Relapsing polychondritis (RP) is an uncommon inflammatory disease, even more rare in pédiatrie age, affecting cartilages of the nose, ears, joints, aorta and major airways. An immune reaction against cartilage antigens is the current etiologic hypothesis, resulting in cell damage and consequent cartilage destruction. A 8-year-old Caucasian girl was referred to our Department for evaluation of worsening dyspnea. Eighteen 18 months earlier, a diagnosis of RP with involvement of the trachéal cartilages was made elsewhere, and immunosuppressive therapy (prednisone, azathioprine and cyclosporin) was begun. At admission, mild dyspnea was evident with decreased breath sound and rhonchi over the left hemithorax. Pulmonary function tests showed decreased lung volumes (V) and flows (F), and the shape of the forced expiratory and inspiratory F-V loop was characteristic of a fixed obstruction of the central airways. Thorax CT scan and NMR demonstrated narrowing of the main left bronchus, leading to air trapping in the left lung. Fiberoptic bronchoscopy demonstrated deep mucosal erosion of the carina between the upper right bronchus and the "truncus intermedius", and severe concentric fibrotic stenosis of the main left bronchus. Complete recanalization of the fibrotic stenosis was obtained with Nd YAG laser therapy, performed through a rigid bronchoscope. In the 10 days following the procedure, four more Fbs were performed to remove fibrinoid material occluding the treated bronchial segment. One month later, the F-V loop was normal and the endoscopie view of the left bronchus showed a good patency. Relapse of the main left bronchus was documented by a pulmonary function test seven months later, despite the ongoing immuno-suppressive therapy. A second rigid bronchoscopy was then performed, and an endobronchial stent was positioned with good bronchial patency. A six-month follow-up so far reveals the success of this treatment, with the patient free of any respiratory symptoms.
|Number of pages||1|
|Issue number||SUPPL. 16|
|Publication status||Published - 1997|
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Pulmonary and Respiratory Medicine