Relapsing Polychondritis: Problems and Solutions

DOI: 10.47026/2413-4864-2024-2-64-77

UDC: 616.72
BBC: 53.4

Zhuravleva N.V., Dibrov D.A., Ivanova A.V., Aksenova A.V., Guryanova E.A., Pushkina N.S., Smirnova T.L.

Relapsing Polychondritis: Problems and Solutions

Keywords: recurrent polychondritis, nonsteroidal anti-inflammatory drugs, corticosteroids, immunosuppressants, biological drugs, dapsone

The purpose of this study is to evaluate the clinical course and effectiveness of pharmacological therapy in a patient with recurrent polychondritis. Material and methods. Retrospective and prospective analyses of the diagnosis, course and treatment of recurrent polychondritis in the patient were performed. The patient was diagnosed based on the McAdam classification criteria. The patient was assigned laboratory tests: general, biochemical and immunological blood analyses; instrumental methods: X-ray and ultrasound examinations of the vessels, spirometry. The results of the study. The patient was first diagnosed with recurrent polychondritis at the age of 58. The patient complained of pain, swelling and hyperemia of the auricles, a change in the shape of the nose; attacks of suffocation, which were stopped by inhalations of salbutamol, hoarseness of voice; prolonged subfebrility (37.5–38°C), arthralgia. In September 2022, he was examined by an otolaryngologist, then sent to a rheumatologist to determine therapeutic and diagnostic tactics. According to the results of laboratory data: erythrocyte sedimentation rate – 48 mm/hour, C-reactive protein – 30.1 mg/l, rheumatoid factor – 10 units/l, antibodies to cyclic citrulline peptide- 7.0 units, the result of immunoblot is negative; antibodies to myeloperoxidase – 4.15 RU/ml, antibodies to proteinase – 32.2 RU/ml. During spirometry, there was a violation of the function of external respiration of an obstructive type. Ultrasound of the joints: signs of synovitis of the proximal interphalangeal joints of both hands. Based on the McAdam classification criteria, a clinical diagnosis was made: recurrent polychondritis with systemic manifestations: chondritis of the auricles and nasal congestion, seronegative arthritis, bronchoobstructive syndrome, constitutional symptom (fever). The patient was prescribed therapy: prednisone 20 mg/day, methotrexate 25 mg/week. Clinical and laboratory remission was achieved, but subsequently relapses of the disease and the appearance of a herpes infection were observed (antiviral therapy was prescribed). In July 2023, he was sent for examination and treatment at the Nasonova Research Institute of Rheumatology. The patient was submitted to the commission on therapy with genetically engineered biological drugs. Taking into account the often recurrent herpetic infection, the appointment of genetically engineered biological drugs was postponed. It is recommended to continue taking prednisolone at a dose of 20 mg/day, antiviral therapy (valciclovir). Dapsone 100 mg per day was added to the treatment, but his patient independently canceled the drug due to the appearance of severe nausea and insomnia. Subsequently, a rheumatologist with an immunosuppressive purpose prescribed azathiroprine 150 mg/day, continued taking prednisone 20 mg/day, however, exacerbations of the disease were registered. The lack of effect of the therapy was the basis for the patient's re-hospitalization at the V.A. Nasonova Research Institute of Rheumatology. Conclusions. Recurrent polychondritis is a rapidly progressive disease, and in the absence of timely diagnosis and treatment, it has a risk of developing life-threatening conditions.

