Polychondritis

Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation and destruction of the cartilaginous structures. Tracheobronchial chondritis is a dreaded complication of RP. We wish to report a case of RP of the trachea and bronchi which was treated with nasal continuous positive airway pressure. (CHEST 1997; 112:1701-04)

Key words: CPAP; relapsing polychondritis; tracheomalacia

Abbreviations: CPAP = continuous positive airway pressure; RP = relapsing polychondritis

Relapsing polychondritis (RP) is a rare disease of unknown cause characterized by inflammation of the cartilaginous structures. It typically manifests as auricular chondritis, polyarthritis, and nasal chondritis.[1,2] Involvement of the respiratory tract is a dreaded complication and typically occurs later in the course of RP. Up to 50% of patients will die from respiratory tract complications. Treatment for RP-related tracheomalacia is limited and surgical repair is generally restricted to patients with localized disease. Herein is the report of a patient who had dramatic and extensive tracheomalacia as the sole feature of RP. Continuous positive airway pressure (CPAP) was used effectively during her illness.

Case Report

A 46-year-old woman was transferred to the Toronto Hospital for management of subglottic stenosis. She had been managed in the past for presumed asthma with bronchodilators and oral corticosteroids. She never smoked and had no occupational exposures. Six months prior to admission she developed increasing paroxysms of breathlessness and cough. Her activities of daily living were becoming increasingly limited owing to persistent dyspnea, and she eventually became completely bedridden. There was no history of fever or sputum production. She had no eye or otolaryngologic complaints. Examination revealed use of accessory muscles, and marked inspiratory and expiratory wheezing was heard throughout both lungs. There was no evidence of auricular or nasal abnormalities. Plain chest radiography was normal. A CT scan of the lung was performed and demonstrated diffuse narrowing of the tracheobronchial tree (Fig 1). There was also diffuse thickening of the bronchial walls. Small flecks of calcium in the bronchi were seen in some areas. Rigid bronchoscopy demonstrated extensive airway collapse during expiration. Repeated endobronchial biopsies taken at that time revealed only dense subepithelial fibrosis and patchy squamous dysplasia. The diagnosis of idiopathic tracheobronchomalacia was made. She was treated with prednison, 30 mg/d. Because of the severity of her symptoms and extensive respiratory tract involvement, she began receiving nasal CPAP at 10 cm [H.sub.2]O. This was accompanied by a marked improvement in her breastlessness and reduction in bronchodilator use. Episodes of wheezing and stridor occurred less frequently and responded to nebulized racemic epinephrine.

Over the next 2 months, her level of activity increased and she felt well. Attempts to discontinue the nasal CPAP were accompanied by a marked increase in respiratory distress. A subsequent CT scan performed with and without CPAP was done, but it could not be reliably determined if CPAP increased the bronchial caliber. A portable battery-operated CPAP unit (Respironics; Murrysville, Pa) was provided to improve ambulation and allowed her to venture outdoors. The unit was attached to a rechargeable 12-V marine battery linked to an AC/DC power converter. The battery lasted approximately 9 h and required 12 h to recharge. The entire system was mounted to a patient walker with the battery bolted into the basket and covered. The CPAP unit was fastened to the seat of the walker to facilitate the unit's movement from the walker to the bedside. To facilitate further independence, she also used a Down's mask with an expiratory positive airway pressure of 10 cm [H.sub.2]O. The Down's mask is a full-face mask with two one-way valves -- an inspiratory valve at the level of the mouth and an expiratory valve above it. A spring-loaded positive end-expiratory pressure valve was attached to the expiratory port to provide an expiratory positive pressure. The patient found this to be less comfortable than nasal CPAP. The Down's mask was used as a backup system in case of equipment failure and for activities such as bathing.

Unfortunately, 3 months following the initiation of nasal CPAP, her breathlessness recurred and was refractory to an increase in CPAP to 12.5 cm [H.sub.2]O. She also noted difficulty with clearing secretions due to pain from recent lumbar fractures related to steroid therapy.

She was readmitted to our hospital and a T-Y stent was placed through the third tracheal cartilage, and its position was confirmed bronchoscopically. This stent is made of a soft pliable plastic (Silastic; Benson Hood Laboratories; Pembroke, Mass) and was positioned cephalad 1 cm below the vocal cords and extended midway down to the left mainstem bronchus and down the right stem bronchus to the upper lobe takeoff of the right lung. Her symptoms were significantly improved with the T-Y stent; however, she experienced difficulty, in clearing secretions. The stent was later replaced with a Montgomery T tube to allow for easier clearance of secretions. After surgery, the patient continued to require nasal CPAP intermittently (with the T tube corked) for episodes of stridor and respiratory distress. Eventually, a tracheobronchoplasty was performed with the use of Marlex surgical mesh (Davol, Inc; Cranston, RI) in an attempt to create permanent airway stenting. Via a right thoracotomy, the entire airway from the apex of the chest to the bronchial bifurcations bilaterally was exposed. A double sheet of Marlex (Trelex; Meadox Medical, Inc; Oakland, NJ) was sutured to the tracheobronchial tree with multiple, interrupted 4-0 Ticron (Ethicon; Somerville, NJ) sutures in order to "mattress" the Marlex to the membranous trachea and bronchus.

