Rheumatology · 2 h ago
Primary cilia implicated in fibrosis across tissues
A review in Nature Reviews Rheumatology discusses emerging evidence that altered primary-cilium function may contribute to fibrotic disease in multiple organs. The authors identify ciliary signalling and cilium length as potential therapeutic research targets, but the evidence described does not establish a clinical treatment approach.
- Primary cilia regulate signalling involved in tissue remodelling.
- Ciliary dysfunction is reported in fibrotic fibroblasts.
- Manipulating cilia length remains a research strategy.
Fibrosis contributes to morbidity and mortality in many rheumatic diseases. This review examines research on primary cilia, solitary organelles found on most vertebrate cells that act as hubs for signalling pathways involved in tissue repair and remodelling. It summarizes evidence linking changes in cilia formation, structure and function with fibrotic processes across tissues and conditions.
The review reports that fibrotic fibroblasts in several settings show impaired ciliary signalling, and that shortening of primary cilia can alter cellular responses to external signals. It also highlights emerging findings that pharmacologically changing cilia length may normalize aspects of the activated myofibroblast phenotype. Examples cited include research in idiopathic pulmonary fibrosis, cardiac fibrosis and systemic sclerosis.
The authors propose “ciliotherapy” as a potential future strategy for fibrotic conditions associated with cilia shortening. The article presents an emerging research concept, not evidence of an established therapy or clinical benefit. The source provides no pooled estimates, patient sample size or clinical trial outcomes, and does not support changes to current care.
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Nature Reviews Rheumatology: Primary cilia as regulators of fibrosis and potential therapeutic targets ↗This is an automated AI-condensed summary that has not yet been reviewed by an editor. Always consult the full item at the original source.