Rare organ sarcoidosis marker for more severe disease, but infrequently requires treatment
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Although rare organ involvement with sarcoidosis may be indicative of more severe disease, it often does not require treatment, according to data published in Respiratory Medicine.
In a retrospective cohort study, Marc A. Judson, MD, professor of medicine and chief of pulmonary and critical care medicine at Albany Medical College, and colleagues sought to determine the frequency of rare organ sarcoidosis, defined as an organ not individually listed in the World Association of Sarcoidosis and Other Granulomatous Disorders Sarcoidosis Organ Assessment Instrument, and examined potential differences in demographic and clinical features among patients with rare organ involvement.
“I was asked to write a review article on rare organ sarcoidosis and, in writing that article, I realized how little information there is concerning the topic. I also realized that it’s an important topic because clinicians are occasionally faced with dealing with rare organs with sarcoidosis and they don’t really know what to do and they don’t have much literature or information available to them,” Judson told Healio.
Among the 1,158 patients with sarcoidosis seen during 11 years at a university outpatient sarcoidosis clinic, 2.2% had rare organ involvement. The most commonly involved rare organ was the gastrointestinal tract, with the stomach being the most commonly involved organ of the gastrointestinal tract, followed by the testicles and female reproductive tract, according to the data.
Results showed that, compared with patients with no rare organ involvement, a greater proportion of patients with rare organ involvement were Black (30.8% vs. 17.6%) and had significantly more organs involved with their sarcoidosis (median, 3 vs. 2 organs). The researchers found no differences between patients with and without rare organ involvement based on sex, age at diagnosis and most recent percent predicted FVC.
The fact that patients with rare organ involvement appeared to have more severe disease, at least based on some criteria, was a particularly interesting finding, Judson noted.
“Sarcoidosis is a disease that doesn’t necessarily need treatment. In mild cases, you can just monitor the patient without therapy. What we found was that the ones who had rare organ involvement were much more likely to require treatment for the disease,” Judson said.
Specifically, 79.6% of those with rare organ involvement required sarcoid-specific therapy at some point during the course of their disease compared with 54.7% of those without such involvement. As for why this might be, Judson noted that these patients likely have more disseminated disease.
“If you have disease in rare organs, it probably means you have more extensive disease throughout the body and the need for therapy is just a manifestation of that,” Judson said. “Also, this is confusing but actually does make sense: Even though patients with rare organ sarcoidosis were more likely to need treatment for sarcoidosis, it was very unlikely for them to require treatment of the rare organ sarcoidosis itself. In fact, in the 26 patients who had rare organ sarcoidosis, only one required treatment. Nevertheless, that group in general required more treatment of their sarcoidosis than those who didn’t have rare organ involvement.”
That one patient requiring therapy received anti-inflammatory treatment for granulomatous inflammation due to a malabsorption syndrome from sarcoidosis involving the small intestine.
The researchers’ findings also highlighted several important points related to treatment of sarcoidosis, according to Judson.
“A key point here is that rare organ sarcoidosis rarely needs to be treated. A lot of times, clinicians just reflexively treat sarcoidosis,” Judson said. “However, the treatment can be toxic and often these patients don’t get any significant consequences from their disease, and this seems to be the case almost universally with rare organ sarcoidosis, so although it’s a marker for severe disease, rarely does it require treatment.”
Judson noted that a future study with a different design would be beneficial and hopefully offer more insight into sarcoidosis.
“It would be hard to do, but it would be interesting if we could get over one of the limitations of this study, which was that rare organ involvement was not searched for systematically,” Judson said. “In other words, we didn’t look at every organ in every patient to really get the true incidence of rare organ involvement. It would be nice to at least systematically look at rare organ involvement in a prospective fashion. We could get a little more information, but that might be hard to do in practical terms.”
For more information:
Marc A. Judson, MD, can be reached at judsonm@amc.edu.