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Original Research: RELAPSING POLYCHONDRITIS |

Relapsing Polychondritis and Airway Involvement

Armin Ernst, MD, FCCP; Samaan Rafeq, MD; Phillip Boiselle, MD; Arthur Sung, MD, FCCP; Chakravarthy Reddy, MD; Gaetane Michaud, MD, FCCP; Adnan Majid, MD; Felix J. F. Herth, MD, FCCP; David Trentham, MD
Author and Funding Information

*From the Departments of Interventional Pulmonology (Drs. Ernst, Rafeq, Sung, Reddy, Michaud, and Majid), and Radiology (Dr. Boiselle), Division of Rheumatology (Dr. Trentham), Beth Israel Deaconess Medical Center, Boston, MA; and Thoraxklinik (Dr. Herth), Heidelberg, Germany.

Correspondence to: Armin Ernst, MD, FCCP, Chief, Interventional Pulmonology, Beth Israel Deaconess Medical Center, Harvard Medical School, Deaconess Building 201, Boston, MA 02215; e-mail: aernst@bidmc.harvard.edu


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).


Chest. 2009;135(4):1024-1030. doi:10.1378/chest.08-1180
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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.

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