Introduction: Systemic sclerosis (SSc) is a chronic autoimmune disease of the connective tissue marked by fibrosis, which can have different clinical forms and variations in the number of cases between populations. Objective: Analyze the clinical and epidemiological characteristics of patients, the profile of autoantibodies and mortality from SSc at the Universitary Hospital of Teresina, PI, Brazil. Methods: Review of electronic medical records, with results presented in tables of absolute and relative frequencies. The association measures were expressed in estimated values of Odds Ratio (OR). For statistical significance, p < 0.05 was considered. Results: The profile found was predominantly of brown women, over 40 years old, with an average evolution of 6 years of illness, although there was a high percentage of men in the study. Mortality was high, with a significant association with male gender, age over 60 years, age at diagnosis over 60 years, patients from the interior of the state, hospitalization for pulmonary site infection and hospitalization over 30 days. The presence of the anti-centromere autoantibody (ACA) was 30.8%, associated with limited disease and pulmonary hypertension. Antitopoisomerase I antibody (ATA) predominated in patients with diffuse cutaneous fibrosis, interstitial pneumopathy and dysphagia, with a high percentage positivity (66.7%). Conclusion: The patient profile found follows Brazilian and international data. Mortality was high, with a greater number of early deaths and infectious causes. The prevalence of anti-Slc-70 was higher than the national average, but the profiles of involvement corroborate the pattern of autoantibodies described in the literature.
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Almeida, M. D. S. T. M. and Moreira, L. N. O. (2022). Analysis of Auto Antibodies, Mortality and Epidemiology of Hospitalized Patients for Systemic Sclerosis in a University Hospital. Open Access Library Journal, 9, e9295. doi: http://dx.doi.org/10.4236/oalib.1109295.
Pearson, D.R., Werth, V.P. and Pappas-Taffer, L. (2019) Systemic Sclerosis: Current Concepts of Skin and Systemic Manifestations. Clinics in Dermatology, 36, 459-474.
https://doi.org/10.1016/j.clindermatol.2018.04.004
Horimoto, A.M.C., Matos, E.N.N., Costa, M.R., Takahashi, F., Rezende, M.C., Kanomata, L.B., et al. (2019) Incidência e prevalência de esclerose sistêmica em Campo Grande, Estado de Mato Grosso do Sul, Brasil. Revista Brasileira de Reumatologia, 57, 107-114. https://doi.org/10.1016/j.rbr.2016.05.008
Sampaio-Barros, P.D., Bortoluzzo, A.B., Marangoni, R.G., Rocha, L.F., Del Rio, A.P.T., Samara, A.M., et al. (2019) Survival, Causes of Death, and Prognostic Factors in Systemic Sclerosis: Analysis of 947 Brazilian Patients. The Journal of Rheumatology, 39, 1971-1978. https://doi.org/10.3899/jrheum.111582
Muller, C.S., Paiva, E.S., Azevedo, V.F., Radominski, S.C. and Lima Filho, J.H.C. (2011) Perfil de autoanticorpos e correlação clínica em um grupo de pacientes com esclerose sistêmica na região sul do Brasil. Revista Brasileira de Reumatologia, 51, 314-324. https://doi.org/10.1590/S0482-50042011000400004
Grassegger, A., Pohla-Gubo, G., Frauscher, M. and Hintner, H. (2008) Autoantibodies in Systemic Sclerosis (Scleroderma): Clues for Clinical Evaluation, Prognosis and Pathogenesis. Wiener Medizinische Wochenschrift, 158, 19-28.
https://doi.org/10.1007/s10354-007-0451-5
Heijnen, I.A., Foocharoen, C., Bannert, B., Carreira, P.E., Roberto Caporali, R., Smith, V., et al. (2013) Clinical Significance of Coexisting Antitopoisomerase I and Anticentromere Antibodies in Patients with Systemic Sclerosis: A EUSTAR Group-Based Study. Clinical and Experimental Rheumatology, 31, 96-102.
Van den Hoogen, F., Khanna, D., Fransen, J., Johnson, S.R., Baron, M., Tyndall, A., et al. (2013) 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League against Rheumatism Collaborative Initiative. Annals of Rheumatic Diseases, 72, 1747-1755.
https://doi.org/10.1136/annrheumdis-2013-204424
Royle, J.G., Lanyon, P.C., Grainge, M.J., Abhishek, A. and Pearce, F.A. (2018) The Incidence, Prevalence and Survival of Systemic Sclerosis in the UK Clinical Practice Research Datalink. Clinical Rheumatology, 37, 2103-2111.
https://doi.org/10.1007/s10067-018-4182-3
Kuo, C.F., See, L.C., Yu, K.H., Chou, I.J., Tseng, W.Y., Chang, H.C., et al.(2019) Epidemiology and Mortality of Systemic Sclerosis: A Nationwide Population Study in Taiwan. Scandinavian Journal of Rheumatology, 40, 373-378.
https://doi.org/10.3109/03009742.2011.553736
Panopoulos, S., Bournia, V.K., Konstantonis, G., Fragiadaki, K., Sfikakis, P.P. and Tektonidou, M.G. (2018) Predictors of Morbidity and Mortality in Early Systemic Sclerosis: Long-Term Follow-Up Data from a Single-Centre Inception Cohort. Autoimmunity Reviews, 17, 816-820. https://doi.org/10.1016/j.autrev.2018.02.008
Batista, S.E.R., Cedeño, E.M. and Carralero, R.R. (2018) Caracterización clínica epidemiológica de pacientes con esclerosis sistémica en Holguín. Revista Cubana de Reumatología, 20, 1-14.
