Oh, what a shocking revelation we have here! In the groundbreaking world of medical research, someone decided to take a peek into the use of renin-angiotensin system (RAS) inhibitors in those rare unicorns known as Fabry disease (FD) patients with kidney issues. Because, you know, it’s not like kidney injury is a big deal in FD or anything. And who would have thought that RAS inhibitors, which are only known to every nephrologist and their grandma for reducing proteinuria in chronic kidney disease (CKD), might actually be used in clinical practice? Mind-blowing!

Enter the J-CKD-DB-Ex, the database hero we didn’t know we needed, with its treasure trove of data on a whopping 187,398 patients. From this vast sea of information, researchers managed to fish out 39 FD patients, confirming a grand total of 22 cases. And hold your applause, because here’s the kicker: half of these patients were actually on RAS inhibitors. The other half? Well, they were just enjoying the natural progression of their disease, I guess.

But wait, there’s more! It turns out that RAS inhibitors were more likely to be used in patients with more severe kidney damage. Because why give a drug that could prevent damage before it’s severe, right? That would just make too much sense.

So, what have we learned from this case series? That in the real world, FD patients with CKD are sometimes treated with RAS inhibitors, sometimes not. And that databases can indeed tell us what we’re actually doing in clinical practice, especially for those rare diseases that we don’t see every day. Who would’ve thought?