Date: Monday, October 22, 2018
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: Tracheomalacia (TM) is pathological diffuse or segmental narrowing of the tracheal lumen, caused by softening of the supporting cartilage and reduction in stiffness of the walls. If the weakness extends to the right or left main bronchi then it is termed tracehobronchomalacia (TBM). Tracheomalacia can occur due to relapsing polychondritis, other etiologies include mechanical compression due to intubation or goitre. Although there have been a number of descriptions of TBM, it has not been recognised as an entity in itself. We present here a series of patients who have TBM without an alternative diagnosis to explain TBM.
Methods: These patients were identified from the TBM database. Patients were identified from rheumatology or respiratory clinics or from acute admissions with severe breathing difficulties and found to have TBM. The TBM database was set up in 2017, although the first patient was diagnosed in 2013. Patients who were initially labelled as TBM and subsequently found to have RP or another explanation for TBM were excluded from this study.
Results: We found 25 patients who had TBM, of these mechanical causes were found in only 1 patient. The diagnosis was established through inspiratory and expiratory CT scans and/or bronchoscopy in most patients and supported with pulmonary function testing which suggested large airway obstruction (intrathoracic) for the majority of patients. Patients often had symptoms for a number of years prior. A number of patients had previous CT scans which had shown evidence of TBM, this was either missed or ignored. 15 patients were treated with immunosuppression, and most of them had extremely good response to immunosuppression. These have been labelled as ‘inflammatory tracheobronchomalacia’ as there is no alternative label for these patients. Drugs used in these patients include steroids (IV and oral), Methotrexate, Azathioprine, Mycophenolate and Rituximab. All patients have noticed very significant results with steroids, 9 patients have not been given immunosuppression currently. CPAP has been tried in 7 patients, only 1 patient struggled to tolerate this. In one patient, tracheobronchomalacia evolved into relapsing polychondritis, suggesting that TBM could be a ‘forme fruste’ of an inflammatory condition.
Death has occurred in 6 patients who have been treated with immunosuppression and 2 patients who has not been treated with immunosuppression.
Conclusion: Tracheobronchomalacia is a relatively common condition with high mortality, which is not very well characterised. Optimal management strategies are yet to be defined.
This is the first report for ‘inflammatory tracheobronchomalacia’ suggesting that immunosuppression might have a significant role in management of TBM.
To cite this abstract in AMA style:Pink G, Dubey S, Ali A, Shakespeare J, Taylor C, Gelder C. Tracheomalacia in Coventry in UK [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/tracheomalacia-in-coventry-in-uk/. Accessed January 22, 2020.
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