Chest
Volume 116, Issue 6, December 1999, Pages 1669-1675
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Clinical Investigations
Airways Disease
Management of Airway Manifestations of Relapsing Polychondritis: Case Reports and Review of Literature

https://doi.org/10.1378/chest.116.6.1669Get rights and content

Study objective

To report the first series of patients with severe airway manifestations of relapsing polychondritis (RP) that were managed successfully with self-expandable metallic stents, and to review the literature.

Design

Retrospective review of medical records, and current clinical follow-up.

Setting

Tertiary care referral hospital.

Patients

All patients with airway manifestations of RP that were managed with self-expandable metallic stents at our institution.

Results

All five patients (four women and one man; age, 40 to 69 years old) had severe airway manifestations, and three of them required mechanical ventilation. Spirometry with flow-volume curves showed severe combined obstructive and restrictive ventilatory defects. Bronchoscopy revealed dynamic collapse of the proximal airways. Diagnosis was made 8 months to 13 years after the first symptom of the disease. Pharmacotherapy included prednisone, methotrexate, cyclosporine, and dapsone. A total of 17 self-expandable metallic stents of varying sizes were placed using flexible bronchoscope from 4 to 19 years after the first symptom. The overall outcome was favorable in four patients. Three patients have survived without ventilatory support 16 to 18 months following the first stent placement, and the fourth patient survived for 20 months without ventilatory support before she died. The fifth patient, who was receiving mechanical ventilation, died in 1 week probably due to persistent dynamic collapse of the airways distal to the stents.

Conclusion

Self-expandable metallic tracheobronchial stents should be considered in the management of airway manifestations of RP, especially in patients who require mechanical ventilation.

Section snippets

Materials and Methods

We reviewed medical records of all patients with airway manifestations of RP who were managed at our institution using a SEMS (Wallstent; Schneider; Minneapolis, MN). The demographic characteristics, clinical features, diagnostic test results, therapeutic interventions, and the latest clinical follow-up were noted. The English-language literature on the management of airway manifestations of RP was also reviewed. The stents were placed using FB by a technique described previously.7 The patients

Results

Between March 1996 and February 1999, five patients with airway manifestations of RP were treated with SEMSs (Table 2). There were four women and one man, with ages ranging from 40 to 69 years (Table 2).

Discussion

The literature on RP is scanty. In a series of 112 patients with RP, Michet et al1 described the natural history, clinical features, and predictors of death. Others have described smaller numbers of patients with airway manifestations and their management.56 Another report reviewed serious airway complications in 62 patients.4

References (18)

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