How would you approach a pulmonary-renal PR3+ ANCA vasculitis patient who has persistent re-narrowing of mainstem bronchus after several dilatation and stenting procedures, with other anca features well-controlled on rituximab & avacopan?

Obviously, this is going to be a very challenging management issue. Bronchial or tracheobronchial stenosis evolves independently of other organ involvement and often fails to respond to immune suppressive regimens. There is one anecdotal study from France, suggesting that cyclophosphamide may offer ...

Tracheobronchial involvement in GPA can be difficult to treat and is often decoupled from other vasculitis disease activity. Both local and systemic therapy are important for treating the disease. Dilation, often with local corticosteroid therapy is often attempted but restenosis is common. Other in...

Agree with @Dr. First Last- the Rx of this is local/regional and requires highly experienced and skilled interventionalists. Medical management is generally futile when this is occurring in the setting of quiescent disease which is common.

This is a true treatment dilemma, unfortunately.

Other than considering a step up on immunosuppression, it might not be a bad idea to take the following into consideration.

1. Dilation itself could induce ischemic injury and increase local inflammation. Sometimes we do have to consider limiting the f...

This is a difficult problem. Assuming surgical sleeve resection is not an option, the other bronchoscopic approach that can be tried in this case is TrueFreeze Cryospray ablation of the tissue at the stenotic site followed by Kenalog injection. This typically requires several treatments but I have h...