Clinical, subclinical features and correlation of vascular diseases in patients with systemic sclerosis
Main Article Content
Abstract
This research was performed to investigate the clinical, subclinical features and correlation of peripheral vascular disease and pulmonary arterial hypertension in patients with systemic sclerosis. This was a cross-sectional study of 71 patients with systemic sclerosis. 100% of patients had nailfold microvascular impairment on capillaroscopy and most were in the early stage, accounting for 47.9%; the total capillaroscopy score was 3.3 ± 1.2. 98.6% of patients had Raynaud's phenomenon and the average process duration of Raynaud's phenomenon was 38.0 ± 37.2 months and the average Raynaud's condition score was 3.9 ± 1.5 points; 6 patients accounting for 8.5% had active digital ulcers; the average quantity of digital pitting scars was 1.1 ± 1.4. The mean systolic pulmonary artery pressure was 40.2 ± 5.1 mmHg. The majority of patients with WHO functional classification belonged to group II, accounting for 53.5%. The WHO functional classification mean was 2.0 ± 0.7. There was no correlation between pulmonary arterial pressure value and RCS, number of active digital ulcers and semi-quantitative nailfold capillaroscopic score of loss of capillaries with p>0.05. All patients had peripheral vascular disease on subclinical and nearly all showed clinical symptoms. Systolic pulmonary artery pressure was not too elevated and most patients had only mild symptoms. There was no correlation between pulmonary arterial hypertension and peripheral vascular diseases.
Article Details
Keywords
Raynaud phenomenon, nail-fold capillaries diseases, capillaroscopy, systolic pulmonary arterial pressure
References
2. S. Proudman, W. Stevens, J. Sahhar et al. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Internal medicine journal. 2007;37(7), 485-494. doi:10.1111/j.1445-5994.2007.01370.x
3. Varga. John, Denton, Christopher P et al. Scleroderma from pathogenesis to comprehensive management. New York: Springer; 2012.
4. John Varga. Systemic sclerosis (scleroderma) and related disorders. In: Dennis L.Kapper, Stephen L.hauster, J. Larry Jameson, eds. Harrison’s principles of internal medicine. 19th ed; New York: McGraw Hill. 2015: 2154-2166.
5. Minh, Vũ Nguyệt. Nghiên cứu sự biến đổi một số cytokin ở bệnh nhân xơ cứng bì hệ thống. Luận án tiến sĩ y học. Trường Đại học Y Hà Nội. 2018.
6. Rahul G Argula, Celine Ward, Carol Feghali-Bostwick. Therapeutic Challenges And Advances In The Management Of Systemic Sclerosis-Related Pulmonary Arterial Hypertension (SSc-PAH), Therapeutics and Clinical Risk Management. 2019;15(7), 1427–1442. doi: 10.2147/TCRM.S219024
7. Soukup T., R. Pudil, K. Kubinova et al. Application of the DETECT algorithm for detection of risk of pulmonary arterial hypertension in systemic sclerosis: data from a Czech tertiary centre. Rheumatology (Oxford). 2016;55(1): 109-14. doi: 10.1093/rheumatology/kev327
8. Cutolo M, Sulli A, Smith V. How to perform and interpret capillaroscopy. Best Pract Res Clin Rheumatol. 2013;27(2):237-248. doi: 10.1016/j.berh.2013.03.001
9. Lewis j. Rubin, david b. Badesch, robyn j. Barst et al. Bosentan therapy for pulmonary arterial hypertension, N Engl J Med. 2002;346(12), 896-908. doi: 10.1056/NEJMoa012212
10. Joglekar A, Tsai FS, McCloskey DA et al (2006). Bosentan in pulmonary arterial hypertension secondary to scleroderma. J Rheumatol. 33(1):61-8.