What is it about?

Systemic lupus erythematosus is a complex disease with genetic and environmental factors contributing to its development. Current research and view on lupus disease focuses on the autoimmune component. In this article we attemped to work out a general mechanism for disease development, which explains skin symptoms, organ damage, female predominance in terms broader than autoimmunity. This is achieved by examining the multiple roles of a molecule called C1q (and molecules with similar function).

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Why is it important?

We think that our approach provides a broader and better understanding of the disease development. This is particularly important because beyond finding effective novel treatments, early intervention or disease prevention also requires holistic approaches.

Perspectives

Current lupus treatment focuses on the autoimmune component of the disease. Biological therapies interfere with B-cell development and antibody production. Classical drugs supress immunity and inflammation, and exert effects we do not know or understand. A fresh look at the overall events occuring during disease may help to trigger novel ideas. For this reason we did not elaborate on the autoimmune events and cytokine profiles accompanying the disease but rather tried to emphasize other disease components. Personally, I think the hypothesis may provide a rational answer for a basic symptom : the butterfly rash. I have not seen any answer explaining the shape of the rash. If one superimposes a skin thickness map with a light exposure map of the face the thinnest+exposed areas nicely line out the butterfly. This we explain by saying that UV radiation reaching the capillaries, the endothelial cells triggers their death, thereby triggering inflammation by C1q-mediated immune complex infiltration into the damaged area. Similarly, predominance of the disease may be caused by differences in immune functions - but a powerful link between disease and women is the fact that they regulate their endothelium by different hormones than man do. The vascular endothelium is an aspect that is somewhat neglected as a lupus factor - we wished to highlight this in our paper.

József Prechl
Diagnosticum

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This page is a summary of: The endothelial deprotection hypothesis for lupus pathogenesis: the dual role of C1q as a mediator of clearance and regulator of endothelial permeability, F1000Research, January 2015, Faculty of 1000, Ltd.,
DOI: 10.12688/f1000research.6075.1.
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