Polychondritis recidivans is a rare immune-mediated autoimmune, multisystemic disease. It proceeds with recurrent inflammation of both cartilage tissue, mainly the ears, nose, larynx, tracheobronchial tree, ribs and joints, as well as the proteoglycan-rich tissues of the heart valves, blood vessels and eyes. The clinical picture is diverse. Symptoms often overlap with other diseases and diagnosis is delayed. Late diagnosis is the main cause of complications, and life-threatening damage to the affected structures. Polychondritis recidivans may be self-contained or associated with other autoimmune diseases, vasculitis, or hematological disorders. The diagnosis is clinical. The main manifestation of diagnostic value is chondritis with sequential or simultaneous involvement of several cartilage organs. There is no specific laboratory test for the disease. A cornerstone of therapy is glucocorticoids. With severe course, immunosuppressants are also used, and recently biological agents. We describe a case of a 51-year-old woman with repeated recurrences of moving inflammation of the ear cartilage, accompanied by arthritis, severely reduced hearing, hoarseness, conjunctivitis and episcleritis, and CNS symptoms. Blood tests showed leukocytosis and elevated C-reactive protein. Polychondritis recidivans is diagnosed based on the diagnostic criteria of L. P. McAdam et al., J. M. Damiani and H. L. Levine and C. J. Michet et al. Dramatic improvement from methylprednisolone treatment supported the diagnosis. An overview of the epidemiology, pathogenesis, clinical picture, complications, laboratory and imaging diagnostic methods, and treatment of polychondritis recidivans are presented.
| Published in | International Journal of Clinical Dermatology (Volume 6, Issue 2) |
| DOI | 10.11648/j.ijcd.20230602.15 |
| Page(s) | 33-38 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2023. Published by Science Publishing Group |
Polychondritis, Recidivans, Epidemiology, Pathogenesis, Clinical Picture, Paraclinical Studies, Treatment
| [1] | Sharma A, Law AD, Bambery P, et al. Relapsing polychondritis: clinical presentations, disease activity and outcomes. Orphanet J Rare Dis. Dec 20, 2014; 9: 198. |
| [2] | Chauhan K, Surmachevska N, Hanna A. Relapsing Polychondritis. [Update: July 4, 2023]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan. 2023 Jul 4. |
| [3] | Jaksch-Wartenhorst R von. Polychondropathia. Wien Arch Inn Med. 1923; 6: 93-100. |
| [4] | Pearson CM, Kline HM, Newcomer VD. Relapsing Polychondritis. N Engl J Med. 1960; 263: 51-58. |
| [5] | Borgia F, Giuffrida R, Guarneri F, et al. Relapsing polychondritis: an updated review. Biomedicines. 2018; 6(3): 84. doi: 10.3390/biomedicines6030084. |
| [6] | Chen N, Zheng Y. Characteristics and clinical outcomes of 295 patients with relapsing polychondritis. J Rheumatol. 2021; 48(12): 1876–1882. |
| [7] | McAdam LP, O'Hanlan MA, BluestoI1e R, et al. Relapsing polychondritis: prospective study of 23 patients and a review of the literature Medicine (Baltimore). 1976; 55(3): 193-215. |
| [8] | Michet CJ Jr, Mckenna CH, Luthra HS, et al. Relapsing polychondritis: survival and predictive role of early disease manifestations. Ann Intern Med. 1986; 104(1): 74–78. |
| [9] | Longo L, Greco A, Rea A, et al. Relapsing polychondritis: A clinical update. Autoimmun Rev. 2016; 15(6): 539–543. |
| [10] | Vitale A, Sota J, Rigante D, et al. Relapsing polychondritis: an update on pathogenesis, clinical features, diagnostic tools, and therapeutic perspectives. Curr Rheumatol Rep. 2016; 18(1): 3. doi: 10.1007/s11926-015-0549-5. |
| [11] | Rednic S, Damian L, Talarico R, et al. Relapsing polychondritis: state of the art on clinical practice guidelines. RMD Open. 2018; 4 (Suppl 1): e000788. doi: 10.1136/rmdop. |
| [12] | Hazra N, Dregan A, Charlton J, et al. Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study. Rheumatol. 2015; 54(12): 2181–2187. |
| [13] | Horváth A, Páll N, Molnár K, et al. A nationwide study of the epidemiology of relapsing polychondritis. Clin. Epidemiol. 2016; 8: 211–230. |
| [14] | Chopra R, Chaudhary N, Kay J. Relapsing polychondritis. Rheum Dis Clin North Am. 2013; 39(2): 263–276. |
