Aortic involvement in a patient with relapsing polychondritis. Clinical observation
https://doi.org/10.47360/1995-4484-2026-202-207
Abstract
Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by inflammation and destruction of cartilage in various organs. Aortic involvement in RP is relatively rare, occurring in 6.7–9.7% of cases, and is associated with a high risk of mortality (27.3–41.3%) and surgical interventions (65%). Typically, cardiac and aortic involvement in patients with RP is identified approximately five years after the onset of the first symptoms, with 19% of cases being asymptomatic. There are currently no universally accepted standards for screening, diagnosis, and treatment of aortic involvement in RP.
We present a clinical case of a patient with RP, aortic involvement, and the development of an aneurysm, who underwent sequential therapy with cyclophosphamide and rituximab. This treatment approach allowed for the control of disease activity and a reduction in glucocorticoid dosage.
About the Authors
D. A. Dibrov
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
Competing Interests:
Russian Federation
Danil A. Dibrov
115522, Moscow, Kashirskoye Highway, 34A
Competing Interests:
none
T. M. Reshetnyak
V.A. Nasonova Research Institute of Rheumatology; Russian Medical Academy of Continuous Professional Education of the Ministry of Healthcare of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Tatiana M. Reshetnyak
115522, Moscow, Kashirskoye Highway, 34A; 125993, Moscow, Barrikadnaya str., 2/1, building 1
Competing Interests:
none
N. V. Demidova
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
Competing Interests:
Russian Federation
Natalia V. Demidova
115522, Moscow, Kashirskoye Highway, 34A
Competing Interests:
none
A. V. Alekseeva
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
Competing Interests:
Russian Federation
Anna V. Alekseeva
115522, Moscow, Kashirskoye Highway, 34A
Competing Interests:
none
L. M. Blank
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
Competing Interests:
Russian Federation
Leonid M. Blank
115522, Moscow, Kashirskoye Highway, 34A
Competing Interests:
none
References
1. Lekpa FK, Chevalier X. Refractory relapsing polychondritis: Challenges and solutions. Open Access Rheumatol. 2018;10:1-11. doi: 10.2147/OARRR.S142892
2. Borgia F, Giuffrida R, Guarneri F, Cannavo SP. Relapsing polychondritis: An updated review. Biomedicines. 2018;6(3):84. doi: 10.3390/biomedicines6030084
3. Denisov LN, Vinogradova IB, Bakhtina LA. Recurrent polychondritis: A review of the literature and a clinical case description. Modern Rheumatology Journal. 2023;17(1):83-88 (In Russ.). doi: 10.14412/1996-7012-2023-1-83-88
4. Lahmer T, Treiber M, von Werder A, Foerger F, Knopf A, Heemann U, et al. Relapsing polychondritis: An autoimmune disease with many faces. Autoimmun Rev. 2010;9(8):540-546. doi: 10.1016/j.autrev.2010.02.016
5. Shimizu J, Oka H, Yamano Y, Yudoh K, Suzuki N. Cardiac involvement in relapsing polychondritis in Japan. Rheumatology (Oxford) 2016;55(3):583-584. doi: 10.1093/rheumatology/kev320
6. D’Cruz DP, Ferrada MA. Relapsing polychondritis and large-vessel vasculitis. J Rheumatol. 2020;47(12):1732-1733. doi: 10.3899/jrheum.200083
7. Frances C, el Rassi R, Laporte JL, Rybojad M, Papo T, Piette JC. Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center. Medicine (Baltimore). 2001;80(3):173-179. doi: 10.1097/00005792-200105000-00003
8. Mertz P, Sparks J, Kobrin D, Ogbonnaya SA, Sevim A, Michet C, et al. Relapsing polychondritis: Best practice & clinical rheumatology. Best Pract Res Clin Rheumatol. 2023;37(1):101867. doi: 10.1016/j.berh.2023.101867
9. Lang B, Rothenfusser A, Lanchbury JS, Rauh G, Breedveld FC, Urlacher A, et al. Susceptibility to relapsing polychondritis is associated with HLA-DR4. Arthritis Rheum. 1993;36(5):660-664. doi: 10.1002/art.1780360513
10. Zeuner M, Straub RH, Rauh G, Albert ED, Scholmerich J, Lang B. Relapsing polychondritis: Clinical and immunogenetic analysis of 62 patients. J Rheumatol. 1997;24(1):96-101.
