Relapsing polychondritis and airway involvement.
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.


CONCLUSIONS
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.
Ernst
Armin
A
Department of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA. Electronic address: aernst@bidmc.harvard.edu.
Rafeq
Samaan
S
Department of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA.
Boiselle
Phillip
P
Department of Radiology, Beth Israel Deaconess Medical Center, Boston, MA.
Sung
Arthur
A
Department of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA.
Reddy
Chakravarthy
C
Department of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA.
Michaud
Gaetane
G
Department of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA.
Majid
Adnan
A
Department of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Boston, MA.
Herth
Felix J F
FJF
Thoraxklinik, Heidelberg, Germany.
Trentham
David
D
Division of Rheumatology, Beth Israel Deaconess Medical Center, Boston, MA.
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D007829:Laryngostenosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000150:complications / Q000453:epidemiology* / Q000628:therapy; D015995:Prevalence; D012141:Respiratory Tract Infections / Q000150:complications / Q000453:epidemiology* / Q000628:therapy; D055090:Tracheomalacia / Q000209:etiology