Chest
Volume 135, Issue 4, April 2009, Pages 1024-1030
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Original Research
Relapsing Polychondritis
Relapsing Polychondritis and Airway Involvement

https://doi.org/10.1378/chest.08-1180Get rights and content

Objective

To assess the prevalence and characteristics of airway involvement in relapsing polychondritis (RP).

Methods

Retrospective chart review and data analysis of RP patients seen in the Rheumatology Clinic and the Complex Airway Center at Beth Israel Deaconess Medical Center from January 2004 through February 2008.

Results

RP was diagnosed in 145 patients. Thirty-one patients had airway involvement, a prevalence of 21%. Twenty-two patients were women (70%), and they were between 11 and 61 years of age (median age, 42 years) at the time of first symptoms. Airway symptoms were the first manifestation of disease in 17 patients (54%). Dyspnea was the most common symptom in 20 patients (64%), followed by cough, stridor, and hoarseness. Airway problems included the following: subglottic stenosis (n = 8; 26%); focal and diffuse malacia (n = 15; 48%); and focal stenosis in different areas of the bronchial tree in the rest of the patients. Twelve patients (40%) required and underwent intervention including balloon dilatation, stent placement, tracheotomy, or a combination of the above with good success. The majority of patients experienced improvement in airway symptoms after intervention. One patient died during the follow-up period from the progression of airway disease. The rest of the patients continue to undergo periodic evaluation and intervention.

Conclusion

In this largest cohort described in the English language literature, we found symptomatic airway involvement in RP to be common and at times severe. The nature of airway problems is diverse, with tracheomalacia being the most common. Airway intervention is frequently required and in experienced hands results in symptom improvement.

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

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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).

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