Soluble biglycan hasbeen identified as a DAMP, capable of activating TLR2 and TLR4 signaling andpromoting sterile inflammation [102, 103, 104], a hallmark of acute aortic syndromes.Elevated circulating levels of biglycan have been documented in a range ofinflammatory and cardiovascular conditions, including atherosclerosis [105, 106],pulmonary hypertension [107] and chronic liver disease [108], suggesting itspotential as a nonspecific indicator of vascular ECM remodeling. The gene discussed is BGN; the disease is pulmonary hypertension.