Más allá de dos enfermedades: Revelando las realidades clínicas únicas del síndrome de superposición de esclerosis sistémica y lupus eritematoso sistémico
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Keywords
Esclerodermia Sistémica, Lupus Eritematoso Sistémico, Enfermedades del colágeno
Resumen
Introducción: La esclerosis sistémica (ES) y el lupus erite- matoso sistémico (LES) son enfermedades autoinmunes crónicas del tejido conectivo. Su coexistencia, denominada síndrome de superposición ES-LES (SSP), configura un cuadro clínico complejo y poco estudiado, especialmente en poblaciones brasileñas. El objetivo de este estudio fue caracterizar y comparar el perfil clínico de pacientes con ES, LES y SSP. Métodos: Se realizó un estudio retrospectivo, observacional y transversal, basado en la revisión de historias clínicas de un servicio de reumatología. Se incluyeron 103 pacientes: 44 con ES, 39 con LES y 20 con SSP, siguiendo los criterios ACR/EULAR 2013 para ES y 2019 para LES. Se analizaron datos demográficos, clínicos, laboratoriales e imagenológicos. Resultados: Los pacientes con LES fueron diagnosticados a menor edad (32,8 años) que los de ES (47,7 años), con valores intermedios en SSP (35,2 años). Predominaron mujeres y afrodescendientes en SSP (52,6%). El fenómeno de Raynaud fue universal en SSP (100%), y la enfermedad pulmonar intersticial más frecuente en ES (75%) y SSP (50%). El anti-U1RNP fue positivo en 70% de SSP, y la linfopenia afectó al 95%. Conclusión: El SSP representa una entidad clínica singular, con combinación de manifestaciones pulmonares y articulares, heterogeneidad serológica y predominio en mujeres jóvenes afrodescendientes.
Citas
1. Jury EC, D’Cruz D, Morrow WJW. Autoantibodies and overlap syndromes in autoimmune rheumatic disease. J Clin Pathol. 2001;54(5):340-7. DOI: https://doi.org/10.1136/jcp.54.5.340
2. Jog NR, James JA. Biomarkers in connective tissue diseases. J Allergy
Clin Immunol [Internet]. 2017;140(6):1473-83. Disponible en: https:// doi.org/10.1016/j.jaci.2017.10.003
3. Stochmal A, Czuwara J, Trojanowska M, Rudnicka L. Antinuclear antibodies in systemic sclerosis: an update. Clin Rev Allergy Immunol [Internet]. 2020;58(1):40-51. Disponible en: http://link.springer. com/10.1007/s12016-018-8718-8 DOI: https://doi.org/10.1007/s12016-018-8718-8
4. Hiepe F, Dorner T, Burmester GR. Editorial overview: antinuclear antibody- and extractable nuclear antigen-related diseases. Int Arch Allergy Immunol. 2000;123(1):5-9. DOI: https://doi.org/10.1159/000024418
5. Moutsopoulos HM. Autoimmune rheumatic diseases: one or many diseases? J Transl Autoimmun [Internet]. 2021;4:100129. Disponible en: https://doi.org/10.1016/j.jtauto.2021.100129 DOI: https://doi.org/10.1016/j.jtauto.2021.100129
6. Cervera R, Khamashta MA, Hughes GRV. Overlap syndromes. Ann Rheum Dis. 1990;49(11):947-8. DOI: https://doi.org/10.1136/ard.49.11.947
7. Denton CP, Khanna D. Systemic sclerosis. Lancet [Internet]. 2017;390(10103):1685-99. Disponible en: http://dx.doi.org/10.1016/ S0140-6736(17)30933-9 DOI: https://doi.org/10.1016/S0140-6736(17)30933-9
8. Lepri G, Di Battista M, Codullo V, Bonomi F, Sulis A, Guiducci S, et al. Systemic sclerosis: one year in review 2024. Clin Exp Rheumatol. 2024;42(8):1517-28. DOI: https://doi.org/10.55563/clinexprheumatol/is29he
9. Aringer M. Inflammatory markers in systemic lupus erythematosus. J Autoimmun [Internet]. 2020;110:102374. Disponible en: https://doi. org/10.1016/j.jaut.2019.102374 DOI: https://doi.org/10.1016/j.jaut.2019.102374