11. Terao C, Yoshifuji H, Yamano Y, Kojima H, Yurugi K, Miura Y, et al. Genotyping of relapsing polychondritis identified novel susceptibility HLA alleles and distinct genetic characteristics from other rheumatic diseases. Rheumatology (Oxford). 2016;55(9):16861692. doi: 10.1093/rheumatology/kew233
12. Beck DB, Ferrada MA, Sikora KA, Ombrello AK, Collins JC, Pei W, et al. Somatic Mutations in UBA1 and severe adult-onset autoinflammatory disease. N Engl J Med. 2020;383(27):2628-2638. doi: 10.1056/NEJMoa2026834
13. Chaltsev BD, Torgashina AV, Lila AM, Markova TV, Kutsev SI, Ryzhkova OP, et al. VEXAS syndrome: On the threshold of changing perceptions of known diseases. Modern Rheumatology Journal. 2023;17(6):92-101 (In Russ.)] doi: 10.14412/1996-7012-2023-6-92-101
14. Shimizu J, Murayama MA, Mizukami Y, Arimitsu N, Takai K, Miyabe Y. Innate immune responses in Behcet disease and relapsing polychondritis. Front Med (Lausanne). 2023;10:1055753. doi: 10.3389/fmed.2023.1055753
15. Liu Y, Li X, Cheng L, Zhan H, Huang Y, Li H, et al. Progress and challenges in the use of blood biomarkers in relapsing polychondritis. Clin Exp Immunol. 2023;212(3):199-211. doi: 10.1093/cei/uxad014
16. Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004;16(1):56-61. doi: 10.1097/00002281200401000-00011
17. Hazra N, Dregan A, Charlton J, Gulliford MC, D’Cruz DP. Incidence and mortality of relapsing polychondritis in the UK: A population-based cohort study. Rheumatology (Oxford). 2015;54(12):2181-2187. doi: 10.1093/rheumatology/kev240
18. Horváth A, Páll N, Molnár K, Kováts T, Surján G, Vicsek T, et al. A nationwide study of the epidemiology of relapsing polychondritis. Clin Epidemiol. 2016;8:211-230. doi: 10.2147/CLEP.S91439
19. Mathew SD, Battafarano DF, Morris MJ. Relapsing polychondritis in the Department of Defense population and review of the literature. Semin Arthritis Rheum. 2012;42(1):70-83. doi: 10.1016/j.semarthrit.2011.12.007
20. Le Besnerais M, Arnaud L, Boutémy J, Bienvenu B, Lévesque H, Amoura Z, et al. Aortic involvement in relapsing polychondritis. Joint Bone Spine. 2018;85(3):345-351. doi: 10.1016/j.jbspin.2017.05.009
21. Erdogan M, Esatoglu SN, Hatemi G, Hamuryudan V. Aortic involvement in relapsing polychondritis: Case-based review. Rheumatol Int. 2021;41(4):827-837. doi: 10.1007/s00296-01904468-5
22. Luo Y, Bolek EC, Quinn KA, Wells K, Rose E, Sikora K, et al. A prospective observational cohort study and systematic review of 40 patients with mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome. Semin Arthritis Rheum. 2022;52:151924. doi: 10.1016/j.semarthrit.2021.10.007
23. Stein JD, Lee P, Kuriya B, Tenenbaum J, Daniel LB, Butany J, et al. Critical coronary artery stenosis and aortitis in a patient with relapsing polychondritis. J Rheumatol. 2008;35(9):1898-900
24. Vaidyanathan RK, Byalal JR, Sundaramoorthi T, Sankar NM, Thomas JM, Meenakshi NA, et al. Rapidly progressive coronary ostial stenosis after aortic valve replacement in relapsing polychondritis. J Thorac Cardiovasc Surg. 2006;131(6):1395-1396. doi: 10.1016/j.jtcvs.2006.01.032
25. Dormoy L, Belin A, Labombarda F. Heart transplantation as last resort treatment for relapsing polychondritis with severe cardiac involvement. Prog Transplant. 2017;27(3):321-323. doi: 10.1177/1526924817715464
26. Puechal X, Terrier B, Mouthon L, Costedoat-Chalumeau N, Guillevin L, Le Jeunne C. Relapsing polychondritis. Joint Bone Spine. 2014;81(2):118-124. doi: 10.1016/j.jbspin.2014.01.001
Review
For citations:
Dibrov D.A., Reshetnyak T.M., Demidova N.V., Alekseeva A.V., Blank L.M. Aortic involvement in a patient with relapsing polychondritis. Clinical observation. Rheumatology Science and Practice. 2026;64(2):202-207. (In Russ.) https://doi.org/10.47360/1995-4484-2026-202-207
Views:
184
JATS XML
Email this article 