Post 10 - Rapidly Progressive ILD Phenotype
About RP-ILD
Case:
60-years old with a usual interstitial pneumonia (UIP) pattern first presented in Jun 23.

The coronal image showed a straight-edge sign and the anterior upper lobes showed more honeycombing than the bases.

He was worked up for connective tissue disease, but all the markers were negative.
He had 3 more scans till the last one of April 2026.

This April 2026 scan shows marked increase in the fibrosis, traction bronchiectasis and honeycombing.

There was rapid progression from June 23 to April 26.
If you see the rate of progression, this is a rapidly progressive ILD (RP-ILD) phenotype, commonly seen with inflammatory myositis associated ILD. However, it is conceivable that in some instances, any ILD, including IPF, which this would now be diagnosed as (idiopathic since there is no etiology) could progress rapidly.
He has been extensively worked up but till Apr 2026, there was no obvious cause found. Genetic studies have been non-contributory.
Here is a short video, where I go through the case on Osirix.
I also did a video, case of the day 80 on this subject last year (see below).
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