Abstract
Relapsing polychondritis (RP) is a rare systemic inflammatory disease primarily affecting the ears, nose and tracheobronchial tree cartilage, but also the cardiovascular system. Cardiovascular complications are the second cause of mortality in RP. We report the case of a woman with a corticosteroid-resistant RP-associated aortitis, who was successfully treated with tocilizumab (TCZ). The FDG-PET/CT was a useful tool for diagnosing aortitis and assessing the effect of biotherapy. We conducted a systematic literature review confirming this is the first case of rapid and sustained remission in a patient with corticosteroid-resistant RP-associated aortitis after TCZ treatment administered as a first-line immunotherapy. However, further studies are needed to confirm the beneficial effect of TCZ used in this life-threatening condition.
Similar content being viewed by others
References
Le Besnerais M, Arnaud L, Boutémy J et al (2017) Aortic involvement in relapsing polychondritis. Jt Bone Spine Rev Rhum. doi:10.1016/j.jbspin.2017.05.009
Mathian A, Miyara M, Cohen-Aubart F et al (2016) Relapsing polychondritis: a 2016 update on clinical features, diagnostic tools, treatment and biological drug use. Best Pract Res Clin Rheumatol 30:316–333. doi:10.1016/j.berh.2016.08.001
Gasparyan AY, Ayvazyan L, Blackmore H, Kitas GD (2011) Writing a narrative biomedical review: considerations for authors, peer reviewers, and editors. Rheumatol Int 31:1409–1417. doi:10.1007/s00296-011-1999-3
Hazra N, Dregan A, Charlton J et al (2015) Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study. Rheumatology 54:2181–2187. doi:10.1093/rheumatology/kev240
Michet CJ, McKenna CH, Luthra HS, O’Fallon WM (1986) Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med 104:74–78
Del Rosso A, Petix NR, Pratesi M, Bini A (1997) Cardiovascular involvement in relapsing polychondritis. Semin Arthritis Rheum 26:840–844
Surgical treatment of the cardiac manifestations of relapsing polychondritis: overview of 33 patients identified through literature review and the mayo clinic records - Mayo Clinic Proceedings. http://www.mayoclinicproceedings.org/article/S0025-6196(11)61731-X/pdf. Accessed 24 July 2017
Shimizu J, Oka H, Yamano Y et al (2016) Cardiac involvement in relapsing polychondritis in Japan. Rheumatol Oxf Engl 55:583–584. doi:10.1093/rheumatology/kev320
Rousselin C, Pontana F, Puech P, Lambert M (2016) Differential diagnosis of aortitis. Rev Med Int 37:256–263. doi:10.1016/j.revmed.2016.02.002
Arnaud L, Mathian A, Haroche J et al (2014) Pathogenesis of relapsing polychondritis: a 2013 update. Autoimmun Rev 13:90–95. doi:10.1016/j.autrev.2013.07.005
Ouchi N, Uzuki M, Kamataki A et al (2011) Cartilage destruction is partly induced by the internal proteolytic enzymes and apoptotic phenomenon of chondrocytes in relapsing polychondritis. J Rheumatol. doi:10.3899/jrheum.101044
Stabler T, Piette J-C, Chevalier X et al (2004) Serum cytokine profiles in relapsing polychondritis suggest monocyte/macrophage activation. Arthritis Rheum 50:3663–3667. doi:10.1002/art.20613
Ohwatari R, Fukuda S, Iwabuchi K et al (2001) Serum level of macrophage migration inhibitory factor as a useful parameter of clinical course in patients with Wegener’s granulomatosis and relapsing polychondritis. Ann Otol Rhinol Laryngol 110:1035–1040. doi:10.1177/000348940111001108
Wallace ZS, Stone JH (2013) Refractory relapsing polychondritis treated with serial success with interleukin 6 receptor blockade. J Rheumatol 40:100–101. doi:10.3899/jrheum.120381
Kawai M, Hagihara K, Hirano T et al (2009) Sustained response to tocilizumab, anti-interleukin-6 receptor antibody, in two patients with refractory relapsing polychondritis. Rheumatol Oxf Engl 48:318–319. doi:10.1093/rheumatology/ken468
