Sjögren's syndrome and dry eye: managing it well

Sjögren's syndrome is an autoimmune condition in which the immune system attacks the glands that make tears and saliva, so dry eyes and a dry mouth are its two defining symptoms. If you have been told you might have it, or you already have the diagnosis, the practical question is usually the same: does it change what you should be doing about your eyes?

The short answer is that the treatments are largely those used for any dry eye, but started earlier, used more consistently, and escalated sooner. What genuinely changes is the team. Sjögren's is a systemic disease, and it needs a rheumatologist alongside whoever looks after your eyes.

The other thing worth knowing early is that Sjögren's dry eye is not purely a tear-volume problem. It is primarily aqueous-deficient, but hybrid disease with an evaporative component on top is common, as we set out below, and the gland side responds to different treatment from the tear-volume side.

How Sjögren's is classified

The current reference point is the 2016 classification criteria developed jointly by the American College of Rheumatology and the European League Against Rheumatism, published by Shiboski and colleagues. They score five items in people who already have signs or symptoms suggestive of the condition. Anti-SSA/Ro antibody positivity scores three, and focal lymphocytic sialadenitis on a minor salivary gland biopsy with a focus score of at least one focus per four square millimetres also scores three. An abnormal ocular staining score of five or more, a Schirmer's test result of five millimetres or less in five minutes, and an unstimulated salivary flow rate of 0.1 millilitres per minute or less each score one. A total of four or more meets the criteria for primary Sjögren's syndrome. In the final validation cohort, sensitivity against expert clinical judgement was 96% and specificity was 95%.

Two caveats matter for a patient reading that list. These are classification criteria, developed to define consistent groups for research, and the authors describe them as well suited to enrolment in clinical trials. Your rheumatologist diagnoses you; the criteria do not. And two of the five items are eye tests, which is why your optometrist's findings can end up as part of a rheumatology work-up.

Why dry eye clinics screen for it

Because it turns up more often than people expect, and often undiagnosed. Akpek and colleagues reviewed the records of 220 patients seen with a primary diagnosis of dry eye at a single dry eye centre over two years. Fifty-seven of them, 25.9%, had an underlying rheumatic condition: 25 patients, 11.4%, had rheumatoid arthritis, and 24 patients, 10.9%, had primary Sjögren's syndrome.

The revealing part is who already knew. Of the patients with rheumatoid arthritis, 96% carried the diagnosis when they arrived. Of the 24 with primary Sjögren's syndrome, only a third, 8 of 24, did. Half of them, 12 of 24, were diagnosed as a result of that initial dry eye evaluation. Among those twelve, only two thirds tested positive for anti-Ro or anti-La antibodies; a third tested positive only for antinuclear antibody at a titre below 1 in 320 and needed a minor salivary gland biopsy for a definitive answer. A further four patients who were initially seronegative eventually had a biopsy and were diagnosed that way. The authors concluded that primary Sjögren's syndrome appears underdiagnosed in dry eye patients and should be a focus of diagnostic evaluation.

Read that with its limitations in view. It is a retrospective record review at a specialist referral centre, so the proportion in a general optometric practice will be lower. The practical lesson still holds. A negative blood test does not close the question, and dry eye that is severe, symmetrical, or accompanied by a persistently dry mouth deserves a proper look.

If your mouth is dry as well as your eyes, say so at your eye appointment. It is the single most useful piece of history you can offer, and it is the one patients most often leave out because it does not seem like an eye problem.

Aqueous-deficient, but rarely only that

Sjögren's damages the lacrimal glands, so the classic picture is low tear volume: a poor Schirmer result, surface staining, and eyes that feel raw rather than merely tired. That is the aqueous-deficient end of the spectrum, explained further in our piece on evaporative versus aqueous-deficient dry eye.

But the DEWS II definition and classification report is explicit that aqueous-deficient and evaporative dry eye exist as a continuum, such that elements of each are considered in diagnosis and management. The DEWS II pathophysiology report goes further, noting that hybrid dry eye disease with features of both aqueous deficiency and increased evaporation is common, and that efforts should be made to determine the relative contribution of each form to the total picture.

In clinic that plays out predictably. Someone with Sjögren's who is also blinking incompletely at a screen, or who has blocked meibomian glands, is losing tears from both directions at once. Working out the mix is worth doing properly, because it decides which treatments are worth your time. Our guide to working out which type of dry eye you have covers what that assessment involves.

