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Relapsing polychondritis – analysis of symptoms and criteria

OBJECTIVES: Relapsing polychondritis (RP) is a rare disease characterised by recurrent inflammation of the cartilaginous structures and proteoglycan-rich organs. The aim of this case series study is to share the 10-year clinical experience of our department in diagnosing RP patients in the context o...

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Autores principales: Maciążek-Chyra, Beata, Szmyrka, Magdalena, Skoczyńska, Marta, Sokolik, Renata, Lasocka, Joanna, Wiland, Piotr
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6409832/
https://www.ncbi.nlm.nih.gov/pubmed/30858626
http://dx.doi.org/10.5114/reum.2019.83234
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author Maciążek-Chyra, Beata
Szmyrka, Magdalena
Skoczyńska, Marta
Sokolik, Renata
Lasocka, Joanna
Wiland, Piotr
author_facet Maciążek-Chyra, Beata
Szmyrka, Magdalena
Skoczyńska, Marta
Sokolik, Renata
Lasocka, Joanna
Wiland, Piotr
author_sort Maciążek-Chyra, Beata
collection PubMed
description OBJECTIVES: Relapsing polychondritis (RP) is a rare disease characterised by recurrent inflammation of the cartilaginous structures and proteoglycan-rich organs. The aim of this case series study is to share the 10-year clinical experience of our department in diagnosing RP patients in the context of data from available published studies. MATERIAL AND METHODS: A retrospective case analysis of 10 patients with symptoms of RP, hospitalised at the Department of Rheumatology and Internal Diseases of Wrocław University Hospital between January 2008 and December 2018. RESULTS: Nine out of 10 patients fulfilled at least one of the three sets of the diagnostic criteria. The mean age (±standard deviation) at diagnosis was 54.4 ±13.3 years and ranged from 32 to 73 years. The symptoms suggestive of the RP diagnosis were mainly inflammation of the pinna (in 80% of patients) and laryngeal stenosis (in 20% of patients). The mean age at which initial symptoms were observed was 52.3 ±12.0 years and ranged from 31 to 69 years. Auricular chondritis was the first manifestation of the disease in 40% of cases (two women and two men) laryngeal chondritis in 20%, nasal chondritis in 10%, and bronchial stenosis in 10%. Other initial symptoms were polyarthritis, which was present in 10% of cases (male) and general symptoms observed in 10%. CONCLUSIONS: A thorough analysis of the entire medical history with specific questions about the occurrence of the manifestations of the disease in the past leads to the diagnosis of RP. The RP also should be considered in differential diagnosis of respiratory track narrowings. It is very useful to apply the three sets of criteria simultaneously in the diagnostic process.
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spelling pubmed-64098322019-03-11 Relapsing polychondritis – analysis of symptoms and criteria Maciążek-Chyra, Beata Szmyrka, Magdalena Skoczyńska, Marta Sokolik, Renata Lasocka, Joanna Wiland, Piotr Reumatologia Original Paper OBJECTIVES: Relapsing polychondritis (RP) is a rare disease characterised by recurrent inflammation of the cartilaginous structures and proteoglycan-rich organs. The aim of this case series study is to share the 10-year clinical experience of our department in diagnosing RP patients in the context of data from available published studies. MATERIAL AND METHODS: A retrospective case analysis of 10 patients with symptoms of RP, hospitalised at the Department of Rheumatology and Internal Diseases of Wrocław University Hospital between January 2008 and December 2018. RESULTS: Nine out of 10 patients fulfilled at least one of the three sets of the diagnostic criteria. The mean age (±standard deviation) at diagnosis was 54.4 ±13.3 years and ranged from 32 to 73 years. The symptoms suggestive of the RP diagnosis were mainly inflammation of the pinna (in 80% of patients) and laryngeal stenosis (in 20% of patients). The mean age at which initial symptoms were observed was 52.3 ±12.0 years and ranged from 31 to 69 years. Auricular chondritis was the first manifestation of the disease in 40% of cases (two women and two men) laryngeal chondritis in 20%, nasal chondritis in 10%, and bronchial stenosis in 10%. Other initial symptoms were polyarthritis, which was present in 10% of cases (male) and general symptoms observed in 10%. CONCLUSIONS: A thorough analysis of the entire medical history with specific questions about the occurrence of the manifestations of the disease in the past leads to the diagnosis of RP. The RP also should be considered in differential diagnosis of respiratory track narrowings. It is very useful to apply the three sets of criteria simultaneously in the diagnostic process. Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie 2019-02-28 2019 /pmc/articles/PMC6409832/ /pubmed/30858626 http://dx.doi.org/10.5114/reum.2019.83234 Text en Copyright: © 2019 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie http://creativecommons.org/licenses/by-nc-sa/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
spellingShingle Original Paper
Maciążek-Chyra, Beata
Szmyrka, Magdalena
Skoczyńska, Marta
Sokolik, Renata
Lasocka, Joanna
Wiland, Piotr
Relapsing polychondritis – analysis of symptoms and criteria
title Relapsing polychondritis – analysis of symptoms and criteria
title_full Relapsing polychondritis – analysis of symptoms and criteria
title_fullStr Relapsing polychondritis – analysis of symptoms and criteria
title_full_unstemmed Relapsing polychondritis – analysis of symptoms and criteria
title_short Relapsing polychondritis – analysis of symptoms and criteria
title_sort relapsing polychondritis – analysis of symptoms and criteria
topic Original Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6409832/
https://www.ncbi.nlm.nih.gov/pubmed/30858626
http://dx.doi.org/10.5114/reum.2019.83234
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