Diagnostic Challenges & Monitoring
The correct diagnosis of Sjögren’s syndrome is a challenge for many clinicians. Many patients experience delay before receiving a correct diagnosis. Dr. Rasmussen points out that one challenge is the lack of a single gold-standard diagnostic test.
Disease classification is performed via a composite score from a combination of multiple tests, per the ACR/EULAR guideline.1-3 Some of these tests, such as salivary gland biopsy, are invasive. “Many of these tests are not readily available to most rheumatologists,” says Dr. Rasmussen, “for example, the type of eye exam to look at the damage to the ocular surface. If you’re not in a tertiary care center, it’s going to be very difficult for you to get someone specifically trained who can do that for you.”
Native Americans showed higher levels of disease activity, as assessed by the ESSDAI (EULAR Sjögren’s Syndrome Disease Activity Index), than whites.
Diagnosis may be even more challenging in patients who present with more extraglandular symptoms and fewer classic sicca symptoms. Dr. Rasmussen urges clinicians to keep Sjögren’s syndrome on the differential, and to revisit it as a possibility in rheumatology patients not responding to treatment for other diagnoses.
“In the case of the Native Americans, they are not so dry, and they have joint pain and arthritis, so they may get misdiagnosed [with] rheumatoid arthritis,” she says. “And we know that drugs used for rheumatoid arthritis often make Sjögren’s syndrome worse—or at least they don’t help. So it’s important to suspect the diagnosis and tailor the treatment.”
In general, Dr Rasmussen believes some delays may be attributable to lack of awareness on the part of some healthcare providers, especially non-rheumatologists. “The community is a lot more aware [of] rheumatoid arthritis and lupus, and those end up being the default diagnoses. We really need to work hard as researchers and clinicians in the Sjögren’s syndrome community to raise awareness about this option.”
It’s also important that clinicians consider the possibility of Sjögren’s syndrome in African American patients, even though it appears to be less common in this group. Once diagnosed, Dr. Rasmussen suggests more frequent visits for these patients, perhaps once every six months, to more carefully monitor for lymphoma. She also suggests keeping an extra close eye on their bloodwork (e.g., for increased hypergammaglobulinemia).
Carefully monitoring the parotid gland size is also critical, because it is an important origin site for lymphomas associated with Sjögren’s syndrome. “If [a patient] had an enlarged gland, I would definitely be monitoring it with ultrasound and a biopsy or maybe some additional imaging studies,” says Dr. Rasmussen.
She adds that it is also important to check for enlarged lymph nodes and educate patients to come to the office if they notice any. “That can be part of Sjögren’s syndrome without necessarily meaning lymphoma, but you would want to confirm that.”