Graña, D., Vargas, A., Bérez, A., Goñi, M. and Danza, A. (2018) Esclerosis sistémica: forma de presentación y manejo terapéutico. Experiencia de un grupo de trabajo en Enfermedades Autoinmunes Sistémicas. Revista Uruguaya de Medicina Interna, 3, 15-22. https://doi.org/10.26445/RMU.3.1.2
Rosa, J.E., Soriano, E.R., Narvaez-Ponce, L., Cid, C.C., Imamura, P.M. and Catoggio, L.J. (2011) Incidence and Prevalence of Systemic Sclerosis in a Healthcare Plan in Buenos Aires. Journal of Clinical Rheumatology, 17, 59-63.
https://doi.org/10.1097/RHU.0b013e31820e7e8d
Walker, U.A., Tyndall, A., Czirják, L., Denton, C.P., Farge-Bancel, D., Kowal-Bielecka, O., et al. (2009) Geographical Variation of Disease Manifestations in Systemic Sclerosis: A Report from the EULAR Scleroderma Trials and Research (EUSTAR) Group Database. Annals of the Rheumatic Diseases, 68, 856-862.
https://doi.org/10.1136/ard.2008.091348
Aguila, L.A., Silva, H.C., Medeiros-Ribeiro, A.C., Bunjes, G., Luppino-Assad, A.P. and Sampaio-Barros, P.D. (2021) Is Exposure to Environmental Factors Associated with a Characteristic Clinical and Laboratory Profile in Systemic Sclerosis? A Retrospective Analysis. Rheumatology International, 41, 1143-1150.
https://doi.org/10.1007/s00296-020-04693-3
Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Yannick, A., et al. (2016) Update of EULAR Recommendations for the Treatment of Systemic Sclerosis. Annals of the Rheumatic Diseases, 76, 1327-1339.
https://doi.org/10.1136/annrheumdis-2016-209909
Guillevin, L., Bérezné, A., Seror, R., Teixeira, L., Pourrat, J., Mahr, A., et al. (2012) Scleroderma Renal Crisis: A Retrospective Multicentre Study on 91 Patients and 427 Controls. Rheumatology, 51, 460-467. https://doi.org/10.1093/rheumatology/ker271
Batista, S.E.R., Cedeño, E.M., Carralero, R.R., Avilés, E.C., Torres, L.P. and Fajardo, H.L.C. (2017) Supervivencia en pacientes con esclerosis sistémica en la provincia de Holguín. Revista Cubana de Reumatología, 19, 65-72
Elombila, M., Otiobanda, G., Mbaki, H., Outsouta, G. and Ngala, M. (2018) Epedemiology of Mortality in Polyvalent Intensive Care Unit at University Hospital of Brazzaville. Open Journal of Emergency Medicine, 6, 112-121.
https://doi.org/10.4236/ojem.2018.64013
Sampaio-Barros, P.D., Zimmermann, A.F., Müller, C.S., Borges, C.T.L., Freire, E.A.M., Maretti, G.B., et al. (2013) Recomendações sobre diagnóstico e tratamento da esclerose sistêmica. Revista Brasileira de Reumatologia, 53, 258-275.
https://doi.org/10.1590/S0482-50042013000300004
Stochmal, A., Czuwara, J., Trojanowska, M. and Rudnicka, L. (2020) Antinuclear Antibodies in Systemic Sclerosis: An Update. Clinical Reviews in Allergy & Immunology, 58, 40-51. https://doi.org/10.1007/s12016-018-8718-8
Horimoto, A.M.C. and Costa, I.P. (2015) Autoanticorpos em esclerose sistêmica e sua correlação com as manifestações clínicas da doença em pacientes do Centro-Oeste do Brasil. Revista Brasileira de Reumatologia, 55, 229-239.
https://doi.org/10.1016/j.rbr.2014.09.007
Mendes, C, Viana, V.S.T., Pasoto, S.G., Leon, E.P., Bonfa, E. and Sampaio-Barros, P.D. (2020) Clinical and Laboratory Features of African-Brazilian Patients with Systemic Sclerosis. Clinical Rheumatology, 39, 9-17.
https://doi.org/10.1007/s10067-019-04575-5
Skare, T.L., Fonseca, A.E., Luciano, A.C. and Azevedo, P.M. (2011) Autoantibodies in Scleroderma and Their Association with the Clinical Profile of the Disease. A Study of 66 Patients from Southern Brazil. Anais Brasileiros de Dermatologia, 86, 1075-1081. https://doi.org/10.1590/S0365-05962011000600003
Hasegawa, M. (2016) Biomarkers in Systemic Sclerosis: Their Potential to Predict Clinical Courses. The Journal of Dermatology, 43, 29-38.
https://doi.org/10.1111/1346-8138.13156
Tashkin, D.P., Elashoff, R., Clements, P.J., Goldin, J., Roth, M.D., Furst, D.E., et al. (2006) Cyclophosphamide versus Placebo in Scleroderma Lung Disease. New England Journal of Medicine, 354, 2655-2666. https://doi.org/10.1056/NEJMoa055120
Batista, S.E.R., Grass, A.V., Avilés, E.D.C., Pérez, L.T. and Portelles, A.F. (2015) Ciclofosfamida en el tratamiento de la esclerosis sistémica. Correo Cientifico Medico (CCM) de Holguin, 19, 706-717.
