CLEARING THE UNDERBRUSH AND PROTECTING THE CANOPY: WILDFIRE MANAGEMENT IN TRACHEOBRONCHIAL STENOSIS FROM GRANULOMATOSIS WITH POLYANGIITIS
Nicholas Clough, MD, Jesse E. Doyle, MD, Adrian Singson, MD, Roger F. Johnson, MD, FCCP
Introduction: Upper airway stenosis is a known complication of Granulomatosis with polyangiitis (GPA). We present a case demonstrating the complexities of managing this potentially life-threatening disease and discuss evidence for interventions.
Case Presentation: A 73-year-old man was admitted to the ICU with acute exacerbation of chronic obstructive pulmonary disease provoked by community-acquired pneumonia, complicated by tracheobronchial stenosis (TBS) attributable to granulomatosis with polyangiitis (GPA). A prior episode of acute respiratory failure necessitated urgent direct laryngoscopy and dilation of a newly diagnosed tracheal stenosis. At that time, 60% subglottic stenosis (SGS) was revealed. A 5.5 endotracheal tube (ETT) was used to maintain airway patency in between initial dilatation attempts. One month after discharge, re-evaluation by laryngoscopy showed an improved intraluminal diameter with 50% SGS, accommodating a 6.5 ETT with noted reduction in tracheal mucosal erythema. The patient was diagnosed with GPA via positive p-ANCA and myeloperoxidase antibodies. This prompted referral for potential endoscopic intervention by interventional pulmonology. However, the patient's non-adherence with proposed medical management led to subsequent loss of follow-up. After 18 months without incident, he sought care again for the above scenario. Given normal inflammatory markers and absence of extra-pulmonary vasculitis signs, the on-call otolaryngologist advised transfer for interventional pulmonology assessment and outpatient rheumatology referral. The patient opted against immediate transfer and elected to postpone intervention until further recovery with a provisional plan for outpatient treatment.
Discussion: GPA is an ANCA-associated vasculitis that selectively targets small and medium-sized arteries with a predilection for the respiratory and renal systems. Historically, GPA was fatal within months; contemporary treatments now achieve over 80% five-year survival and 75% complete remission. Despite these advances, relapses occur in 50-70% of cases, some of which result in irreversible disease features. There is limited literature on the prognosis and characteristics of SGS and TBS within the context of systemic inflammatory diseases. These stenotic sequelae of GPA can cause upper airway obstruction and pose life-threatening risks. These stenoses, independent of disease activity, present diagnostic challenges in differentiating active inflammation from scarring. GPA is estimated to involve the tracheobronchial tree in 12-23% of cases. SGS–characterized by a circumferential distribution without calcification–is the most common manifestation. A minority of cases include bronchial involvement and tracheobronchomalacia is rare. Clinical manifestations such as stridor and dyspnea may necessitate urgent interventions, including tracheostomy in severe cases. Although endobronchial disease occurs less commonly, its symptomatology parallels that of SGS. Management of SGS and TBS associated with GPA remains complex, as systemic treatments are often inadequate, and relies on bronchoscopic interventions. Endoscopic interventions, while beneficial, carry a high restenosis rate. Effective management requires dilatation with further local intervention. Local steroid injections, laser therapy, cryotherapy, or topical mitomycin may mitigate restenosis. Preliminary data indicate that adjunctive high-dose corticosteroids during intervention could foster better outcomes, while the benefits of immunosuppressive therapies remain equivocal.
Conclusions: GPA's airway manifestations, notably SGS and TBS, pose significant management challenges. Future studies should focus on early detection, risk stratification, and therapeutic refinement to improve patient outcomes.
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