains elusive. An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably involved. Multiple genes contribute to disease susceptibility. The interaction of sex, hormonal milieu, and the hypothalamo–pituitary–adrenal axis modifies this susceptibility and the clinical expression of the disease. Defective immune regulatory mechanisms, such as the clearance of apoptotic cells and immune complexes, are important contributors to the development of SLE. The loss of immune tolerance, increased antigenic load, excess T cell help, defective B cell suppression, and the shifting of T helper 1 (Th1) to Th2 immune responses leads to B cell hyperactivity and the production of pathogenic autoantibodies. Finally, certain environmental factors are probably required to trigger the disease.

Keywords: aetiology; pathogenesis; genetic; interaction; autoimmune; autoantibody

Abbreviations: APC, antigen presenting cell; CD40L, CD40 ligand; CRH, corticotrophin releasing hormone; DHEA, dehydroepiandrosterone; dsDNA, double stranded DNA; FasL, Fas ligand; GnRH, gonadotrophin releasing hormone; HLA, human leucocyte antigen; HPA, hypothalamo–pituitary–adrenal; HRT, hormonal replacement therapy; IL, interleukin; LH, luteinising hormone; MBP, mannose binding protein; MHC, major histocompatibility complex; NK, natural killer; nRNP, nuclear ribonuclear protein; OC, oral contraceptive; PBMC, peripheral blood mononuclear cell; SLE, systemic lupus erythematosus; sm, Smith antigen; snRPN, small nuclear ribonuclear protein; Th1, T helper type 1

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