Blood clots in brain may be rare complication of primary Sjögren’s
Patients affected with CVT may not show dry mouth, eye symptoms: Study
A rare condition called cerebral venous thrombosis or CVT, characterized by blood clots forming in veins in the brain, can occur in people with primary Sjögren’s disease, a new study reports.
The findings suggest that Sjögren’s patients with co-occurring CVT don’t always show well-known Sjögren’s symptoms, such as dry mouth and dry eyes. Based on this, the researchers emphasized the need to screen for autoimmune diseases, including Sjögren’s, in people who experience CVT without a clear underlying cause.
Importantly, the researchers noted that all but one of the dozen primary Sjögren’s patients identified in their study and literature review as having these brain blood clots were women, with an average age slightly older than 40. As such, female patients may potentially be more likely to be affected by the rare condition.
“Middle-aged women with [primary Sjögren’s syndrome] should be alert to the presence of CVT,” the researchers wrote, noting that, overall, “patients may have [a] good prognosis when effective treatment is administrated.”
The study, “Cerebral venous thrombosis as a rare complication of Sjögren’s syndrome: case series and literature review,” was published in the journal Clinical Rheumatology.
Brain blood clots seen in 12 primary Sjögren’s patients, 11 of them women
Sjögren’s disease is an autoimmune condition typically characterized by inflammation in the glands that produce saliva and tears, leading to its hallmark symptoms of dry mouth and dry eyes. The disease can be classified as primary, meaning it appears on its own, or secondary, when it arises in association with another condition.
CVT is an extremely rare neurological condition that’s estimated to affect fewer than 2 of every 100,000 people each year; it accounts for 1% or fewer of all strokes. Some studies have suggested the risk of CVT is elevated in people with autoimmune diseases. However, there is minimal research on the occurrence of the rare condition in people with Sjögren’s disease.
Here, scientists in China reported the outcomes of five Sjögren’s patients who experienced CVT and were treated at their center in Beijing. The team also combed through existing medical literature, identifying seven other reports of Sjögren’s patients with blood clots that formed in the brain.
“By combining seven cases in the previous literature with five cases in our hospital, a total of twelve patients were analyzed and summarized in this study, which is the largest of [primary Sjögren’s] combined with CVT reported at present,” the scientists wrote.
Among the 12 patients in the study, 11 were female; the one male was a patient at the researchers’ hospital, “which indicates male [primary Sjögren’s] patients could also have CVT,” the team wrote.
The ages of all 12 individuals ranged from 26 to 57, with an average age of 44. Given the ages and sexes of the identified patients, the researchers singled out “middle-aged women” as those who should be most aware of the potential for this complication.
It is of great importance to screen for autoimmune diseases during the clinical course of CVT. … For patients with [brain blood clots] of unknown cause, a detailed history and immunological examination are necessary, even if the [primary Sjögren’s] symptoms are not obvious.
Many of the affected patients started to experience neurological symptoms, such as headache, and were not aware they had Sjögren’s before being evaluated for CVT, the researchers noted. In fact, one-third of these individuals did not have the dry eye and dry mouth symptoms that normally characterize Sjögren’s, even though medical tests confirmed they had the disease.
According to the researchers, the fact that CVT alongside Sjögren’s often occurs in the absence of obvious Sjögren’s symptoms means that clinicians may not think to test for the autoimmune disease in patients with CVT of unexplained origin.
“It is of great importance to screen for autoimmune diseases during the clinical course of CVT,” the researchers wrote. “For patients with CVT of unknown cause, a detailed history and immunological examination are necessary, even if the [primary Sjögren’s] symptoms are not obvious.”
Most of the patients in the report were successfully treated with anti-clotting medications, or anticoagulants, sometimes given in combination with steroids or other immune-suppressing medications. The researchers noted, however, that there are no clear guidelines for how to treat CVT co-occurring with primary Sjögren’s.
From the small amount of data available, it’s also unclear whether having Sjögren’s increases the risk of CVT and, if so, why. As such, the team emphasized the need for more research into CVT as a possible complication of Sjögren’s.
About the Author
