Polychondritis

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 eases, 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

Abbreviations: EBUS = endobronchial ultrasonography; RP = relapsing polychondritis

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The diagnosis of relapsing polychondritis (RP) usually depends on a constellation of clinical and histologic features caused by chondritis. (1-3) a 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 Levinee (2): one or more of the criteria of McAdams et al (1) with histologic confirmation or chondritis at least in two distinct locations with therapeutic response.

Endobronchial ultrasonography (EBUS) rereads 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 Levine (2) and facilitated the diagnosis.

CASE REPORTS

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.

[FIGURES 1&2 OMITTED]

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[degrees] 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 hyperechoic 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).

DISCUSSION

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 Mill 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.

REFERENCES

(1) McAdams LP, O' Hanlan MA, Bluestone R, et al. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine 1976; 55:193-215

(2) Damiani JM, Levine HL Relapsing polychondritis: report of ten cases. Laryngoscope 1979; 89:929-946

(3) Sarodia BP, Dasgupta MD, Mehta AC. Management of the airway manifestations of relapsing polychondritis. Chest 1999; 116:1669-1675

(4) 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

(5) Miyazu Y, Miyazawa T, Kurimoto N, et al. Endobronchial ultrasonography in the assessment of centrally ]neared early stage lung cancer before photodynamic therapy. Am J Respir Crit Care Med 2002; 165:832-837

(6) Miyazawa T, Yamakido M, Ikeda S, et al. Implantation of Ultraflex stents in malignant tracheobronchial stenoses, Chest 2000; 118:959-965

* 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.

Manuscript received February 14, 2003; revision accepted July 16, 2003.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: permissions@chestnet.org).

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@city-hosp.naka.hiroshima.jp

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