Main Article Content
Introduction: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by progressive fibrosis of the skin and internal organs that promotes high morbidity and mortality. Objective: To evaluate the functionality, disability and quality of life of patients with systemic sclerosis and to compare the clinical forms of the disease. Methods: Cross-sectional, descriptive and analytical study performed at the Rheumatology Clinic of the Hospital das Clínicas of the Federal University of Pernambuco (HC-UFPE) from August 2018 to April 2019. The non-probabilistic, convenience sample consisted of 60 patients diagnosed with systemic sclerosis (SSc), followed at the Rheumatology outpatient clinic of the Hospital das Clínicas, Federal University of Pernambuco. To evaluate the outcomes, the following instruments were used: Cochin Hand Functional Scale (CHFS) for hand function; 12-Item Short-Form Health Survey (SF-12) for quality of life; and Scleroderma Health Assessment Questionnaire (SHAQ) for functionality and disability. Results: The mean results for CHFS, SHAQ, SF-12 Physical Component Summary and SF-12 Mental Component Summary were 14.5 (6.0-29.75), 1.01±0.56, 35.04±8.09, 40.94±10.56, respectively. There were no significant differences in CHFS outcomes between patients with diffuse and limited forms of SSc, SHAQ and the mental component of SF-12. However, in the physical component of SF-12, a better score was found in patients with the diffuse form of the disease (p=0.04). Conclusion: Patients with SSc present an important impairment of hand function, quality of life and functional capacity, and those with limited cutaneous form present worse scores of the physical component in the evaluation of quality of life.
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License (CC BY) that allows others to share and adapt the work with an acknowledgement of the work's authorship and initial publication in this journal.Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
2. Sticherling M. Systemic sclerosis-dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings. J Dtsch Dermatol Ges. 2012;10(10):705-18. https://doi.org/10.1111/j.1610-0387.2012.07999.x
3. Justo AC, Guimarães FS, Ferreira AS, Soares MS, Bunn PS, Lopes AJ. Muscle function in women with systemic sclerosis: Association with fatigue and general physical function. Clin Biomech. 2017;47:33-9. https://doi.org/10.1016/j.clinbiomech.2017.05.011
4. Kucharz EJ, Magdalena KM. Systemic sclerosis sine scleroderma. Adv Clin Exp Med. 2017;26(5):875-880. http://doi.org/10.17219/acem/64334
5. Lórand V, Czirják L, Minier T. Musculoskeletal involvement in systemic sclerosis. Presse Med. 2014;43(10):e315-28. http://doi.org/10.1016/j.lpm.2014.03.027
6. Bretterklieber A, Painsi C, Avian A, Wutte N, Aberer E. Impaired quality of life in patients with systemic sclerosis compared to the general population and patients with other chronic dermatoses. BMC Res Notes. 2014;7:594. http://doi.org/10.1186/1756-0500-7-594
7. Almeida C, Almeida I, Vasconcelos C. Quality of life in systemic sclerosis. Autoimmun Rev. 2015;14(12):1087-96. http://doi.org/10.1016/j.autrev.2015.07.012
8. Horváth J, Bálint Z, Szép E, Deiszinger A, Minier T, Farkas N, et al. Efficacy of intensive hand physical therapy in patients with systemic sclerosis. Clin Exp Rheumatol. 2017;106(4):159-66.
