Подходы к диагностике и ведению пациентов с гипермобильным типом синдрома Элерса — Данло

Abstract

This review provides information about the patterns of the pathogenesis, clinical picture of Ehlers-Danlos syndrome (EDS), as well as the diagnosis and treatment of hypermobile type. Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited autosomal dominant diseases characterized by the development disorder of the collagen molecular structure, which leads to joint hypermobility, hyperelastic skin and its vulnerability, as well as advanced fragility of the entire body’s tissues. The clinical picture of EDS hypermobile type is dominated by musculoskeletal signs, characterized by joint hypermobility, arthralgias, dorsalgias, spontaneous joint dislocations in combination with hyperelastic skin and its vulnerability. The criteria presented in the 2017 EDS clinical classification are applied in order to make a diagnosis. Timely diagnosis of the hypermobile type of EDS syndrome contributes to early detection of changes in other organs and systems: aortic root dilation, mitral valve prolapse, abdominal hernias, pelvic organ prolapse, and others. Treatment methods include medication, physiotherapy, and surgery, the individual choice of which depends on the specific disease manifestations, the intensity and duration of pain. Competent selection of examination methods and treatment for patients with EDS hypermobil type can effectively stop chronic pain of the musculoskeletal syndrome and prevent severe disabling complications concerning internal organs.KEYWORDS: joint hypermobility, chronic pain, Ehlers-Danlos syndrome, hyperelastic skin, spontaneous dislocations, arthralgia.FOR CITATION: Viktorova I.A., Ivanova D.S., Kochimov R.Sh., Adyrbayev A.M. Methods for the diagnosis and management of patients with hypermobile Ehlers-Danlos syndrome. Russian Medical Inquiry. 2020;4(8):498–503. DOI: 10.32364/2587-6821-2020-4-8-498-503.