Yamashita H, Takahashi H, Kubota K et al (2014) Utility of fluorodeoxyglucose positron emission tomography/computed tomography for early diagnosis and evaluation of disease activity of relapsing polychondritis: a case series and literature review. Rheumatology 53:1482–1490. doi:10.1093/rheumatology/keu147
Lei W, Zeng H, Zeng D-X et al (2016) (18)F-FDG PET-CT: a powerful tool for the diagnosis and treatment of relapsing polychondritis. Br J Radiol 89:20150695. doi:10.1259/bjr.20150695
Wang J, Li S, Zeng Y et al (2014) 18F-FDG PET/CT is a valuable tool for relapsing polychondritis diagnose and therapeutic response monitoring. Ann Nucl Med 28:276–284. doi:10.1007/s12149-014-0805-1
Soussan M, Abisror N, Abad S et al (2014) FDG-PET/CT in patients with ANCA-associated vasculitis: case-series and literature review. Autoimmun Rev 13:125–131. doi:10.1016/j.autrev.2013.09.009
Selim AG, Fulford LG, Mohiaddin RH, Sheppard MN (2001) Active aortitis in relapsing polychondritis. J Clin Pathol 54:890–892
Rho YH, Choi SJ, Choi YS et al (2005) Relapsing polychondritis with aortitis without valvular involvement. J Rheumatol 32:954–956
Yung A, Charleson HA, Ullal R, Doube A (2000) A case of relapsing polychondritis with coronary ostial stenosis and severe aortic incompetence. Semin Arthritis Rheum 30:144–146
Walker UA, Weiner SM, Vaith P et al (1998) Aortitis in relapsing polychondritis. Br J Rheumatol 37:1359–1361
Barretto SN, Oliveira GH, Michet CJ et al (2002) Multiple cardiovascular complications in a patient with relapsing polychondritis. Mayo Clin Proc 77:971–974. doi:10.4065/77.9.971
Sugrue G, Durcan L, Bell L et al (2014) Unsuspected cardiovascular involvement in relapsing polychondritis: a case of aortitis with critical coronary artery stenosis secondary to relapsing polychondritis. Circ Cardiovasc Imaging 7:409–411. doi:10.1161/CIRCIMAGING.113.001290
Marie I, Lahaxe L, Josse S, Levesque H (2009) Sustained response to infliximab in a patient with relapsing polychondritis with aortic involvement. Rheumatology 48:1328–1329. doi:10.1093/rheumatology/kep224
Seymour MW, Home DM, Williams RO, Allard SA (2007) Prolonged response to anti-tumour necrosis factor treatment with adalimumab (Humira) in relapsing polychondritis complicated by aortitis. Rheumatology 46:1738–1739. doi:10.1093/rheumatology/kem229
Sallés M, Mínguez S, Ros S et al (2017) Sustained response to tocilizumab in a patient with relapsing polychondritis complicated by aortitis. Clin Exp Rheumatol 35(Suppl 103):223
Stael R, Smith V, Wittoek R et al (2015) Sustained response to tocilizumab in a patient with relapsing polychondritis with aortic involvement: a case based review. Clin Rheumatol 34:189–193. doi:10.1007/s10067-014-2670-7
Narshi CB, Allard SA (2012) Sustained response to tocilizumab, anti-IL-6 antibody, following anti-TNF-α failure in a patient with relapsing polychondritis complicated by aortitis. Rheumatology 51:952–953. doi:10.1093/rheumatology/ker451
Loricera J, Blanco R, Castañeda S et al (2014) Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol 32:S79–S89
Risse J, Mandry D, Settembre N et al (2016) Dramatic Response to Tocilizumab Before Emergency Surgery in Severe Active Takayasu Disease. Circ Cardiovasc Imaging. doi:10.1161/CIRCIMAGING.116.004819
Acknowledgements
We thank Dr Jonathan London (Department of Internal Medicine, Hospital Cochin, Université Paris-5, Paris, France) for his critical review of the manuscript.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare no conflict of interest.
Financial support statement
None.
Rights and permissions
About this article
Cite this article
Elourimi, G., Soussan, M., Warzocha, U. et al. Efficacy of tocilizumab highlighted by FDG-PET/CT in a patient with relapsing polychondritis-associated aortitis. Rheumatol Int 37, 1931–1935 (2017). https://doi.org/10.1007/s00296-017-3832-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-017-3832-0