许多患者可健康生存,生活不受影响。只有血管型 EDS (vEDS) 与寿命缩短相关。[41]Pepin M, Schwarze U, Superti-Furga A, et al. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342:673-80.http://www.nejm.org/doi/full/10.1056/NEJM200003093421001#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/10706896?tool=bestpractice.com
对于存在慢性疼痛综合征和/或严重破坏性反复损伤和关节脱位的患者,治疗具有极大的挑战性。已知有部分患者可很好地恢复其正常的日常功能,且生活质量良好,而其余患者仍存在需要持续用药和心理调整的重度残疾。[20]Rombaut L, Malfait F, Cools A, et al. Musculoskeletal complaints, physical activity and health-related quality of life among patients with the Ehlers-Danlos syndrome hypermobility type. Disabil Rehabil. 2010;32(16):1339-45.http://www.ncbi.nlm.nih.gov/pubmed/20156051?tool=bestpractice.com 总体而言,这些患者的残疾具有临床意义,但残疾的严重程度存在个体差异且可能依症状而有所不同。 目前尚不清楚什么因素会导致严重残疾,因此难以确定哪些个体存在出现慢性并发症的风险。[36]Scheper MC, Juul-Kristensen B, Rombaut L, et al. Disability in adolescents and adults diagnosed with hypermobility-related disorders: a meta-analysis. Arch Phys Med Rehabil. 2016 Dec;97(12):2174-87.http://www.ncbi.nlm.nih.gov/pubmed/26976801?tool=bestpractice.com
vEDS 患者的平均生存年龄为 51 岁,患者最终死于动脉或内脏破裂。[42]Byers PH, Belmont J, Black J, et al. Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome. Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):40-7.http://onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31553/fullhttp://www.ncbi.nlm.nih.gov/pubmed/8306228?tool=bestpractice.com 最好避免手术和侵入性的放射学检查,因其有危及生命的并发症的风险。[41]Pepin M, Schwarze U, Superti-Furga A, et al. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342:673-80.http://www.nejm.org/doi/full/10.1056/NEJM200003093421001#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/10706896?tool=bestpractice.com 这些患者妊娠是危险的,但成功的例子也不少。