References

Chernyak V.I., Savel’ev A.I., Men’shikova I.V. Porazhenie nervnoi sistemy pri retsidiviruyushchem polikhondrite [Nervous system lesions associated with relapsing polychondritis]. Klinicheskaya medicina, 2016, vol. 94, no. 2, pp. 108–113.
Chernjak V.I., Savel’ev A.I., Pogromov A.P. Recidiviruyushchii polikhondrit [Relapsing polychondritis]. Klinicheskaya medicina, 2014, vol. 92, no. 11, pp. 22–28.
Alexis, Makoto M., Fleur C.A. et al. Relapsing polychondritis: A 2016 update on clinical features, diagnostic tools, treatment and biological drug use. Best Pract Res Clin Rheumatol, 2016, vol. 30, no. 2, pp. 316–333. DOI: 10.1016/j.berh.2016.08.001.
Aman S., Karthik G., Kusum S. et al. Relapsing polychondritis: a review. Clin Rheumatol, 2013, vol. 32, no. 11, pp. 1575–1583. DOI: 10.1007/s10067-013-2328-x.
Arnaud L., Costedoat-Chalumeau N., Mathian A. et al. French practical guidelines for the diagnosis and management of relapsing polychondritis. Rev Med Interne, 2023, vol. 44, no. 6, pp. 282– DOI: 10.1016/j.revmed.2023.05.005.
Arthur P., Cédric S., Laura D. et al. Treatment of relapsing polychondritis: a systematic review. Clin Exp Rheumatol, 2022, vol. 40, suppl. 134, no. 5, pp. 81–85. DOI: 10.55563/clinexprheumatol/h9gq1o.
Bachor E., Blevins N.H., Karmody C. et al. Otologic manifestations of relapsing polychondritis. Review of literature and report of nine cases. Auris Nasus Larynx, 2006, vol. 33, no. 2, pp. 135–141. DOI: 10.1016/j.anl.2005.11.020.
Barranco V.P., Minor D.B., Soloman H. Treatment of relapsing polychondritis with dapsone. Arch Dermatol, 1976, vol. 112, no. 9, pp. 1286–
Biro C., Köhler J. Polychondritis. Dtsch Arztebl Int. 2020, vol. 117, no. 35–36, p. 582DOI: 10.3238/ARZTEBL.2020.0582.
Damiani J.M., Levine H.L. Relapsing polychondritis-report of ten cases. Laryngoscope, 1979, vol. 89, no. 6, pt. 1, pp. 929–
Dion J., Costedoat-Chalumeau N., Sène D. et al. Relapsing polychondritis can be characterized by 3 different clinical phenotypes: analysis of a series of 142 patients. Arthritis Rheumatol, 2016, vol. 68, no. 12, pp. 2992–3001. DOI: 10.1002/art.39790.
Erik L., Panayotis Z., Stefanos B. et al. Relapsing polychondritis: a clinical review. Semin Arthritis Rheum, 2002, vol. 31(6), pp. 384– DOI: 10.1053/sarh.2002.32586.
Ferrada M.A., Sikora K.A., Luo Y. et al. Somatic Mutations in UBA1 Define a Distinct Subset of Relapsing Polychondritis Patients With VEXAS. Arthritis Rheumatol, 2021, vol. 73(10), pp. 1886– DOI: 10.1002/art.41743.
File I., Trinn C., Mátyus Z. et al. Relapsing polychondritis with p-ANCA associated vasculitis: which triggers the other? World J. Clin. Cases, 2014, vol. 2, no. 12, pp. 912–917. DOI: 10.12998/wjcc. v2.i12.912.
Fukuda K., Mizobuchi T., Nakajima I. et al. Ocular involvement in relapsing polychondritis. Clin. Med, 2021, vol. 10, no. 21, pp. 4970. DOI: 10.3390/JCM10214970.
Guillaume M., Grégory P., Nathalie C.C. et al. Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study. Ann Rheum Dis, 2018, vol. 77, no. 8, pp. 1172– DOI: 10.1136/annrheumdis-2017-212705.
Hazra N., Dregan A., Charlton J. et al. Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study. Rheumatology (Oxford), 2015, vol. 54, no. 12, pp. 2181–2187. DOI: 10.1093/rheumatology/kev240.
Hebbar M., Brouillard M., Wattel E. et al. Association of myelodysplastic syndrome and relapsing polychondritis: further evidence. Leukemia, 1995, vol. 9, pp. 731–733.
Ikeda S., Arita M., Ikeo S. et al. Chronological assessment of airway lesions in relapsing polychondritis by positron emission tomography. Med, 2015, vol. 54, no. 9, pp. 1099–1102. DOI: 10.2169/internalmedicine.54.3775.
Jaksch-Wartenhorst R. Wien Arch. F Inn. Med, 1923, vol. 6, pp. 93–100.
Jun S., Yoshihisa Y., Kazuo Y. et al. Organ involvement pattern suggests subgroups within relapsing polychondritis: comment on the article by Dion et al. Arthritis Rheumatol, 2018, vol. 70, no. 1, pp. 148– DOI: 10.1002/art.40330.
Kötter I., Daikeler T., Amberger C., Tyndall A. et al. Autologous stem cell trans-plantation of treatment-resistant systemic vasculitis – a single center experience and review of the literature. Nephrol. 2005, vol. 64, no. 6, pp. 485–489. DOI: 10.5414/cnp64485.
Lei W., Zeng H., Zeng D.X. et al. (18) F-FDG PETCT: a powerful tool for the diagnosis and treatment of relapsing polychondritis. J. Radiol, 2016, vol. 89 (1057). DOI: 10.1259/bjr.20150695.
Lekpa F.K., Chevalier X. Refractory relapsing polychondritis: challenges and solutions. Open Access Rheumatol. Res. Rev. 2018, vol. 10, pp. 1–11. DOI: 10.2147/OARRR.S142892.
Longo L., Greco A., Rea A., Lo Vasco V.R. et al. Relapsing polychondritis: a clin-ical update. Autoimmun Rev, 2016, vol. 15(6), pp. 539–543. DOI: 10.1016/j.autrev.2016.02.013.
Lu P.H., Lin Y.C., Wu Y.H. Relapsing polychondritis complicated with sweet’s syndrome and normolipemic neutrophilic xanthomatosis successfully treated by dapsone. J. Dermatol, 2011, vol. 50, no. 2, pp. 212–214. DOI: 10.1111/j.1365-4632.2010. 04477.x.
Lucia L., Antonio G., Andrea R. et al. Relapsing polychondritis: A clinical update. Autoimmun Rev, 2016, vol. 15, no. 6, pp. 539–543. DOI: 10.1016/j.autrev.2016.02.013.
Martin J., Roenigk H.H., Lynch W. et al. Relapsing polychondritis treated with dapsone. Arch Dermatol, 1976, vol. 112, no. 9, pp. 1272–1274.
Mertz P., Belot A., Cervera R., Chuah T.Y. et al. The relapsing polychondritis damage index (RPDAM): Development of a disease-specific damage score for relapsing polychondritis. Joint Bone Spine. 2019, vol. 86, no. 3, pp. 363–368. DOI: 10.1016/j.jbspin.2018.11.001.
Michet C.J.Jr, McKenna C.H., Luthra H.S. et al. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med, 1986, vol. 104, no. 1, pp. 74–78. DOI: 10.7326/0003-4819-104-1-74.
Nakano K., Aritomi T., Ohkubo N. et al. Relapsing polychondritis diagnosed by fusion images of gallium-67 uptake on computed tomography and single-photon– emission computed tomography. Arthritis Rheumatol, 2017, vol. 69, no. 12, p. 2406. DOI: 10.1002/art.40234.
Pearson C.M., Kline H.M., Newcomer V.D. Relapsing polychondritis. N Engl J Med, 1960, vol. 14(263), pp. 51–58. DOI: 10.1056/NEJM196007142630201.
Pruthi P., Munganda H., Bangia A. et al. Leprosy with atypical skin lesions masquerading as relapsing polychondritis. Case Rep. Infect. Dis, 2016, vol. 2016, 7802423. DOI: 10.1155/2016/7802423.
Sabine S., Herwig P. Relapsing polychondritis: a chameleon among orphan diseases. Wien Med Wochenschr, 2017, vol. 167, no. 9–10, pp. 227–233. DOI: 10.1007/s10354-017-0559-1.
Tlish M.M., Kuznetsova T.G., Naatyzh Z.Y., Psavok F.A. et al. Relapsing polychondritis in medical practice of dermatovenerologist. Ter Arkh., 2018, vol. 90, no. 1, pp. 65–68. DOI: 10.26442/terarkh201890165-68.
Trentham D.E., Le C.H. Relapsing polychondritis. Ann Intern Med, 1998, vol. 15, no. 129(2), pp. 114–122. DOI: 10.7326/0003-4819-129-2-199807150-00011.

