What immunosuppression regimens do you use in patients with progressive RB-ILD despite smoking cessation and prednisone?

In patients with progressive RB-ILD who continue to worsen despite quitting tobacco use and prednisone, treatment options are very limited. Since there are no RCTs specifically studying immunosuppressive treatment in RB-ILD, most available evidence comes from observational studies, and most recommendations are drawn from research on other ILDs. Limited data shows that drugs like azathioprine or mycophenolate may lead to some improvement, but usually it is not meaningful, and the disease progress...

I have definitely used immune suppressants (azathioprine, mycophenolate, and tacrolimus) in progressive RB-ILD despite smoking cessation and prednisone. As with other forms of ILD, I try to limit exposure to prednisone if at all possible.

If there is fibrosis, I would not hesitate to use an anti-fibrotic drug (at the time of this writing, in the US, only nintedanib is approved for this indication).