How do you approach patients with RA and severe bronchiectasis with associated findings of UIP?

Usual Interstitial Pneumonia or UIP is among the most serious forms of RA-associated lung disease. Clinical studies have demonstrated that its prognosis is similar to idiopathic pulmonary fibrosis (IPF). UIP is extremely challenging to treat and we have learned from experience that our DMARDs and biological therapies do not favorably impact its clinical course. 

For the patient with RA and bronchiectasis and UIP, the decision would be whether to try an anti-fibrotic therapy such as pirfenidone or nintedanib. There is preliminary data suggesting slight benefit for their use in patients with IPF and related fibrosing disorders.

The need for either a DMARD or a biological drug to treat the underlying RA may still be necessary though one should not expect these agents to be helpful in managing the lung disease.