10. Scherlinger M, Guillotin V, Truchetet ME, Contin-Bordes C, Sisirak V, Duffau P, et al. Systemic lupus erythematosus and systemic sclerosis: all roads lead to platelets. Autoimmun Rev [Internet]. 2018;17(6):625- 35. Disponible en: https://doi.org/10.1016/j.autrev.2018.01.012 DOI: https://doi.org/10.1016/j.autrev.2018.01.012
11. Alharbi S, Ahmad Z, Bookman AA, Touma Z, Sanchez-Guerrero J, Mitsakakis N, et al. Epidemiology and survival of systemic sclerosis- systemic lupus erythematosus overlap syndrome. J Rheumatol. 2018;45(10):1406-10. DOI: https://doi.org/10.3899/jrheum.170953
12. Andraos R, Ahmad A, Eriksson P, Dahlstrom O, Wirestam L, Dahle C, et al. Autoantibodies associated with systemic sclerosis in three autoimmune diseases imprinted by type I interferon gene dysregulation. Lupus Sci Med. 2022;9(1):e000793. DOI: https://doi.org/10.1136/lupus-2022-000732
13. Fanouriakis A, Kostopoulou M, Alunno A, Aringer M, Bajema I, Boletis JN, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736-45. DOI: https://doi.org/10.1136/annrheumdis-2019-215089
14. Van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis. Arthritis Rheum. 2013;65(11):2737-47. DOI: https://doi.org/10.1002/art.38098
15. Ortega-Hernández OD, Shoenfeld Y. Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment. Best Pract Res Clin Rheumatol [Internet]. 2012;26(1):61-72. Disponible en: http://dx.doi.org/10.1016/j.berh.2012.01.009 DOI: https://doi.org/10.1016/j.berh.2012.01.009
16. Foocharoen C, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R. Clinical characteristics of scleroderma overlap syndromes: comparisons with pure scleroderma. Int J Rheum Dis. 2016;19(9):913-23. DOI: https://doi.org/10.1111/1756-185X.12884
17. Iaccarino L, Gatto M, Bettio S, Caso F, Rampudda M, Zen M, et al. Overlap connective tissue disease syndromes. Autoimmun Rev [Internet]. 2013;12(3):363-73. Disponible en: http://dx.doi. org/10.1016/j.autrev.2012.06.004 DOI: https://doi.org/10.1016/j.autrev.2012.06.004
18. Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM, Denton CP. Clinical and serological hallmarks of systemic sclerosis overlap syndromes. J Rheumatol. 2011;38(11):2406-9. DOI: https://doi.org/10.3899/jrheum.101248
19. Xiao ZX, Miller JS, Zheng SG. An updated advance of autoantibodies in autoimmune diseases. Autoimmun Rev. 2021;20(2):102743. DOI: https://doi.org/10.1016/j.autrev.2020.102743
20. Sibanda EN, Chase-Topping M, Pfavayi LT, Woolhouse MEJ, Mutapi F. Evidence of a distinct group of Black African patients with systemic lupus erythematosus. BMJ Glob Health. 2018;3(5):e000697. DOI: https://doi.org/10.1136/bmjgh-2017-000697
21. Nagy G, Czirják L, Kumánovics G. Patients with systemic sclerosis with and without overlap syndrome show similar microvascular abnormalities. Diagnostics. 2021;11(9):1654. DOI: https://doi.org/10.3390/diagnostics11091606
22. Lambova SN. Scleroderma-like capillaroscopic pattern in SLE is not a sign of overlap syndrome in both adults and children. Lupus Sci Med. 2022;9(1):e000699. DOI: https://doi.org/10.1136/lupus-2022-000749
23. Richter P, Cardoneanu A, Dima N, Bratoiu I, Rezus C, Burlui AM, et al. Interstitial lung disease in systemic lupus erythematosus and systemic sclerosis: how can we manage the challenge? Int J Mol Sci. 2023;24(11):9472. DOI: https://doi.org/10.3390/ijms24119388
24. Seleoglu I, Demirel A. Pulmonary rehabilitation in connective tissue disease-associated interstitial lung disease: a systematic review. Sarcoidosis Vasc Diffuse Lung Dis. 2024;41(4):e2024061.