| [15] | Belot A, Duquesne A, Job-Deslandre C, et al. Pediatric-onset relapsing polychondritis: case series and systematic review. J Pediatr. 2010; 156(3): 484–489. |
| [16] | Papo T, Wechsler B, Bletry O, et al. Pregnancy in relapsing polychondritis: Twenty-five pregnancies in eleven patients. Arthritis Rheum. 1997; 40(7): 1245–1249. |
| [17] | Ludvigsson JF, van Vollenhoven R. Prevalence and comorbidity of relapsing polychondritis. Clin Epidemiol. 2016; 8: 361–362. |
| [18] | Gange RW. Relapsing polychondritis. Report of two cases with an immunopathological review. Clin Exp Dermatol. 1976; 1(3): 261-268. |
| [19] | Halber S, Kastor D, Saldeld K. Polychondritis chronica atrophicans. Z Hautkr. 1984; 59(17): 1147-1159. |
| [20] | Foidart JM, Abe S, Martin GR, et al. Antibodies to type II collagen in relapsing polychondritis. N Engl J Med. 1978; 299(22): 1203–1207. |
| [21] | Buckner JH, Wu JJ, Reife RA, et al. Autoreactivity against matrilin-1 in a patient with relapsing polychondritis. Arthritis Rheum. 2000; 43(4): 939–943. |
| [22] | Hu FY, Wang J, Zhang SX, et al. Absolute reduction of peripheral regulatory T cell in patients with relapsing polychondritis, Clin Exper Rheumatol. 2021; 39(3): 487–493. |
| [23] | Stabler T, Piette J-C, Chevalier X, et al. Serum cytokine profiles in relapsing polychondritis suggest monocyte/macrophage activation. Arthr Rheum. 2004; 50(11): 3663–3667. |
| [24] | Arnaud L, Mathian A, Haroche J, et al. Pathogenesis of relapsing polychondritis: A 2013 update. Autoimmun Rev. 2014; 13(2): 90–95. |
| [25] | Yoshida T, Yoshifuji H, Shirakashi M, et al. Risk factors for the recurrence of relapsing polychondritis. Arth Res Ther. 2022; 24(1): 127-130. |
| [26] | Lang B, Rothenfusser A, Lanchbury JS, et al. Susceptibility to relapsing polychondritis is associated with HLA-DR4. Arthritis Rheum. 1993; 36(5): 660–664. |
| [27] | Kingdon J, Roscamp J, Sangle S, et al. Relapsing polychondritis: a clinical review for rheumatologists. Rheumatology. 2017; 57(9): 1525–1532. |
| [28] | Eng J, Sabanathan S. Airway complications in relapsing polychondritis. Ann Thor Surg. 1991; 51(4): 686–692. |
| [29] | Fukuda K, Mizobuchi T, Nakajima I, et al. Ocular involvement in relapsing polychondritis. J Clin Med. 2021; 10(21): 4970. |
| [30] | Francès C, El Rassi R, Laporte JL, et al. Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center. Med (Baltim). 2001; 80(3): 173–179. doi: 10.1097/00005792-200105000-00003. |
| [31] | Lang-Lazdunski L, Hvass U, Paillole C, Pansard Y, Langlois J. Cardiac valve replacement in relapsing polychondritis. A review. J Heart Valve Dis. 1995; 4(3): 227–235. |
| [32] | Selim AG, Fulford LG, Mohiaddin RH, Sheppard MN. Active aortitis in relapsing polychondritis. J Clin Pathol. 2001; 54(11): 890–892. |
| [33] | Requena MA, Palma DA, Popescu BM. Neurologic involvement in relapsing polychondritis. Medicina (B Aires). 2007; 67(2): 156–158. |
| [34] | Kumakiri K, Sakamoto T, Karahashi, T, et al. A case of relapsing polychondritis preceded by inner ear involvement, Auris Nasus Larynx. 2005; 32(1): 71–76. |
| [35] | Swen SJ, Leonards DJ, Swen WA, et al. Reversible cognitive decline in a patient with relapsing polychondritis. Tijdschr Gerontol Geriatr. 2009; 40(5): 203–207. |
| [36] | Chang-Miller A, Okamura M, Torres VE et al. Renal involvement in relapsing polychondritis. Med (Baltim). 1987; 66(3): 202–217. |
| [37] | Piette J-C, El-Rassi R, Amoura Z. Antinuclear antibodies in relapsing polychondritis. Ann Rheum Dis. 1999; 58(10): 656–657. |
| [38] | Smal P, Frankiel S. Relapsing polychondritis. A feature of systemic lupus erythematosus. Arthritis Rheum. 1980; 23(3): 361-363. |
| [39] | Krishnan Y, Grodzinsky AJ. Cartilage diseases. Matrix Biol. 2018 Oct; 71-72: 51-69. |
| [40] | Sidi Y, Weinberger A, Berliner Sh, et al. Relapsing polychondritis associated with mixed cryoglobulinemia. Dermatologica. 1982; 165(1): 66-70. |
| [41] | Herman JH, Dennis MV. Relapsing polychondritis. J Clin Invest. 1973; 52(3): 549-558. |
| [42] | Damiani JM, Levine HL. Relapsing polychondritis–report of ten cases. Laryngoscope. 1979; 89 (6 Pt 1): 929–946. |
