At ILD conference this week, a patient with progressive RA-ILD was discussed. A change in the patient’s rheumatoid arthritis medication was to be determined with her rheumatologist, but she was also recommended to start nintendanib
I. What is the evidence for anti-fibrotic therapy outside of IPF?
Prior to 2019, the efficacy of antifibrotic therapy in non-IPF fibrosing lung disease was unknown. INBUILD was a double-blind, placebo controlled RCT to investigate the efficacy of antifibrotic therapy in non-IPF fibrosing lung disease.
Let’s approach this study using the PICO framework!
Population:
All patients had to meet criteria for progression of ILD in the past 24 months despite treatment with an FVC =< 45% and DLCO <80%.
Breakdown of population by diagnosis:
·
Intervention:
Nintedanib 150mg BID
Comparison:
Placebo
Outcomes:
- Primary endpoint=annual rate of decline in FVC
- The annual rate of decline in FVC was significantly lower in patients who received nintedanib than those who received placebo.
- Diarrhea was a common adverse event
The patient population was stratified by ILD with or without a UIP pattern of fibrosing ILD. Nintendanib decreased the rate of decline in FVC regardless of the pattern of fibrosis in this patient population.
In another case, our thoracic radiologist Dr. Parekh pointed out an example of dendritic pulmonary ossification.
II. What is dendritic pulmonary ossification (DPO)?
- Chronic, progressive metaplastic ossification of lung parenchyma
- Pattern of ossification resembles the dendrite of a neuron
Top: neuron with dendrites
Bottom: coronal view of CT showing dendritic pulmonary ossification
- Dendritic pulmonary ossification is a rare condition (1.6/1000 autopsies) and is associated with IPF, ARDS, COPD, organizing pneumonia, rare earth pneumoconiosis, asbestosis, heavy metal exposure, and chronic aspiration.
- Many cases are idiopathic and several weak associations with other non-pulmonary diseases have been reported in the literature.
Sources:
- Flaherty et al. N Engl J Med 2019;381:1718-27. DOI: 10.1056/NEJMoa1908681
- Fernández-Bussy et al. Respiratory Care April 2015, 60 (4) e64-e67; DOI: https://doi.org/10.4187/respcare.03531
- Reddy TL, von der Thüsen J, Walsh SF. . Idiopathic Dendriform Pulmonary Ossification. Journal of Thoracic Imaging. 2012; 27 (5): W108-W110. doi: 10.1097/RTI.0b013e3182326c38.