9. van den Hoogen F, Khanna D, Fransen J, Johnson RS, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: An american college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747-55. http://doi.org/10.1136/annrheumdis-2013-204424
10. Landim SF, Bertolo MB, Abreu MFM, Del Rio AP, Mazon CC, Marques-Neto JF, et al. The evaluation of a home-based program for hands in patients with systemic sclerosis. J Hand Ther. 2019;32(3):313-21. http://doi.org/10.1016/j.jht.2017.10.013
11. Chiari A, Sardim SCC, Natour J. Translation, cultural adaptation and reproducibility of the Cochin Hand Functional Scale questionnaire for Brazil. Clinics. 2011;66(5):731-6. http://dx.doi.org/10.1590/S1807-59322011000500004
12. Nguyen CB, Bérezné A, Mestre-Stanislas C, Lefevre-Colau MM, Rannou F, Guillevin L, et al. Changes over time and responsiveness of the cochin hand function scale and mouth handicap in systemic sclerosis scale in patients with systemic sclerosis. Am J Phys Med Rehabil. 2016;95(12):e189-97. http://dx.doi.org/10.1097/PHM.0000000000000611
13. Shou J, Ren L, Wang H, Yan F, Cao X, Wang H, et al. Reliability and validity of 12-item Short-Form health survey (SF-12) for the health status of Chinese community elderly population in Xujiahui district of Shanghai. Aging Clin Exp Res. 2015;28(2):339-46. http://dx.doi.org/10.1007/s40520-015-0401-9
14. Corbacho MI, Dapueto JJ. Assessing the functional status and quality of life of patients with rheumatoid arthritis. Rev Bras Reumatol. 2010;50(1):31-43. http://dx.doi.org/10.1590/S0482-50042010000100004
15. Westergren A, Hagell P. Measurement properties of the 12-item short-form health survey in stroke. J Neurosci Nurs. 2014;46(1):34-45. http://doi.org/10.1097/JNN.0000000000000027
16. Andrade TL, Camelier AA, Rosa FW, Santos MP, Jezler S, Silva JLP. Applicability of the 12-Item Short-Form Health Survey in patients with progressive systemic sclerosis. J Bras Pneumol. 2007;33(4):414-422. http://dx.doi.org/10.1590/S1806-37132007000400010
17. Rocha LF, Marangoni RG, Sampaio-Barros PD, Levy-Neto M, Yoshinari NH, Bonfa E, et al. Cross-cultural adaptation and validation of the Brazilian version of the Scleroderma Health Assessment Questionnaire (SHAQ). J Clin Rheumatol. 2014;33(5):699-706. http://doi.org/10.1007/s10067-013-2370-8
18. Kwakkenbos L, Sanchez TA, Turner KA, Mouthon L, Carrier ME, Hudson M, et al. The association of sociodemographic and disease variables with hand function: a Scleroderma Patient-centered Intervention Network cohort study. Clin Exp Rheumatol. 2018;113(4):88-94.
19. Peytrignet S, Denton CP, Lunt M, Hesselstrand R, Mouthon L, Silman A, et al. Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study. Rheumatology. 2018;57(2):370-81. http://doi.org/10.1093/rheumatology/kex410
20. Silva GS, Almeida Lourenço M, Assis MR. Hand strength in patients with RA correlates strongly with function but not with activity of disease. Adv Rheumatol. 2018;58:20. http://dx.doi.org/10.1186/s42358-018-0020-1
21. Barney BJ, Wang XS, Lu C, Liao Z, Johnson VE, Cleeland CS, et al. Prognostic value of patient-reported symptom interference in patients with late-stage lung cancer. Qual Life Res. 2013;22(8):2143-50. http://doi.org/10.1007/s11136-013-0356-2
22. Hudson M, Thombs BD, Steele R, Panopalis P, Newton E, Baron M, et al. Health-related quality of life in systemic sclerosis: a systematic review. Arthritis Care Res. 2009;61(8):1112-20. http://doi.org/10.1002/art.24676
23. Morrisroe K, Hudson M, Baron M, Vries-Bouwstra J, Carreira PE, Wuttge DM, et al. Determinants of health-related quality of life in a multinational systemic sclerosis inception cohort. Clin Exp Rheumatol. 2018;36(113):53-60.
24. Jaeger VK, Distler O, Maurer B, Czirják L, Lóránd V, Valentini G, et al. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group. Rheumatology. 2018;57(3):441-50. http://doi.org/10.1093/rheumatology/kex182