| [43] | Okuda S, Hirooka Y, Itami T, et al. FDG-PET/CT and auricular cartilage biopsy are useful for diagnosing with relapsing polychondritis in patients without auricular symptoms. Life. 2021; 11(9): 956. |
| [44] | Ferrada M, Rimland CA, Quinn K, et al. Defining clinical subgroups in relapsing polychondritis: a prospective observational cohort study. Arthritis Rheumatol. 2020; 72(8): 1396–1402. |
| [45] | Dion J, Costedoat-Chalumeau N, Sène D, et al. Relapsing polychondritis can be characterized by three different clinical phenotypes: analysis of a recent series of 142 patients. Arth Rheumatol. 2016; 68(12): 2992–3001. |
| [46] | Shimizu J, Yamano Y, Kawahata K, et al. Relapsing polychondritis patients were divided into three subgroups: patients with respiratory involvement (R subgroup), patients with auricular involvement (A subgroup), and overlapping patients with both involvements (O subgroup), and each group had distinctive clinical characteristics. Medicine. 2018; 97(42): E12837. doi: 10.1097/md.0000000000012837. |
| [47] | Arnaud L, Devilliers H, Peng SL, et al. The Relapsing Polychondritis Disease Activity Index: Development of a disease activity score for relapsing polychondritis. Autoimm Rev. 2012; 12(2): 204–209. |
| [48] | Firestein GS, Gruber HE, Weisman MH et al. Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Am J Med. 1985; 79: 69-72. |
| [49] | Mark KA, Franks AG. Colchicine and indomethacin for the treatment of relapsing polychondritis. J Am Acad Dermatol. 2002; 46: S22-4. |
| [50] | Barranco VP, Minor DB, Solonom H. Treatment of polychondritis with dapsone. Arch DermatoI. 1976; 112: 1286-1288. |
| [51] | Petitdemange A, Sztejkowski C, Damian L, et al. Treatment of relapsing polychondritis: a systematic review. Clin Exp Rheumatol. 2022; 40 Suppl 134(5): 81-85. |
| [52] | Lekpa FK, Chevalier X. Refractory relapsing polychondritis: challenges and solutions. Open Access Rheumatol. 2018; 10: 1–11. |
| [53] | Padoan R, Campaniello D, Iorio L, et al. Biologic therapy in relapsing polychondritis: navigating between options. Expert Opin Biol Ther. 2022; 22(5): 661-671. |
| [54] | Shah R, Alford EL. Reconstructive rhinoplasty using cadaver cartilage in relapsing polychondritis. Proc (Bayl Univ Med Cent). 2022; 36(1): 130-131. |
| [55] | Handa H, Ooka S, Shimizu J, et al. Evaluation of airway involvement and treatment in patients with relapsing polychondritis. Sci Rep. 2023; 13(1): 8307. doi: 10.1038/s41598-023-35616-4. |
| [56] | Dib C, Moustafa SE, Mookadam M, et al. Surgical treatment of the cardiac manifestations of relapsing polychondritis: overview of 33 patients identified through literature review and the Mayo Clinic records. Mayo Clinic Proc. 2006; 81: 772–776. doi: 10.4065/81.6.772. |
APA Style
Yungareva, I., Broshtilova, V., Trenovski, A., Velevska, Y., Vatov, P., et al. (2023). Polychondritis Recidivans – A Case Report with a Review of the Literature. International Journal of Clinical Dermatology, 6(2), 33-38. https://doi.org/10.11648/j.ijcd.20230602.15
ACS Style
Yungareva, I.; Broshtilova, V.; Trenovski, A.; Velevska, Y.; Vatov, P., et al. Polychondritis Recidivans – A Case Report with a Review of the Literature. Int. J. Clin. Dermatol. 2023, 6(2), 33-38. doi: 10.11648/j.ijcd.20230602.15
AMA Style
Yungareva I, Broshtilova V, Trenovski A, Velevska Y, Vatov P, et al. Polychondritis Recidivans – A Case Report with a Review of the Literature. Int J Clin Dermatol. 2023;6(2):33-38. doi: 10.11648/j.ijcd.20230602.15
Share This Article
Twitter
Linked In
Facebook
Copy
Download@article{10.11648/j.ijcd.20230602.15,
author = {Irina Yungareva and Valentina Broshtilova and Aleksandar Trenovski and Yoanna Velevska and Petar Vatov and Sonya Marina},
title = {Polychondritis Recidivans – A Case Report with a Review of the Literature},
journal = {International Journal of Clinical Dermatology},
volume = {6},
number = {2},
pages = {33-38},
doi = {10.11648/j.ijcd.20230602.15},
url = {https://doi.org/10.11648/j.ijcd.20230602.15},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijcd.20230602.15},
