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[Clinical features and treatment of relapsing polychondritis with involvement of the respiratory tract-report of thirteen cases].

OBJECTIVE
To describe the clinical features and treatment of relapsing polychondritis with involvement of the respiratory tract.


METHOD
Thirteen cases (admitted from Aug 2000 to Oct 2006) of relapsing polychondritis with involvement of the respiratory tract treated in our hospital were retrospectively analyzed.


RESULTS
There were 9 males and 4 females, with ages ranging from 30 to 61 years (mean 50 years). At early stage of the disease, clinical manifestations included cough, throat pain and hoarseness. Patients in later stage usually complained of chest distress, shortness of breath and dyspnea. Severe complications were repeated lower respiratory tract infections and/or respiratory failure. Bronchoscopic examination revealed an edematous larynx, narrowing of the glottis, tracheobronchial edema, turgescence of bronchial cartilage rings and airway stenosis at early stage. At later stage of the disease, malacia of trachea and bronchi due to disappearance of bronchial cartilage rings, and partly obliteration of the both trachea and main bronchus on expiration were demonstrated. Thoracic CT scan, with three-dimensional reconstruction of the airways, demonstrated a diffusely thickened tracheobronchial wall with tracheobronchial stenosis in earlier period of the disease and showed severe narrowing of both trachea and main bronchi in later period. Lung function measurements showed a moderate obstructive ventilatory disorder in 5 patients. Medical treatment with corticosteroids and immunosuppressive drugs was given in 12 patients. Symptoms were improved in 6 patients in earlier period of the disease, however, 6 patients in later period were not relieved. Twelve self-expanding metallic stents were placed in the airways (trachea and/or main bronchi), and obstruction of the respiratory tract was relieved in 5 patients, but there was no improvement in 1 patient who later was treated with positive airway pressure support. The 13 patients were followed for 1 to 48 months, and 12 patients survived, but one patient died 2 years after diagnosis.


CONCLUSIONS
Corticosteroid therapy is effective in improving the symptoms and delaying the progression of relapsing polychondritis with involvement of the respiratory tract at early stage. At later stage of the disease, airway interventional therapy, such as metallic stent placement, tracheostomy or positive airway pressure support, can be used to treat airway obstruction and to improve the survival.