Relapsing polychondritis - A single Centre study in the United Kingdom.

Autor: Sangle SR; The Louise Coote Lupus Unit, 4th Floor, Tower Wing, Guy's Hospital, Guy's and St Thomas' and King's College Medical School, London SE1 9RT, UK., Hughes CD; The Louise Coote Lupus Unit, 4th Floor, Tower Wing, Guy's Hospital, Guy's and St Thomas' and King's College Medical School, London SE1 9RT, UK., Barry L; The Louise Coote Lupus Unit, 4th Floor, Tower Wing, Guy's Hospital, Guy's and St Thomas' and King's College Medical School, London SE1 9RT, UK., Qureshi S; The Louise Coote Lupus Unit, 4th Floor, Tower Wing, Guy's Hospital, Guy's and St Thomas' and King's College Medical School, London SE1 9RT, UK., Cheah CK; The Louise Coote Lupus Unit, 4th Floor, Tower Wing, Guy's Hospital, Guy's and St Thomas' and King's College Medical School, London SE1 9RT, UK., Poh YJ; The Louise Coote Lupus Unit, 4th Floor, Tower Wing, Guy's Hospital, Guy's and St Thomas' and King's College Medical School, London SE1 9RT, UK., D'Cruz DP; The Louise Coote Lupus Unit, 4th Floor, Tower Wing, Guy's Hospital, Guy's and St Thomas' and King's College Medical School, London SE1 9RT, UK. Electronic address: david.d'cruz@kcl.ac.uk.
Jazyk: angličtina
Zdroj: Autoimmunity reviews [Autoimmun Rev] 2023 Aug; Vol. 22 (8), pp. 103352. Date of Electronic Publication: 2023 May 04.
DOI: 10.1016/j.autrev.2023.103352
Abstrakt: Introduction and Objectives: Relapsing Polychondritis (RP) is a rare immune mediated inflammatory disorder that may result in damage and destruction of cartilaginous tissues.
Patients and Methods: We retrospectively analysed patients with a clinical diagnosis of RP. Patients were investigated using pulmonary function tests, dynamic high-resolution CT scans, bronchoscopy, laryngoscopy and/or PET-CT scans along with autoimmune serology. Patients had other specialist reviews when indicated.
Results: We identified 68 patients with a diagnosis of RP, 55 (81%) were Caucasian, 8 (12%) Afro Caribbean, 4 (6%) Asian and 1 patient had Mixed Ethnicity. Twenty-nine (43%) had pulmonary involvement and in 16, pulmonary involvement was the initial presentation. The mean age at onset was 44 years (range 17-74). There was a mean diagnostic delay of 55 weeks. Sixty-six (97%) patients received a combination of oral Prednisolone and disease modifying anti-rheumatic drugs. Twelve of 19 (63%) received biologics, with an initial good response, and 10 remain on treatment. Eleven patients with respiratory collapse required CPAP to maintain airway patency. Twelve (18%) patients died due to RP and 9 had respiratory complications. Two patients developed myelodysplasia and one had lung carcinoma. In a multivariate regression analysis, the prognostic variables were ethnicity, nasal chondritis, laryngotracheal stricture and elevated serum creatinine.
Conclusion: RP is a rare autoimmune condition often associated with significant delays in diagnosis and initiation of treatment. Pulmonary involvement in RP may cause significant morbidity and mortality due to organ damage. Disease modifying anti rheumatic drugs and biologics should be considered early in the disease course to minimise adverse effects of long-term corticosteroid therapy and organ damage.
Competing Interests: Declaration of Competing Interest There are no conflicts of interest to declare; Sangle, Barry, Qureshi, Cheah, and Poh contributed in collecting data. Sangle and Hughes helped in writing the manuscript. D'Cruz helped in collecting data, supervising the project and finalising the manuscript. The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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