Chest
Original ResearchRelapsing PolychondritisRelapsing Polychondritis and Airway Involvement
Section snippets
Materials and Methods
This study is a retrospective chart review and data analysis of RP patients who were referred to the Rheumatology Clinic and the Complex Airway Center at Beth Israel Deaconess Medical Center over a 4-year period (January 2004 to February 2008). Our institutional review board approved the study. Informed consent was waived for conducting a retrospective analysis.
RP was diagnosed based on the following criteria set by MacAdam et al6 and Damiani and Levine9: (1) bilateral auricular chondritis; (2)
Results
In the chosen 4-year period, 145 patients were seen with a confirmed diagnosis of RP; 31 of those patients had symptomatic airway involvement, for a prevalence of 21%. They constitute the reviewed cohort for this study. The ages ranged from 11 to 61 years (median age, 42 years), and 22 patients were women (70%).
Discussion
Respiratory complications are felt to be responsible for the significant morbidity and mortality seen in patients with RP, but most reports have been anecdotal or related to small case series. Purcelli et al19 described the first case report of RP with tracheal collapse in 1962, and McAdam et al6 reported in a case series in 1976 that the majority of patients with RP and a known cause of death had respiratory tract involvement.
In our series, which to our knowledge is the largest cohort of
References (21)
- et al.
Treatment of diffuse tracheomalacia secondary to relapsing polychondritis with continuous positive airway pressure
Chest
(1997) - et al.
Airway complications in relapsing polychondritis
Ann Thorac Surg
(1991) - et al.
Management of airway manifestations of relapsing polychondritis: case reports and review of literature
Chest
(1999) - et al.
Airway complications in relapsing polychondritis
Ann Thorac Surg
(1991) - et al.
Relapsing polychondritis
Ann Intern Med
(1998) - et al.
Ocular and systemic findings in relapsing polychondritis
Ophthalmology
(1986) - et al.
Relapsing polychondritis: survival and predictive role of early disease manifestations
Ann Intern Med
(1986) - et al.
Prevalence of functional airway abnormalities in relapsing polychondritis
Radiology
(2006) - et al.
Relapsing polychondritis: prospective study of 23 patients and a review of the literature
Medicine (Baltimore)
(1976) - et al.
Relapsing polychondritis affecting the lower respiratory tract
AJR Am J Roentgenol
(2002)
Cited by (145)
Interventionnal bronchoscopy for the treatment of tracheobronchomalacia
2023, Revue des Maladies RespiratoiresRelapsing polychondritis - A single Centre study in the United Kingdom
2023, Autoimmunity ReviewsFrench practical guidelines for the diagnosis and management of relapsing polychondritis
2023, Revue de Medecine InterneClinical analysis of relapsing polychondritis with airway involvement
2023, Journal of Laryngology and OtologyRisk factors associated with severe adverse events in patients with relapsing polychondritis undergoing flexible bronchoscopy
2024, Orphanet Journal of Rare Diseases
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).