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Angiotensin-converting enzyme (ACE) inhibitors are the mainstay of therapy for patients with systemic sclerosis in scleroderma renal crisis, however mortality remains high even in the post-ACE inhibitor era. presented at the American College of Rheumatology annual meeting showed the effects of early initiation of ACE inhibitor treatment on long-term outcomes for patients in scleroderma renal crisis.

In this exclusive 番茄社区app video, study investigator Aditi Patel, MD, discusses the findings.

Following is a transcript of her remarks:

So scleroderma renal crisis is a rare complication of scleroderma where this can be associated with high mortality. Over the last few years, it has been seen that scleroderma renal crisis, until ACE inhibitors were introduced, was a setting that had very high mortality. But despite this, and despite ACE inhibitors use over the last few years, the mortality has still remained high. When we looked at our data over the past few years and compared with other centers where scleroderma renal crisis is seen, we found that there was a timing to initiation of therapy that was critical, and we wanted to explore this more into how it affects our outcomes.

So our cohort included 27 patients that had scleroderma renal crisis. There were two patients that were normotensive, 93% of the patients had high blood pressure, and all of them had an acute kidney injury.

It was found that dialysis was initiated for about 50% of the patients, whereas there were three patients that did not want to be treated with dialysis. The patients who were started on ACE inhibitors after 48 hours of symptoms were more likely to need dialysis compared to the others who were started on early ACE inhibitors. Early initiation of ACE inhibitors within 48 hours, although, did not affect the survival, and the mortality still remained high.

When we looked at a follow-up cohort of our patients, the mortality rate was as high as 70%. Whereas within the first year the mortality rate was about 58%, whereas within 2 years it was 21%, and within 5 years it was 21% as well.

So it is really important in our study that it's critical to identify patients that have scleroderma, one, as we did have a lot of patients that had a newer diagnosis of scleroderma that was diagnosed when this complication was being manifested. And, it is important to identify triggers as we already know, such as steroids, that will prevent this from happening down the road.

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