Welcome back to Dry Eye Fireside Chat. In this episode, Damon Dierker, OD, FAAO, sits down with Jacob Lang, OD, FAAO, to discuss how optometrists can identify dry eye caused by Sjögren’s disease (SD), treat mixed-disease dry eye in SD patients, and co-manage these patients to optimize treatment outcomes.
What is Sjögren's disease?
Sjögren's disease is an autoimmune disorder in which lymphocytic infiltration of the exocrine (predominantly salivary and lacrimal) glands and B-cell hyperactivity manifest clinically as oral and ocular dryness.1 Besides sicca features, easy fatigability is one of the most common symptoms of SD and occurs in 70% of patients with primary SD.2
Patients with SD may report that their eyes feel sandy or itchy and tend to experience reduced tear secretion, chronic irritation, and chronic destruction of the corneal and bulbar conjunctival epithelia (keratoconjunctivitis sicca [KCS]).3
Differential diagnoses for SD-related dry eye can include conditions such as:4
- Blepharitis
- Dry eye syndrome
- Juvenile idiopathic arthritis uveitis
- Keratopathies (i.e., superficial punctate, filamentary, neurotrophic, and exposure)
Expanding ocular surface offerings at your optometry practice
Dr. Lang noted that his clinic has a relatively large Sjögren's disease patient population, which has grown as a result of expanded ocular surface offerings and increased promotion of these services.
In addition, his network outside of the clinic has grown as he has developed relationships with patients’ primary care providers (PCPs), rheumatologists, and internal medicine doctors. This illustrates how heightened awareness of dry eye and collaborative care have allowed for an ever-expanding array of treatment options for dry eye patients, noted Dr. Dierker.
What to look for in a patient history and exam to indicate Sjögren's disease
During an appointment the patient examination starts the second he walks in the door, explained Dr. Lang. Two traits, in Dr. Lang’s experience, that he tends to observe right off the bat in SD patients are that they have a water bottle within grasp and the “Sjögren’s smack” when discussing their complaints. The “smack” refers to an audible smacking sound that SD patients tend to make when speaking, in order to move the saliva around their mouths.
Noting these traits at first glance prompts Dr. Lang to expand the patient conversation to include discussions of whether the patient is seeing other doctors, a list of current medications, and other health conditions. He also tends to ask patients he suspects of having SD if they notice that they have dry mouth and dry eyes and if they struggle with cavities, tooth loss, or oral infections.
Dr. Lang explained that he also tends to see more conjunctival staining with lissamine green dye in SD patients compared to immunocompetent ocular surface disease (OSD) patients. Seeing the conjunctiva light up significantly with staining suggests that there may be a systemic or autoimmune cause of the dry eye. Additionally, conjunctival staining scores make up part of the grading scale in the American College of Rheumatology’s classification criteria for Sjögren's disease.5
Dr. Dierker observed that the majority of Sjögren's disease patients at his clinic have mixed-mechanism dry eye, i.e., not only aqueous deficient dry eye but also evaporative dry eye due to meibomian gland dysfunction (MGD). Dr. Lang agreed, noting that in his clinical practice, he tends to see SD patients develop aqueous deficient dry eye first, but over time this may progress to include evaporative dry eye as the meibomian glands are impacted as well.
Efficient and effective management of SD patients
Patients with rheumatological or systemic conditions need a number of providers to manage the various disease aspects. So when a patient comes in that Dr. Lang suspects may have SD, in an optimal situation, he would contact the patient’s PCP to recommend lab tests or, if necessary, order the labs himself.
He added that the situation is usually not emergent, so eyecare practitioners can recommend that patients request the lab tests at their next annual physical if it is soon. However, he has also written the orders and CC’d the PCP so they are aware. He then follows up with a phone call to both the patient and PCP after the labs are completed in order to convey the results.
If there is significant conjunctival staining or if the patient has other notable rheumatologic complaints, he may opt to loop in a rheumatologist. One rheumatologist that Dr. Lang spoke with indicated that he prefers only seropositive patients with measurable blood dysfunction to be referred.
However, this is not necessarily the perspective of every rheumatologist, and much like with glaucoma, there are likely many SD suspects that would benefit from monitoring, emphasized Dr. Lang.
Managing dry eye caused by Sjögren's disease
Due to the fact that many SD patients have mixed-mechanism dry eye, including aqueous deficiency, meibomian gland issues, and keratopathy, Dr. Lang prefers to employ a broad arsenal of treatments. He starts by aggressively treating the inflammation with topical steroids to calm things down.
Subsequently, he prescribes a long-term topical anti-inflammatory, and if that does not provide sufficient results, he may turn to punctal plugs to trap as many tears on the ocular surface as possible. If indicated, he then treats the meibomian glands with intense pulsed light (IPL) and thermal treatments and locks this in with drops that target evaporative dry eye.
Dr. Dierker noted a spectrum of options for SD patients. He often recommends amniotic membranes if indicated and tear stimulators such as Tyrvaya (varenicline solution nasal spray 0.03mg, Viatris). He added that many SD patients also tend to benefit from cyclosporine and autologous serum or platelet-rich plasma (PRP) drops.
What’s in the pipeline for SD treatments?
Dr. Lang highlighted that there are ongoing clinical trials with biological therapies for Sjögren's disease that, while being systemic treatments, would likely have implications for ocular health as well.6
He added that this is where he sees the next frontier of eyecare, particularly for inflammation- and autoimmune-related conditions, in which biologics have expanded in other areas of healthcare.
Conclusion
When examining patients suspected of having SD, features to keep in mind are patients who need to drink frequently (for dry mouth), the telltale smacking sound when they speak, and significant conjunctival staining with lissamine green.
Sjögren's disease is a key example of the importance of collaborative medical care, as eyecare practitioners can detect issues that indicate systemic infections or diseases that would have likely otherwise gone undiagnosed and unaddressed.
