Pulmonary Procedures |

Tracheobronchial Amyloidosis: Therapy and Outcomes in 24 Patients FREE TO VIEW

Sumedh Hoskote, MBBS; Ana Zamora, MD; Sanjay Kalra, MD; Jay Ryu, MD; Fabien Maldonado, MD
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Mayo Clinic, Rochester, MN

Chest. 2015;148(4_MeetingAbstracts):798A. doi:10.1378/chest.2266029
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SESSION TITLE: Interventional Pulmonary

SESSION TYPE: Original Investigation Slide

PRESENTED ON: Sunday, October 25, 2015 at 04:30 PM - 05:30 PM

PURPOSE: Management options for tracheobronchial amyloidosis (TBA) include watchful waiting, external beam radiation therapy (EBRT), endobronchial and surgical excision. We aimed to review our experience to identify effective therapeutic options for TBA.

METHODS: Retrospective review of patients treated for biopsy-proven TBA between 1997 and 2015.

RESULTS: A total of 24 patients with follow-up data available were identified, of which 14 (58%) were male and 17 (71%) were current/former smokers. Median age at diagnosis was 58.5 (interquartile range [IQR] 48-70) years. Median follow-up was 4 (IQR 1-11) years. Initial therapy used was EBRT (20 Gy in 10 divided doses) in 11 (46%), surgery in 5 (21%), bronchoscopy with laser or forceps-assisted debulking in 2 (8%) and watchful waiting in 6 (25%) patients. Those treated with EBRT required 1-3 courses of treatment (median=1). Endobronchial laser was used in 10 (42%) patients during the course of disease. Clinical improvement was seen in 12 (50%) patients. Median time to improvement after initial EBRT was 4.5 (IQR 3-12.5) months. If surgical or bronchoscopic excision of amyloid was complete, improvement was immediate, but required further procedures if excision was partial. In 9 (38%) patients, symptoms remained stable, and in 3 (12%) patients, deterioration was noted. Initial surgical excision was more likely to result in clinical improvement (100% vs. 37%, p=0.04). Symptomatic recurrence was seen in 7/18 (39%) treated patients, and was similar whether initial therapy was surgical 2/5 (40%), EBRT 4/11 (36%) or bronchoscopic 1/2 (50%) (p=1.0). Median time to recurrence was 64 (IQR 27-144) months. No recurrences were noted after pneumonectomy or lobectomy. Patients who had no TBA-related symptoms were observed without specific treatment. Seven (29%) deaths occurred during follow-up, of which 5 were from TBA-related complications. Median duration from TBA diagnosis to death was 77 (IQR 42-135) months.

CONCLUSIONS: TBA often has a chronic course after initial diagnosis. Initial therapies are variably effective and a combination of modalities is commonly used. Recurrences are common and surgery may be considered in difficult cases. Patients often remain stable for prolonged periods and short-term mortality was uncommon.

CLINICAL IMPLICATIONS: Surgical excision of amyloid deposits, when anatomically possible, offers the best chance of clinical improvement but is still associated with recurrence rates similar to EBRT or endobronchial laser therapy.

DISCLOSURE: The following authors have nothing to disclose: Sumedh Hoskote, Ana Zamora, Sanjay Kalra, Jay Ryu, Fabien Maldonado

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