(Chest. 2003;124:2393-2395.)
© 2003
American College of Chest Physicians
Endobronchial Ultrasonography in the Diagnosis and Treatment of Relapsing Polychondritis With Tracheobronchial Malacia*
Yuka Miyazu, MD;
Teruomi Miyazawa, MD, PhD, FCCP;
Noriaki Kurimoto, MD;
Yasuo Iwamoto, MD;
Atsuko Ishida, MD;
Koji Kanoh, MD and
Nobuoki Kohno, MD, FCCP
* From the Department of Pulmonary Medicine, Hiroshima City Hospital, Hiroshima; Department of Surgery, Hiroshima National Hospital, Higashi-Hiroshima; and Department of Molecular and Internal Medicine, Graduate School of Biomedical Science, Hiroshima University, Hiroshima, Japan.
Correspondence to: Teruomi Miyazawa, MD, PhD, FCCP, Director, Department of Pulmonary Medicine, Hiroshima City Hospital, 7-33 Motomachi, Naka-ku, Hiroshima 730-0011, Japan; e-mail: ikyoku{at}city-hosp.naka.hiroshima.jp
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Abstract
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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.
Key Words: endobronchial ultrasonography • nitinol stent • polychondritis
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Introduction
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The diagnosis of relapsing polychondritis (RP) usually depends on a constellation of clinical and histologic features caused by chondritis.1
2
3
According to McAdams et al,1
if patients have at least three of the following six signs, the diagnosis is conclusive: bilateral auricular chondritis, nonerosive inflammatory polyarthritis, nasal chondritis, ocular inflammation, laryngotracheobronchial chondritis, and audiovestibular damage. The modified criteria was proposed by Damiani and Levine2
: one or more of the criteria of McAdams et al1
with histologic confirmation or chondritis at least in two distinct locations with therapeutic response.
Endobronchial ultrasonography (EBUS) reveals the tracheobronchial wall structure and cartilaginous layer clearly.4
5
In this report, EBUS demonstrated changes in the tracheobronchial cartilage in two patients with RP who met the criteria of Darmiani and Levine2
and facilitated the diagnosis.
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Case Reports
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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 severely narrowed lumen. EBUS showed thickening of the bronchial wall due to submucosal edema, and the cartilage layer appeared ill-defined and absent in places; this continued along the trachea into both main bronchi (Fig 2
, top left, A, and top right, B). Biopsy of the tracheal cartilage showed degeneration with fibrous changes and inflammatory cell infiltration. RP was diagnosed. The administrations of high-dose corticosteroids resulted in no improvement; therefore, it was decided to proceed with airway stenting.
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Figure 1.. Bronchoscopic views of stent placement before (top left, A) and after (top right, B) in a patient with RP (case 1). The openings for the left and right mainstem bronchi appeared dynamically collapsed before stenting. Two months after stenting, the airway is patent, and tracheal stent has been integrated into the tracheal wall. Bronchoscopic views before (bottom left, C) and after (bottom right, D) stent placement in case 2 show that the collapse of both mainstem bronchi is prevented and patency restored completely.
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Figure 2.. Top left, A: EBUS of case 1; top right, B: detail of left, A. The right mainstem bronchus shows a thickening of the bronchial wall with destruction of the cartilage. The weak signals in the hyperechoic third and fifth layers of the bronchial wall would be clearer if the cartilage were intact. Bottom left, C: EBUS of case 2; bottom right, D: detail of bottom left, C. EBUS of left main bronchus shows marked swelling and degeneration of the cartilage layer and submucosal edema of the bronchial wall.
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Before stenting, we measured the diameter of the affected tracheobronchial tree using EBUS ensheathed with a balloon. Once water fills the balloon until it blocks the airway completely, EBUS provided a view of 360° so that we could evaluate the diameter. Through a flexible bronchoscope, we implanted four uncovered Ultraflex stents (Boston Scientific; Natick, MA) in the right mainstem bronchus (width/length: 10/20 mm), the left mainstem bronchus (10/40 mm), and the trachea (14/60 mm and 14/20 mm). After stenting, bronchoscopy showed that the stents were keeping the airway patent and were epithelized and integrated into the bronchial wall (Fig 1
, top right, B). As a result, the patient reported improved dyspnea and returned to normal activities.
Case 2
A 61-year-old woman presented with sudden progressive dyspnea, necessitating emergency mechanical ventilation. After methylprednisolone pulse therapy, she was successfully extubated, but she remained dyspneic and required a tracheotomy. The severity of the dyspnea resulted in her being bedridden.
Bronchoscopy showed severe narrowing of the trachea, extending into the mainstem bronchi and beyond (Fig 1
, bottom left, C). EBUS revealed destruction of the normal cartilages, this continued along the central airway (Fig 2
, bottom left, C, and bottom right, D). The hyperechoic third and fifth layers of the bronchial wall were indistinct, and the hypoechoic fourth layer was markedly swollen, indicating cartilage degeneration. Biopsy of tracheal cartilage confirmed chronic chondritis with plasma cell and lymphocytic infiltration. To maintain the airway, we implanted four uncovered Ultraflex stents in the right mainstem bronchus (width/length: 10/20 mm), the truncus intermedius (8/20 mm), the left mainstem bronchus (10/40 mm), and the trachea (14/40 mm). Two months after stent implantation bronchoscopy revealed a widely patent airway and her daily activities were unrestricted (Fig 1
, bottom right, D).
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Discussion
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In the EBUS image, the tracheobronchial wall appears as a layered structure with distinct cartilaginous layers.4
5
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 considered it an important adjunct to the diagnosis of RP, and one intrinsic to RP. Furthermore, the dynamic changes in airway diameter make it difficult to determine accurately what size stent is needed. The utility of CT and MRI is limited because they generate static images, but EBUS generates a real time image and we can evaluate the size of the airway using a water-filled balloon.
Of all materials currently in use for airway stenting, we consider the Ultraflex nitinol stent to be the best, because of properties similar to those of natural cartilage.6
In the present cases, properly deployed stents become epithelialized within a few months, which prevents migration and provides almost normal clearance of mucus.
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Footnotes
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Abbreviations: EBUS = endobronchial ultrasonography; RP = relapsing polychondritis
Received for publication February 14, 2003.
Accepted for publication July 16, 2003.
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References
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- McAdams, LP, O’ Hanlan, MA, Bluestone, R, et al (1976) Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine 55,193-215[Medline]
- Damiani, JM, Levine, HL Relapsing polychondritis: report of ten cases. Laryngoscope 1979;89,929-946[ISI][Medline]
- Sarodia, BP, Dasgupta, MD, Mehta, AC Management of the airway manifestations of relapsing polychondritis. Chest 1999;116,1669-1675[Abstract/Free Full Text]
- Kurimoto, N, Murayama, M, Yoshioka, S, et al Assessment of usefulness of endobronchial ultrasonography in determination of depth of tracheobronchial tumor invasion. Chest 1999;115,1500-1506[Abstract/Free Full Text]
- Miyazu, Y, Miyazawa, T, Kurimoto, N, et al Endobronchial ultrasonography in the assessment of centrally located early stage lung cancer before photodynamic therapy. Am J Respir Crit Care Med 2002;165,832-837[Abstract/Free Full Text]
- Miyazawa, T, Yamakido, M, Ikeda, S, et al Implantation of Ultraflex stents in malignant tracheobronchial stenoses, Chest 2000;118,959-965[Abstract/Free Full Text]
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Abstract
Introduction