abstract = {Polychondritis recidivans is a rare immune-mediated autoimmune, multisystemic disease. It proceeds with recurrent inflammation of both cartilage tissue, mainly the ears, nose, larynx, tracheobronchial tree, ribs and joints, as well as the proteoglycan-rich tissues of the heart valves, blood vessels and eyes. The clinical picture is diverse. Symptoms often overlap with other diseases and diagnosis is delayed. Late diagnosis is the main cause of complications, and life-threatening damage to the affected structures. Polychondritis recidivans may be self-contained or associated with other autoimmune diseases, vasculitis, or hematological disorders. The diagnosis is clinical. The main manifestation of diagnostic value is chondritis with sequential or simultaneous involvement of several cartilage organs. There is no specific laboratory test for the disease. A cornerstone of therapy is glucocorticoids. With severe course, immunosuppressants are also used, and recently biological agents. We describe a case of a 51-year-old woman with repeated recurrences of moving inflammation of the ear cartilage, accompanied by arthritis, severely reduced hearing, hoarseness, conjunctivitis and episcleritis, and CNS symptoms. Blood tests showed leukocytosis and elevated C-reactive protein. Polychondritis recidivans is diagnosed based on the diagnostic criteria of L. P. McAdam et al., J. M. Damiani and H. L. Levine and C. J. Michet et al. Dramatic improvement from methylprednisolone treatment supported the diagnosis. An overview of the epidemiology, pathogenesis, clinical picture, complications, laboratory and imaging diagnostic methods, and treatment of polychondritis recidivans are presented.
},
year = {2023}
}
TY - JOUR T1 - Polychondritis Recidivans – A Case Report with a Review of the Literature AU - Irina Yungareva AU - Valentina Broshtilova AU - Aleksandar Trenovski AU - Yoanna Velevska AU - Petar Vatov AU - Sonya Marina Y1 - 2023/12/11 PY - 2023 N1 - https://doi.org/10.11648/j.ijcd.20230602.15 DO - 10.11648/j.ijcd.20230602.15 T2 - International Journal of Clinical Dermatology JF - International Journal of Clinical Dermatology JO - International Journal of Clinical Dermatology SP - 33 EP - 38 PB - Science Publishing Group SN - 2995-1305 UR - https://doi.org/10.11648/j.ijcd.20230602.15 AB - Polychondritis recidivans is a rare immune-mediated autoimmune, multisystemic disease. It proceeds with recurrent inflammation of both cartilage tissue, mainly the ears, nose, larynx, tracheobronchial tree, ribs and joints, as well as the proteoglycan-rich tissues of the heart valves, blood vessels and eyes. The clinical picture is diverse. Symptoms often overlap with other diseases and diagnosis is delayed. Late diagnosis is the main cause of complications, and life-threatening damage to the affected structures. Polychondritis recidivans may be self-contained or associated with other autoimmune diseases, vasculitis, or hematological disorders. The diagnosis is clinical. The main manifestation of diagnostic value is chondritis with sequential or simultaneous involvement of several cartilage organs. There is no specific laboratory test for the disease. A cornerstone of therapy is glucocorticoids. With severe course, immunosuppressants are also used, and recently biological agents. We describe a case of a 51-year-old woman with repeated recurrences of moving inflammation of the ear cartilage, accompanied by arthritis, severely reduced hearing, hoarseness, conjunctivitis and episcleritis, and CNS symptoms. Blood tests showed leukocytosis and elevated C-reactive protein. Polychondritis recidivans is diagnosed based on the diagnostic criteria of L. P. McAdam et al., J. M. Damiani and H. L. Levine and C. J. Michet et al. Dramatic improvement from methylprednisolone treatment supported the diagnosis. An overview of the epidemiology, pathogenesis, clinical picture, complications, laboratory and imaging diagnostic methods, and treatment of polychondritis recidivans are presented. VL - 6 IS - 2 ER -
Department of Skin and Venereal Diseases, Medical Institute of the Ministry of Internal Affairs, Sofia, Bulgaria
Information