View Abstract

A6448 - Unrelenting Tracheobronchial Stenosis in Relapsing Polychondritis
Author Block: S. Murney1, C. Radchenko1, R. W. Shepherd2; 1Virginia Commonwealth University Health System, Richmond, VA, United States, 2VCU Med Ctr, Richmond, VA, United States.
Introduction:
Relapsing polychondritis (RP) is a rare autoimmune disease characterized by periodic episodes of cartilaginous inflammation, most commonly affecting the ears, nose, and airways. The lower respiratory tract is affected in 20-50% of patients and portends a poor prognosis. The higher mortality rate in this subset is attributed to luminal collapse from severe tracheobronchomalacia and large airway stenosis.
Case:
62 y/o female with rheumatoid arthritis and chronic hepatitis C was diagnosed with RP thirty years prior and had been maintained solely on hydroxychloroquine. After establishing care with rheumatology seven years ago, azathioprine was started for uncontrolled joint pains. She suffered recurrent tracheobronchial stenosis initially requiring eighteen bronchoscopies with balloon dilation of multiple bronchial segments, often the left mainstem bronchus (LMB), to maintain airway patency. Given recurrence of tracheobronchial stenosis, a 9 x 30 mm silicone stent was placed within the LMB. Unfortunately within months her stent occluded with granulation tissue and had migrated such that it was removed along with debridement of the granulation tissue. Subcutaneous etanercept was then added to her medical regimen without improvement in tracheobronchial inflammation. The LMB continued to restenose requiring frequent balloon dilations, intra-lesional methylprednisolone injections, and spray cryotherapy that were all unsuccessful in providing sustained effect. Attempts at repeat stenting were made with a customized 9 x 40 mm beveled silicone stent in the LMB and the patient seemed to have significant improvement in symptoms. Nevertheless, within three months the stent had developed significant granulation tissue and distal tracheal obstruction necessitating retrieval. Thirty-seven bronchoscopies later, the unrelenting process of restenosis continues despite extensive efforts in treatment.
Discussion:
The pathophysiology of RP is poorly understood and given its rarity, the treatment remains largely empirical. Glucocorticoids are the mainstay treatment for acute flares, however steroid-sparing agents are the preferred maintenance therapies. Methotrexate is first-line treatment followed by azathioprine and mycophenolate mofetil as second-line. In more severe cases, such as refractory tracheobronchial involvement, TNF-α inhibitors have been used with reported benefit. Failure of early diagnosis of airway involvement can lead to irreversible cartilage damage making adequate therapeutic options limited. Such as with this case, escalating immunosuppression, frequent balloon tracheobronchoplasty, tissue debridement, customized stent placement, and adjunctive endobronchial salvage therapies may be required. This unfortunate case highlights the frustrating reality of RP with airway involvement despite adequate immunosuppression and the necessity that these patients are established in a healthcare system well equipped to perform advanced bronchoscopy.