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2015 ; 27
(6
): 555-62
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Origin of fibrosing cells in systemic sclerosis
#MMPMID26352735
Ebmeier S
; Horsley V
Curr Opin Rheumatol
2015[Nov]; 27
(6
): 555-62
PMID26352735
show ga
PURPOSE OF REVIEW: Systemic sclerosis, an autoimmune disease of unknown origin,
is characterized by progressive fibrosis that can affect all organs of the body.
To date, there are no effective therapies for the disease. This paucity of
treatment options is primarily because of limited understanding of the processes
that initiate and promote fibrosis in general and a lack of animal models that
specifically emulate the chronic nature of systemic sclerosis. Most models
capitulate acute injury-induced fibrosis in specific organs. Yet, regardless of
the model a major outstanding question in the field is the cellular origin of
fibrosing cells. RECENT FINDINGS: A multitude of origins have been proposed in a
variety of tissues, including resident tissue stroma, fibrocytes, pericytes,
adipocytes, epithelial cells and endothelial cells. Developmentally derived
fibroblast lineages have recently been elucidated with fibrosing potential in
injury models. Increasing data support the pericyte as a fibrosing cell origin in
diverse fibrosis models and adipocytes have recently been proposed. Fibrocytes,
epithelial cells and endothelial cells also have been examined, although data do
not as strongly support these possible origins. SUMMARY: In this review, we
discuss recent evidence arguing in favor of and against proposed origins of
fibrosing cells in diverse models of fibrosis. We highlight outstanding
controversies and propose how future research may elucidate how fibrosing cells
arise and what processes can be targeted in order to treat systemic sclerosis.
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