PURPOSE: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 December 2007.
RESULTS:One hundred forty-four (144) patients were diagnosed with RP. Thirty had airway involvement, a prevalence of 21%. Twenty- two were females (73%) and ages were 11–61 years (median 42.5) at the time of first symptoms.Airway symptoms were the first manifestation of disease in 16 patients (53%). Dyspnea was the most common symptom in 19 patients (63%) followed by cough, stridor, and hoarseness. Airway problems included: subglottic stenosis (n=8, 26%), focal and diffuse malacia (n=14, 46%) 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, tracheostomy, or a combination of the above with good success.The majority of patients experienced improvement in airway symptoms after intervention. One patient expired during the follow-up period from progression of airway disease. The rest of the patients continue to undergo periodic evaluation and intervention.
CONCLUSION:In this largest cohort in the English 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.
CLINICAL IMPLICATIONS:Airway involvement in RP patients is common and frequently requires intervention which results in symptom improvement when performed in an experienced center.
DISCLOSURE:Samaan Rafeq, None.