25. Falasinnu T, Nguyen T, Jiang TE, Chaichian Y, Rector A, Darnall BD, et al. The problem of pain in rheumatology: clinical profiles associated with chronic overlapping pain conditions. ACR Open Rheumatol. 2022;4(10):890-6. DOI: https://doi.org/10.1002/acr2.11488
26. Chew E, Barnado A, Ikizler TA, Zent R, Frech T. Evaluation of hypertension in systemic sclerosis and systemic lupus erythematosus overlap. J Scleroderma Relat Disord. 2023;8(1):14-9. DOI: https://doi.org/10.1177/23971983221122673
27. Kuzuya K, Tsuji S, Matsushita M, Ohshima S, Saeki Y. Systemic sclerosis and systemic lupus erythematosus overlap syndrome with pulmonary arterial hypertension successfully treated with immunosuppressive therapy and riociguat. Cureus. 2019;11(3):e4320. DOI: https://doi.org/10.7759/cureus.4327
28. Padureanu V, Boldeanu V, Albu VC. Neuroinflammation in systemic lupus erythematosus - a review. Rom J Morphol Embryol. 2019;60(3):781-6.
29. Akashi K, Saegusa J, Morinobu A. Progress of autoantibody examinations for connective tissue diseases. Rinsho Byori. 2015;63(5):562-9.
30. Chevalier K, Chassagnon G, Leonard-Louis S, Cohen P, Dunogue B, Regent A, et al. Anti-U1RNP antibodies are associated with a distinct clinical phenotype and worse survival in patients with systemic sclerosis. J Autoimmun. 2024;146:103200. DOI: https://doi.org/10.1016/j.jaut.2024.103220
31. Yu H, Nagafuchi Y, Fujio K. Clinical and immunological biomarkers for systemic lupus erythematosus. Biomolecules. 2021;11(7):995. DOI: https://doi.org/10.3390/biom11070928
32. Aringer M, Petri M. New classification criteria for systemic lupus erythematosus. Curr Opin Rheumatol. 2020;32(6):590-6. DOI: https://doi.org/10.1097/BOR.0000000000000740
33. Di Battista M, Lepri G, Codullo V, Da Rio M, Fiorentini E, Della Rossa A, et al. Systemic sclerosis: one year in review 2023. Clin Exp Rheumatol. 2023;41(8):1567-74. DOI: https://doi.org/10.55563/clinexprheumatol/ki76s5
34. Lai SW, Selvarajah L. The intersection of dermatology and immunology: cutaneous manifestations, autoantibodies and quality of life in connective tissue diseases. Med J Malaysia. 2024;79(5):597- 603.
35. Matatiele P, Tikly M, Tarr G, Gulumian M. DNA methylation similarities in genes of Black South Africans with systemic lupus erythematosus and systemic sclerosis. J Biomed Sci. 2015;22:34. DOI: https://doi.org/10.1186/s12929-015-0142-2
36. Muro Y, Hosono Y, Sugiura K, Ogawa Y, Mimori T, Akiyama M. Anti-PM/Scl antibodies are found in Japanese patients with various systemic autoimmune conditions besides myositis and scleroderma. Arthritis Res Ther. 2015;17:57. DOI: https://doi.org/10.1186/s13075-015-0573-x
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