Endobronchial Ultrasonography in the Diagnosis and Treatment of Relapsing Polychondritis With Tracheobronchial Malacia

doi:10.1378/chest.124.6.2393

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Volume 124, Issue 6, December 2003, Pages 2393-2395

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Relapsing polychondritis (RP) with tracheobronchial involvement has a poor prognosis, and a delay in diagnosis increases morbidity and mortality; however, the diagnosis is difficult to make. Endobronchial ultrasonography (EBUS) revealed changes in the tracheobronchial cartilage in two patients who met the criteria for RP, and facilitated the diagnosis. In these cases, EBUS revealed a poorly defined bronchial wall structure with two patterns of cartilaginous damage: fragmentation and edema. These cases were successfully treated by the implantation of nitinol stents, the sizes of which were determined by EBUS. EBUS was found to be useful in the diagnosis and treatment of RP.

Section snippets

Case 1

A 67-year-old woman had undergone an emergency tracheotomy 5 years previously. Dyspnea initially improved following the tracheotomy, but then worsened over time and she was referred to our institution.

There was no evidence of auricular or nasal abnormalities. Flexible bronchoscopy demonstrated malacia of the tracheobronchial tree, with collapse of the airway on expiration (Fig 1, top left, A). Three-dimensional CT images demonstrated diffuse thickening of the tracheobronchial wall with a

Discussion

In the EBUS image, the tracheobronchial wall appears as a layered structure with distinct cartilaginous layers.⁴⁵ In this report, we identified two patterns of cartilaginous damage using EBUS, fragmentation and edema, which continued along the trachea into both main bronchi. While we have used EBUS in some patients with tracheobronchial malacia and tracheomegaly, their tracheobronchial cartilages are usually intact. This EBUS image of cartilage in RP may not be always unique to RP; we

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Citation Excerpt :
High-frequency endobronchial ultrasonography (EBUS) using a 20-MHz radial scanning probe was shown to identify the hypoechoic and hyperechoic layers that correlate with the laminar histologic structures of the central airways.97 Cartilage abnormalities (weakness, fracture, edema) was described in patients with malacia caused by tuberculosis, relapsing polychondritis, lung cancer, and compression by vascular rings.97–99 EBUS could potentially distinguish between TBM and EDAC because in the latter it seems that the cartilage is intact, and the posterior membrane is thinner than normal, likely because of atrophy of elastic fibers.99
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Les atteintes oto-rhino-laryngologiques (ORL) sont parmi les plus fréquentes des manifestations observées dans les maladies systémiques ou auto-immunes. Certaines d’entre elles ont une expression ORL prédominante, c’est le cas de la polychondrite atrophiante ou de certains vascularites à ANCA : granulomatose avec polyangéite (Wegener) et syndrome de Churg-Strauss. Pour de nombreuses autres maladies, l’atteinte ORL est plus rare mais vient parfois révéler la maladie et poser des problèmes diagnostiques parfois difficiles. Une coopération entre spécialiste ORL, rhumatologues et internistes est indispensable.
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Selected ReportsEndobronchial Ultrasonography in the Diagnosis and Treatment of Relapsing Polychondritis With Tracheobronchial Malacia

Section snippets

Case 1

Discussion

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Selected Reports
Endobronchial Ultrasonography in the Diagnosis and Treatment of Relapsing Polychondritis With Tracheobronchial Malacia