INTRODUCTION: Relapsing polychondritis is a chronic multisystem disease associated with recurrent inflammation and destruction of cartilaginous structures. Typical areas of involvement are the ears, nose, and airways with the lower respiratory tract becoming involved in 20-50% of cases. In those who develop respiratory tract manifestations, respiratory symptoms may be the initial presentation in up to one half of these patients. Historically, airway involvement has been associated with poor outcomes.
CASE PRESENTATION: A 44-year old female presented to the pulmonary clinic for progressive dyspnea on exertion and wheezing. She had been diagnosed with asthma on the basis of these symptoms along with severe obstruction noted on spirometry in the past. She had been treated with bronchodilators and inhaled corticosteroids with minimal response. Her initial examination was remarkable for a mild saddle nose deformity, attributed to damage following a transphenoidal surgery for a pituitary adenoma several years ago. At the time of referral, she had no wheezing on exam; however, her spirometry was significant for severe obstruction without bronchodilator reversibility. Impulse oscillometry, an effort-independent technique to determine airway resistance, was found to be normal in this patient, suggesting the possibility of dynamic airway obstruction. Bronchoscopy was performed, which demonstrated dynamic collapse of the bilateral mainstem bronchi during forced exhalation. Subsequent laboratory evaluation revealed elevated IgG to Type II Collagen which can be found with relapsing polychondirits.
DISCUSSION: Patients with relapsing polychondritis frequently develop airway complications that can be devastating, causing significant morbidity and mortality. The airway complications may consist of tracheobronchomalacia (TBM) and/or excessive dynamic airway collapse (EDAC). In normal individuals, forceful expiration or coughing can lead to 40% narrowing of the tracheobronchial lumen. Such reductions become pathologic when they exceed 50% of the lumen. With TBM, there is weakness of the walls of the lower airway secondary to cartilaginous destruction and hypotonia of the myoelastic components. However, if the narrowing is predominantly due to collapse of the posterior lumen, EDAC is the likely underlying pathophysiology. By bronchoscopic and dynamic computed tomography evaluation, this patient had predominantly EDAC related airway changes. Regardless of the predominant pathophysiology, patients with this condition are treated through a multidisciplinary approach aimed at relieving this dynamic obstruction.
CONCLUSIONS: Relapsing polychondritis with tracheobronchomalacia often presents with non-specific symptoms similar to patients with lung diseases such as COPD or asthma. The typical symptoms of dyspnea and wheezing, often made worse by exertion, may lead clinicians to treat these more commonly encountered lung pathologies without ever considering this condition. When pulmonary manifestations are the first clinically relevant signs of relapsing polychondritis, it can be appreciated how the correct diagnosis is often delayed. The diagnosis is often suspected once patients have failed to respond to escalating therapy for the more common obstructive lung diseases and when CT imaging demonstrates tracheal narrowing. In this patient’s case, an alternative diagnosis to asthma was suspected after impulse oscillometry demonstrated no increase in airway resistance during tidal volume breathing, in sharp contrast to the severe obstruction seen with the force maneuver on her spirometry.
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Reference #2 Murgu S, Colt H. Tracheobronchomalacia and excessive dynamic airway collapse. Respirology 2006; 11:388-406.
Reference #3 Rafeq S, Trentham D, Ernst A. Pulmonary Manifestations of Relapsing Polychondritis. Clin Chest Med 2010;31:513-18.
DISCLOSURE: The following authors have nothing to disclose: Nicholas Ondrasik, David Bell
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