Polychondritis

Airway complications of relapsing polychondritis (RP), including tracheobronchial stenosis, can be fatal. This paper describes a life-saving technique (placement of multiple metallic endobronchial stents under conscious sedation) to prevent life-threatening airway closure in a 50-year-old woman with BP. Using fluoroscopic and bronchoscopic guidance, a tracheal stent and three endobronchial metallic stents were deployed in the central airways, with good functional outcome. There were no complications. In critical airway compromise caused by BP, the insertion of endobronchial stents can result in improved symptoms, pulmonary function, and a return to daily activities, without the use of tracheotomy and mechanical ventilation.

(CHEST 1999; 116:825-827)

Key words: bronchial; relapsing polychondritis; stenosis; stent; trachea

Abbreviation: RP = relapsing polychondritis

Relapsing polychondritis (RP) is a rare multisystem disorder of unknown cause, characterized by recurrent inflammation and destruction of cartilage. Airway complications, including laryngeal collapse and tracheal stenosis, occur late in the disease course and can be fatal.[1,2] Therapies for RP include high-dose corticosteroids,[2,3] cyclophosphamide, dapsone, Nd YAG laser,[4] tracheotomy, bronchial surgery, and endobronchial stenting.[4,5] Endobronchial stenting, however, may lead to short-term loss of mucociliary clearance and acute, life-threatening airway closure. Aspiration pneumonia, ulceration, airway erosion, and infection are other well-recognized complications of endobronchial stent insertion. The use of endobronchial stents, in patients with RP, has been associated with untoward morbidity and mortality.[3,4,6] Moreover, the critical airflow limitation of RP is not easily reversed by tracheotomy or mechanical ventilation, inasmuch as the diffuse narrowing of the airway commonly extends along the trachea into both mainstem bronchi and beyond.[2] This paper describes the use of multiple metallic endobronchial stents to reverse the life-threatening airflow limitation of RP.

CASE REPORT

A 50-year-old woman was referred for management of a critically narrowed airway. She was unable to speak a full sentence and could walk [is less than] 9 m because of dyspnea. There was a 7-year history of progressive shortness of breath and a 6-month history of severe exercise intolerance. She reported a 5-year history of recurrent uveitis and an 8-month history of nose deformity and collapse. For 1 year, she complained of multiple pains and exquisite tenderness, particularly in the sternum, left ear, and ribcage. Her rheumatologic pains, but not her dyspnea, responded to a daily dose of 10 mg of prednisone. She was a nonsmoker, with no family history of arthritis or asthma.

Physical examination revealed hoarseness, stridor, a "saddle" deformity of the midnose, scleromalacia of the left eye, and a deformed left ear. There wits tenderness over all the sternocostal joints and costal cartilages. Auscultation revealed inspiratory stridor, prolonged expiration, and wheeze. Thoracic CT scan, with three-dimensional reconstruction of the neck and thorax, demonstrated a diffusely thickened tracheal wall that partly obliterated the tracheobronchial lumen from the level of the larynx to the lobar bronchi. The maximum diameter of the tracheal lumen, its measured by reconstruction, was 8 mm. Intraparenchymal airway size appeared to be relatively well preserved compared with central airway narrowing (Fig 1). Results of baseline spirometry are contained in Table 1 and Figure 2. A diagnosis of critical airway obstruction caused by RP was made. High-dose corticosteroids and cyclophosphamide were administered, but without improvement in spirometry or respiratory symptoms.

[Figures 1-2 ILLUSTRATION OMITTED]

Table 1--Results of Spirometry at Baseline, After Insertion of Tracheal Stent, and After Insertion of Endobronchial Stents

(*) [FEF.sub.25-75] = forced expiratory flow, mid-expiratory phase.

After informed consent, and with the patient under conscious sedation (topical lidocaine 1% applied directly to the oropharynx and larynx, pethidine 125 mg and midazolam 4 mg IV), an endotracheal tube (size 5) could not be passed because of the severity of tracheal narrowing. Via the flexible bronchoscope, and using fluoroscopic guidance, a Bentson wire was advanced into the right lower lobe bronchus. Over the wire, a metallic Wallstent (14 x 60 mm) was deployed in the trachea with the lower aspect of the stent extending to approximately 1 cm above the carina. The wire wits removed, and repeat bronchoscopy confirmed the position of the stent. The patient tolerated the procedure well and noticed an immediate improvement in her dyspnea. Repeat spirometry demonstrated a significant improvement of airflow (Table 1, Fig 2). However her [FEV.sub.1] level and [FEV.sub.1]/FVC ratio remained severely

reduced, and she continued to have limited exercise tolerance (walking [is less than] 60 m because of dyspnea).

Eight weeks after positioning the tracheal Wallstent, a repeat bronchoscopy and placement of endobronchial stents were performed. The tracheal stent was well incorporated into the tracheal wall. The bronchus intermedius was markedly narrowed to a lumen diameter of 2 mm. The entire left mainstem bronchus was also severely narrowed, and the openings for the left upper and left lower lobe bronchi appeared as slitlike orifices of 2 mm at their widest diameter. The lobar bronchi could not be entered. Using an angled guidewire and Berenstein catheter, the right upper lobe bronchus was selectively catheterized. The guidewire was exchanged for a Bentson wire, and the bronchus intermedius was selectively catheterized. A Palmaz stent (model 154; Johnson & Johnson; Warren, NJ) wits deployed on an 8-mm X 2-cm-long balloon in the bronchus intermedius just below the right upper lobe bronchus origin. A further Palmaz 154 stent loaded onto an 8-mm x 2-cm-long balloon wits deployed into the right mainstem bronchus just above the takeoff of the right upper lobe bronchus. The stent was subsequently dilated to 10 mm, then to 12 mm in diameter. The left mainstem bronchus was selectively catheterized, and a Palmaz 294 stent loaded onto an 8-mm x 3-cm-long balloon was deployed in the left mainstem bronchus, and serially dilated to 10 mm. Repeat bronchoscopy confirmed that the proximal portion of the stent in the bronchus intermedius was below the takeoff of the right upper lobe bronchus. Both of the stents within the mainstem bronchi started at the level of the carina and extended to just above their respective upper lobe bronchi.