About authors

Zhuravleva Nadezhda V.: Candidate of Medical Sciences, Associate Professor, Department of Internal Diseases, Chuvash State University; Rheumatologist, Central City Hospital, Russia, Cheboksary (zhuravlevanv@mail.ru; ORCID: https://orcid.org/0000-0001-6470-7724)

Dibrov Danil A.: Rheumatologist, V.A. Nasonova Research Institute of Rheumatology, Russia, Moscow (dibrovd995@gmail.com; ORCID: https://orcid.org/0000-0003-3183-0464)

Ivanova Anastasia V.: Chief Rheumatologist, Ministry of Health of the Chuvash Republic; Rheumatologist, Republican Clinical Hospital, Russia, Cheboksary (nastya_arh83@mail.ru; ORCID: https://orcid.org/0000-0002-0553-3135)

Aksionova Angelina V.: Doctor of Medical Sciences, Professor, Department of Faculty Therapy named after Academician A.I. Nesterov, N.I. Pirogov Russian National Research Medical University, Russia, Moscow (aksyonova-av@mail.ru; ORCID: https://orcid.org/0000-0002-4259-3807)

Guryanova Evgeniya A.: Doctor of Medical Sciences, Professor, Rector, Postgraduate Doctors' Training Institute, Russia, Cheboksary (z-guryanova@bk.ru; ORCID: https://orcid.org/0000-0003-1519-2319)

Pushkina Natalia S.: Resident, Medical Faculty, Chuvash State University, Russia, Cheboksary (985natalia8845@mail.ru; ORCID: https://orcid.org/0009-0009-6517-4259)

Smirnova Tatyana L.: Candidate of Medical Sciences, Associate Professor, Department of Obstetrics and Gynecology, Chuvash State University, Russia, Cheboksary (tlsmr@mail.ru; ORCID: https://orcid.org/0000-0002-8224-1515)

Article link

Zhuravleva N.V., Dibrov D.A., Ivanova A.V., Aksenova A.V., Guryanova E.A., Pushkina N.S., Smirnova T.L. Relapsing Polychondritis: Problems and Solutions [Electronic resource] // Acta medica Eurasica. – 2024. – №2. P. 64-77. – URL: https://acta-medica-eurasica.ru/en/single/2024/2/8/. DOI: 10.47026/2413-4864-2024-2-64-77.

Single article

Zhuravleva N.V., Dibrov D.A., Ivanova A.V., Aksenova A.V., Guryanova E.A., Pushkina N.S., Smirnova T.L.