Abstract: This paper aims to synthesize current knowledge on the diverse spectrum of rare vascular diseases, their underlying mechanisms, diagnostic approaches, and therapeutic interventions, as identified in the provided structured extraction summary. The review utilises 35 original studies with 1293 total participants (topic deduplicated ΣN). Across 72 mapped records spanning genetic/developmental, inflammatory/autoimmune, portal–hepatic, pulmonary, and other rare vascular entities, the evidence landscape is broad but methodologically uneven, with recurrent signals pointing to diagnostic delay and the need for specialized multidisciplinary care as cross-cutting challenges, exemplified by prolonged time to diagnosis in HHT. Mechanistically, the mapped literature repeatedly implicates endothelial dysfunction, dysregulated vascular signaling pathways (e.g., TGF-β/Wnt/NOTCH), and maladaptive remodeling or calcification processes as shared themes across otherwise heterogeneous diseases. Therapeutic reports and recommendations cluster around targeted or pathway-informed interventions (e.g., bevacizumab in HHT, celiprolol in vEDS, sirolimus in complex lymphatic disease) and procedure-based strategies in select conditions, but the predominance of small, often retrospective designs limits confidence in comparative effectiveness. A key practical implication is that improving recognition pathways and access to specialized diagnostics may reduce preventable morbidity, while a central limitation of this scoping map is the immature and heterogeneous evidence base that constrains generalizability. Future work should prioritize prospective multicenter registries and standardized diagnostic frameworks to better define natural history, stratify risk, and support more definitive evaluations of targeted treatments.
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