Progressive Pulmonary Fibrosis Secondary to Rhupus Syndrome: Diagnostic and Therapeutic Challenges. Case Report and Narrative Literature Review
Resumen
Introducción: el síndrome de Rhupus representa una entidad poco frecuente de superposición entre lupus eritematoso sistémico y artritis reumatoide. La afectación pulmonar intersticial asociada puede evolucionar hacia fibrosis pulmonar progresiva (FPP), con impacto significativo en el pronóstico.
Método: se realizó un reporte de caso clínico acompañado de una revisión narrativa de la literatura, siguiendo las guías CARE.
Caso clínico: se describe el caso de una paciente adulta con diagnóstico de síndrome de Rhupus que desarrolló enfermedad pulmonar intersticial fibrosante con comportamiento progresivo, documentada mediante tomografía computarizada de alta resolución y deterioro funcional respiratorio, requiriendo un abordaje terapéutico multidisciplinario.
Discusión: la fibrosis pulmonar asociada a enfermedades autoinmunes plantea importantes desafíos diagnósticos y terapéuticos. El reconocimiento temprano del fenotipo progresivo permite considerar estrategias antifibróticas además del manejo inmunomodulador.
Conclusión: la fibrosis pulmonar progresiva puede constituir una manifestación grave del síndrome de Rhupus. Un enfoque integral y precoz es fundamental para optimizar los resultados clínicos.
Abstract
Introduction: Rhupus syndrome is a rare overlap between systemic lupus erythematosus and rheumatoid arthritis. Associated interstitial lung disease may progress to progressive pulmonary fibrosis (PPF), significantly affecting prognosis.
Method: A clinical case report with a narrative literature review was conducted following the CARE guidelines.
Case report: We report a patient with Rhupus syndrome who developed progressive fibrosing interstitial lung disease, confirmed by high-resolution computed tomography and pulmonary function decline, requiring a multidisciplinary therapeutic approach.
Discussion: Autoimmune-related pulmonary fibrosis presents diagnostic and therapeutic challenges. Early recognition of a progressive phenotype supports consideration of antifibrotic therapy alongside immunomodulation.
Conclusion: Progressive pulmonary fibrosis may represent a severe manifestation of Rhupus syndrome. Early diagnosis and comprehensive management are essential.
Abstract
Introduction: Rhupus syndrome is a rare overlap between systemic lupus erythematosus and rheumatoid arthritis. Associated interstitial lung disease may progress to progressive pulmonary fibrosis (PPF), significantly affecting prognosis.
Method: A clinical case report with a narrative literature review was conducted following the CARE guidelines.
Case report: We report a patient with Rhupus syndrome who developed progressive fibrosing interstitial lung disease, confirmed by high-resolution computed tomography and pulmonary function decline, requiring a multidisciplinary therapeutic approach.
Discussion: Autoimmune-related pulmonary fibrosis presents diagnostic and therapeutic challenges. Early recognition of a progressive phenotype supports consideration of antifibrotic therapy alongside immunomodulation.
Conclusion: Progressive pulmonary fibrosis may represent a severe manifestation of Rhupus syndrome. Early diagnosis and comprehensive management are essential.
Palabras clave:
síndrome de Rhupus, fibrosis pulmonar progresiva, enfermedad pulmonar intersticial, enfermedades autoinmunes, caso clínicoKeywords:
Rhupus syndrome, progressive pulmonary fibrosis, interstitial lung disease, autoimmune diseases, antifibrotic therapy, case reportReferences
1. Solis Cartas U, Martínez Larrarte JP, Prada Hernández DM, Gómez Morejón JA, Valdés González JL, Molinero Rodríguez C. Rhupus syndrome: a rare combination. Rev Colomb Reumatol. 2017;24(4):237–41. doi: 10.1016/j.rcreu.2017.06.005.
2. Upadhyaya S, Agarwal M, Upadhyaya A, Pathania M, Dhar M. Rhupus syndrome: a diagnostic dilemma. Cureus. 2022 Sep 11;14(9):e29068. doi: 10.7759/cureus.29068.
3. Ahsan H. Rhupus: dual rheumatic disease. J Immunoassay Immunochem. 2022;43(2):119–28. doi: 10.1080/15321819.2021.2019383.
4. Drie T, Khalayli N, Haidar G, Haj Ali D. Rhupus syndrome: a case report of a rare combination. Ann Med Surg (Lond). 2024;86(1):535. doi: 10.1016/j.amsu.2023.105535.
5. Antonini L, Le Mauff B, Marcelli C, Aouba A, de Boysson H. Rhupus: a systematic literature review. Autoimmun Rev. 2020;19(9):102612. doi: 10.1016/j.autrev.2020.102612.
6. Iaccarino L, Gatto M, Bettio S, Caso F, Rampudda M, Zen M, et al. Overlap connective tissue disease syndromes. Autoimmun Rev. 2013;12(3):363–73. doi: 10.1016/j.autrev.2012.06.004.
7. Kang HK, Song JW. Progressive pulmonary fibrosis: where are we now? Tuberc Respir Dis (Seoul). 2023;87(2):123–33. doi: 10.4046/trd.2022.0117.
8. Duman D. Progressive pulmonary fibrosis. Tuberk Toraks. 2022;70(4):375–81. doi: 10.5578/tt.20229910.
9. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718–27. doi: 10.1056/NEJMoa1908681.
10. Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022;205(9):e18–47. doi: 10.1164/rccm.202202-0399ST.
11. Diouf OBK, Dieng M, Ndour MA, Ndiaye M, Tarsa NM, Djiba B, et al. Rhupus: the association of systemic lupus erythematosus and rheumatoid arthritis. Open J Rheumatol Autoimmune Dis. 2024;14(2):84–8.
12. Kulkarni PV, Rathod JR, Kamble AA, Chintale KN, Bhattacharya MA. Overlap syndrome of systemic lupus erythematosus and rheumatoid arthritis (rhupus) with systemic involvement: a rare case report. Int J Med Res Rev. 2021;9(6):395–8.
13. Venugopal V, Yelamanchi SS, Vijayakumari V, Rao VM. Rhupus syndrome presenting as interstitial lung disease: a case report. J Clin Diagn Res. 2021;15(2):OD04–6. doi: 10.7860/JCDR/2021/47048.14563.
14. Khurshid F, Islam A. The enigma of rhupus syndrome: a case report on systemic lupus erythematosus and rheumatoid arthritis convergence. J Tradit Med Chin Med. 2024;1–3.
15. Kay J, Upchurch KS. ACR/EULAR 2010 rheumatoid arthritis classification criteria. Rheumatology (Oxford). 2012;51(Suppl 6):vi5–9. doi: 10.1093/rheumatology/kes279.
16. Aringer M, Costenbader KH, Daikh DI, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 EULAR/ACR classification criteria for systemic lupus erythematosus. Arthritis Rheumatol. 2019;71(9):1400–12. doi: 10.1002/art.40930.
Downloads
License
Copyright (c) 2026 Revista Neumos
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.