Ab1418 the Frequency and Structure of Undifferentiated Connective Tissue Dysplasia and Joint Hypermobility in the Transnistrian Population

Background: Undifferentiated connective tissue dysplasia (CTD) is a complex disorder with increasing prevalence, absent unified diagnostic criteria and significant association with different comorbidities including rheumatic diseases [1]. Joint hypermobility syndrome (JHS) is one of the key manifestations of CTD widely prevalent in patients with chronic musculoskeletal pain [2]. Objectives: The aim of the study was to evaluate the incidence and structure of CTD and JHS in the Transnistrian population. Methods: Outpatients with history of ≥2 stable chronic diseases were included. Information was collected through direct examination of patients, questionnaires and analysis of medical documents. The diagnosis of CTD was established with the involvement of ≥ 2 systems [1]. Beighton score ≥4 was considered as JHS [3]. p<0.05 was considered significant. Results: 234 patients were included (68.4% women, median age 49 [IQR 32;63] years, 50.8% <45 years). CTD was diagnosed in 124 (53%) patients (27.4% aged 18-30 years, 43.5% aged 31-59 years, 29.1% - ≥60 years). In patients with CTD we found 341 signs of the disease. Involvement of 2 systems was present in 30.6%, 3 systems – in 33.9%, 4 systems – in 24.2%; ≥5 – in 11.3%. Main manifestations included disorders of musculoskeletal system in 89.5% (scoliosis in 46.8%, flat feet in 48.4%, deviated nasal septum in 14.5%, JHS in 11.3%), gastrointestinal tract in 58.9% (disorders of gastrointestinal and gall bladder motility in 58.8%), eyes in 34.7% (myopia in 22.6%, blue sclera in 10.5%), skin in 34.7% (hyperextensibility >3 cm in 18.5%), nervous system in 29.8%, cardiovascular (CV) system in 21.8% (mitral valve prolapse in 10.5%, varicose veins of the lower extremities in 10.5%), upper urinary tract in 4.8%, blood disorders in 0.8%. 53.6% of people with CTD had diseases of the joints and spine: problems with the spine, mostly osteochondrosis in 77 (62.1%), osteoarthritis (OA) in 38 (30.6%) patients, rheumatoid arthritis (RA) in 8 (6.5%), gout in 1 patient. Patients with CTD compared to no CTD more often had back pain (75.8% vs 38.2%, p<0.001). Changes in musculoskeletal system were combined with changes in the gastrointestinal tract in 63.1%, problems with eyes in 36.0%, with skin in 31.5%. Women with CTD compared to men more often had skin and nervous system manifestations (51% vs 25%, p=0.043 and 50% vs 18%, p=0.024, respectively). Subjects aged <60 years compared with the elderly more often had nervous system (44% vs 24%, p=0.04) and CV manifestations (22% vs 11%, p<0.001, respectively). JHS was present in 9.4% (59.1% women, median age 29.5 [23.8;39] years, 90.9% aged <45 years, p<0.05 compared to CTD group), median Beighton score 4 (4;6). Signs of CTD were observed in 63.6%: musculoskeletal manifestations in all, eye manifestations – in 54.5, nervous system – in 18.2%. Patients with CTD and JHS, compared with the group without JHS more often had musculoskeletal (100% vs 53%) and eye manifestations (55% vs 17%) (p<0.001) and less often gastrointestinal manifestations (9% vs 35% (p=0.01). Patients with JHS compared to no JHS less often had back pain (36.4 vs 60.4%, p=0.03) and diseases of large joints (22.7 vs 45.3%, p=0.04) and spine (36.4 vs 61.3%, p=0.02), possibly because of younger median age compared to the whole group and group with CTD. Conclusion: In outpatients in Transnistria CTD is highly prevalent, characterized by diversity of clinical manifestations and gender and age heterogeneity with predominance of musculoskeletal manifestations in all categories. Patients with JHS were significantly younger and had less joint and spine manifestations. REFERENCES: [1] Guarnieri V, Castori M. Clinical Relevance of Joint Hypermobility and Its Impact on Musculoskeletal Pain and Bone Mass. Curr Osteoporos Rep. 2018 Aug;16(4):333-343. [2] Russian Scientific Medical Society of Therapists (RSMST) Undifferentiated connective tissue dysplasia (the project of guidelines). Therapy. 2019; 7 [33]:9–42. [3] Beighton P, Solomon L, Soskolne CL. Articular mobility in an African population. Ann Rheum Dis 1973;32:413-8. Acknowledgements: NIL. Disclosure of Interests: Irina Chekan: None declared, Iana Covbasiuc: None declared, Elena Troitskaya: None declared, Zhanna Kobalava Novartis, Servier, Astra-Zeneca, Bayer, Rita Osipyants: None declared.