The patient described a further improvement in dyspnea and a return to normal exercise. Previously a keen golfer, she played her first round of golf in 3 years, 2 weeks after the procedure. The results of repeat spirometry demonstrated a significantly improved [FEV.sub.1] level and [FEV.sub.1]/FVC ratio (Table 1, Fig 2), although they remained reduced. Repeat thoracic CT scan with reconstruction demonstrated improved airway diameter within the stents (Fig 3). On expiration, the caliber of the proximal intralobar bronchus and the distal left mainstem bronchus narrowed to [is less than] 1 mm, whereas on inspiration their diameter expanded to 4 mm. One year after the procedure, the patient remains well and has normal activities of daily living.

[Figure 3 ILLUSTRATION OMITTED]

DISCUSSION

This paper demonstrates that the placement of multiple metallic bronchial stents (under conscious sedation) can successfully alleviate critical airflow limitation caused by RP. We further report that airway stenting in RP wall result ill a good patient outcome and return tv normal activities.[4,6] In this patient, RP was diagnosed on the basis of clinical features: recurrent uveitis, external otitis, costochondritis, arthritis, and laryngotracheobronchial narrowing. In RP, airway closure can arise for one or a combination of reasons: (1) a localized fibrous mass may form within the airway wall, (2) the airway can collapse because of wall destruction, and (3) (as in this case) the airway wall can thicken because of inflammation. Diffuse narrowing of the airway (laryngotracheobronchitis) is a well-recognized complication of RP[1,2] that can progress to life-threatening bronchial infarctions and obstructive pneumonia.[3,6,8] The flow-volume loops and spirometry in this patient revealed severe expiratory airflow limitation (Fig 2).[8,9] The inspiratory and expiratory CT scan reconstructions helped to demonstrate the diffuse nature of the airway disease.

On initial presentation, we were uncertain about the contribution of distal (small) airways tv this patient's airflow limitation, because airways [is greater than] 1 mm in diameter may contain cartilage and be affected by the cartilaginous degeneration of BP. Three-dimensional CT scan reconstructed images (Fig 1) suggested that fourth- and fifth-generation airway diameters were well preserved and that airway narrowing was most severe in the trachea and mainstem bronchi. The dramatic improvement in airway physiology after the insertion of large airway stents supported our theory that narrowing of the trachea and mainstem bronchi contributed significantly to this patient's airflow limitation.[5] A greater improvement in airflow (increases in [FEV.sub.1] level and [FEV.sub.1]/FVC ratio) occurred after the placement of stents in the mainstem bronchi and bronchus intermedius than after the placement of the tracheal stent.

Our limited experience with critical airway compromise in BP suggests that significant improvements in airflow limitation may occur after the placement of metallic endobronchial stents in the trachea and mainstem bronchi. Placement of endobronchial stents at the main points of airflow obstruction can help to avoid tracheotomy and mechanical ventilation, and may lead to improved patient functionality and survival.

REFERENCES

[1] Carrion M, Giron JA, Ventura J, et al. Airway complications in relapsing polychondritis. J Rheumatol 1993; 20:1628-1629

[2] Eng J, Sabanathan S. Airway complications in relapsing polychondritis. Ann Thorac Surg 1991; 51:686-692

[3] Sane DC, Vidaillet HJ Jr, Burton CS 3d. Saddle nose, red ears, and fatal airway collapse: relapsing polychondritis. Chest 1987; 91:268-270

[4] Sacco O, Fregonese B, Oddone M, et al. Severe endobronchial obstruction in a girl with relapsing polychondritis: treatment with Nd YAG laser and endobronchial silicon stent. Eur Respir J 1997; 10:494-496

[5] Lehman JD, Gordon RL, Kerlan RK Jr, et al. Expandable metallic stents in benign tracheobronchial obstruction, J Thorac Imaging 1998; 13:105-115

[6] Dunne JA, Sabanathan S. Use of metallic stents in relapsing polychondritis. Chest 1994; 105:864-867

[7] Wallace MJ, Charnsangavej C, Ogawa K, et al. Tracheobronchial tree: expandable metallic stents used in experimental and clinical applications. Radiology 1986; 158:309-312

[8] Krell WS, Staats BA, Hyatt RE. Pulmonary function in relapsing polychondritis. Am Rev Respir Dis 1986; 133:1120-1123

[9] Mohsenifar Z, Tashkin DP, Carson SA, et al. Pulmonary function in patients with relapsing polychondritis. Chest 1982; 81:711-717

(*) From the Division of Pulmonary and Critical Care Medicine (Drs. Faul and Rizk) and the Department of Interventional Radiology, (Dr. Kee), Stanford University Medical Center, Stanford CA.

Manuscript received March 30, 1999; revision accepted April 18, 1999.

Correspondence to: Norman W. Rizk, MD, FCCP, Division of Pulmonary Medicine, Stanford University Medical Center, Stanford CA 94305; e-mail: rizk@forsythe.stanford.edu3

COPYRIGHT 1999 American College of Chest Physicians
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