After a 2-week period of improvement, she developed episodes of hypoxia, pneumonia, and mucus plugging which required mechanical ventilation. Unfortunately her course was further complicated by dehiscence of the suture line in the posterior membranous trachea. Attempts to ventilate the patient using high-frequency jet ventilation were unsuccessful, and the patient died. An autopsy was performed and revealed erosion of the tracheal and bronchial cartilage by a mixed population of inflammatory consistent with the diagnosis of RP (Fig 2). The inflammatory process was diffuse and extended well beyond the region of the airway that was stented. Consequently, the histologic findings were not secondary to trauma or from manipulation of the airway.

Discussion

Patients with RP typically present with auricular chondritis, inflammatory arthritis, and nasal chondritis. Respiratory tract involvement in RP is an uncommon presenting feature, but its incidence increases as the disease progresses. Females develop airway complications more frequently.[2-4] Patients who present with respiratory tract involvement have a worse prognosis than those with other organ involvement and have a poorer response to oral corticosteroids.[2.3,5] The patient reported herein was unusual in that she had no other organ involvement from RP apart from extensive and severe airway disease.

The mainstay of therapy for RP consists of nonsteroidal anti-inflammatory drugs, high-dose corticosteroids, and immunosuppressants.[2,3,6] There are few therapeutic options available for patients who develop airway complications. During acute exacerbations of respiratory RP, racemic epinephrine may be beneficial in relieving symptoms.[7] Surgical interventions may not be beneficial in patients with extensive airway involvement but may be useful for localized diseased areas.[8] The few surgical options available for RP include tracheotomies and Montgomery T-tube stents. Most case reports, of surgical resection or tracheoplasties have shown poor outcomes and are thus rarely considered palliative.[3,6,9] In fact, if the diagnosis of RP had been made earlier, surgery would not have been performed.

There is no mention in the medical literature of the use of nasal CPAP for the treatment of RP. Nasal CPAP is noninvasive, is relatively simple to use, and has few side effects. It effectively acts as a "pneumatic" splint for the affected airway and prevents tracheobronchial collapse.[10] CPAP has been reported in the treatment of patients with variable intrathoracic obstruction.[11] As best as can be determined, only one previous report has described the use of CPAP in adults with tracheomalacia. Ferguson and Benoist[10] described three patients with tracheobronchomalacia in whom CPAP therapy improved airflow limitation. This was demonstrated by comparing spirometry values with and without the use of CPAP and by using bronchoscopically recorded pictures to demonstrate an improvement in the patency of the lower airway during exhalation while the patient was receiving CPAP. There have been several case reports describing the use of CPAP in tracheobronchomalacia in children.[8,12] In one report in infants, the level of CPAP required was determined fluoroscopically by monitoring airway collapse during the breathing. The level of expiratory pressure was then titrated to the level that abolished the segmental collapse.[8] In the patient reported herein, an attempt was made to use CT to demonstrate the degree of airway collapse and document the effect of CPAP. Improvement in airway caliber could not reliably be detected using this imaging modality. Spiral CT scans may have more reliably demonstrated collapse of the airway during breathing. Unfortunately, this technology was not available at the Toronto Hospital at the time.

Due to the patient's dependence on CPAP, a backup system was required in case of equipment failure. Unfortunately, the expiratory positive airway pressure system or Down's mask was tolerated for only short periods of time. This system required a significant increase in inspiratory effort since large subatmospheric pressures had to be generated to open the mask's valves.

Our patient improved with relatively low levels of expiratory pressure via CPAP. With few surgical options available for patients with diffuse respiratory tract disease from RP, nasal CPAP represents a potentially effective, albeit temporary therapeutic modality.

References

[1] Sane DC, Vidaillet HJ Jr, Burton CS 3rd. Saddle nose, red ears, and fatal airway collapse: relapsing polychondritis. Chest 1987; 91:268-70

[2] McAdam 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

[3] Eng J, Sabanathan S. Airway complications in relapsing polychondritis. Ann Thorac Surg 1991; 51:686-92

[4] Dunne JA, Sabanathan S. Use of metallic stents in relapsing polychondritis. Chest 1994; 105:864-67

[5] Neilly JAB, Winter JH, Stevenson RD. Progressive tracheobronchial polychondritis: need for early diagnosis. Thorax 1984; 40:78-9

[6] Irani BS, Martin-Hirsch DP, Clark D, et al. Relapsing polychondritis -- a study of four cases. J Laryngol Otol 1992; 106:911-14

[7] Gaffney RJ, Harrison M, Blayney AW. Nebulized racemic ephedrine in the treatment of acute exacerbations of laryngeal relapsing polychondritis. J Laryngol Otol 1992; 106:63-64

[8] Sotomayor JL, Godinez RI, Borden S, et al. Large-airway collapse due to acquired tracheobronchomalacia in infancy. Am J Dis Child 1986; 140:367-71

[9] Goddard P. Relapsing polychondritis: report of an unusual case and a review of the literature. Br J Radiol 1991; 64:1064-67

[10] Ferguson GT, Benoist J. Nasal continuous positive airway pressure in the treatment of tracheobronchomalacia. Am Rev Respir Dis 1993; 147:457-61

[11] King JW, Walsh TE. Variable intrathoracic upper airway obstruction due to non-small cell lung cancer: palliation using physiologic and mechanical stenting. Chest 1986; 89:896-98

[12] Kanter RK, Pollack MM, Wright WW, et al. Treatment of severe tracheobronchomalacia with continuous positive airway pressure (CPAP). Anesthesiology 1982; 57:54-56

COPYRIGHT 1997 American College of Chest Physicians
COPYRIGHT 2004 Gale Group