The management ladder

The DEWS II management and therapy report reviewed the available treatments and derived a staged algorithm: a step-wise approach applied according to disease severity. It is also candid about the evidence, stating that many of the treatments available lack the Level 1 evidence needed to support their recommendation, often because of inadequate masking, randomisation or controls. Its authors found that differentiating between aqueous-deficient and evaporative disease was critical to selecting the right strategy, while acknowledging real difficulty in predicting the relative benefit of specific options.

In practice the ladder looks roughly like this. Preservative-free lubricants used often enough to matter, which for moderate Sjögren's usually means more frequently than people expect, with a gel or ointment overnight. Attention to the environment: humidity, air conditioning, screen habits and blink quality. Lid hygiene and warmth if the glands are involved. Then the prescription tier, where anti-inflammatory treatments such as ciclosporin, lifitegrast or a short course of corticosteroid drops may be considered; these are prescribed and monitored by an ophthalmologist or an optometrist with prescribing rights, and we describe how they work in our article on prescription dry eye drops. Conserving the tears you have, through punctal occlusion, is a standard option in aqueous-deficient disease specifically, though the evidence base for it is thinner than its popularity suggests. At the severe end sit options such as autologous serum drops and scleral lenses, arranged through a specialist service.

The habit that helps most is boring. A routine you actually perform every day outperforms an ambitious one abandoned after a fortnight, which is how our daily dry eye routine is built.

Why the rheumatologist matters

Your eyes are one organ affected by a whole-body condition. Fatigue, joint pain, dry mouth and its dental consequences, and the monitoring that goes with an autoimmune diagnosis all sit outside an eye clinic's remit. Systemic treatment decisions belong with the rheumatologist, and the dental consequences of a persistently dry mouth belong with a dentist who knows the diagnosis.

Push for the two to talk to each other. An optometrist who can send an ocular staining score and a Schirmer result to a rheumatologist is contributing two of the five classification items, and a rheumatologist who tells your optometrist what systemic treatment you are on saves everyone guessing.

Honest expectations

No treatment switches Sjögren's off, and anyone promising your dry eye will disappear is overselling. What is realistic is meaningful, sustained control of symptoms and protection of the ocular surface, achieved by stacking several modest measures rather than finding one decisive one. Expect fluctuation, and judge progress over months rather than days.

If the assessment finds that blocked meibomian glands are part of your picture, warming the lids is a first-line, guideline-recommended step for that component, and the Meibocare E-Heated Eye Mask is one way of doing it. Designed in New Zealand and manufactured in China, it is built to bring the eyelids to about 42 °C on the recommended setting and timer. Be clear about scope: heat addresses the meibomian gland side of the problem and does nothing for the tear volume that Sjögren's has reduced, so it is an adjunct here, not the answer. Where there is an eye infection, recent eye surgery, glaucoma or any change in vision, that is a conversation to have with your optometrist or ophthalmologist before heat therapy starts and while it continues.

Frequently asked questions

How is Sjögren's syndrome diagnosed?

The 2016 ACR/EULAR classification criteria score five items in people with suggestive signs or symptoms: anti-SSA/Ro antibodies and a positive minor salivary gland biopsy score three each, while an ocular staining score of five or more, a Schirmer result of 5 mm or less in five minutes, and unstimulated salivary flow of 0.1 ml per minute or less score one each. Four or more meets the criteria.

How often does dry eye turn out to be Sjögren's?

In a review of 220 patients at one dry eye centre, 24 of them, or 10.9%, had primary Sjögren's syndrome, and only a third of those already knew. Half were diagnosed through that initial dry eye evaluation. That was a specialist referral centre, so the rate in general practice is lower, but it explains why clinics ask about dry mouth and joint symptoms.

Is Sjögren's dry eye the same as ordinary dry eye?

It is primarily aqueous-deficient, meaning the lacrimal glands make too little tear fluid, whereas most everyday dry eye is evaporative. In practice many people have both, and the DEWS II reports describe the two forms as a continuum, with hybrid disease being common. Working out the relative contribution of each decides which treatments are worth using.

Can a warm compress help Sjögren's dry eye?

Only for the meibomian gland component, if you have one. Warming the lids is a first-line step for gland dysfunction and evaporative dry eye, but it does nothing about the reduced tear volume that Sjögren's causes. Treat it as one part of a wider plan alongside preservative-free lubricants and whatever your prescriber advises.

Educational information only, not medical advice. References: Shiboski CH, et al. Arthritis Rheumatol. 2017;69:35–45; Akpek EK, et al. Cornea. 2009;28:493–497; Craig JP, et al. Ocul Surf. 2017;15:276–283; Bron AJ, et al. Ocul Surf. 2017;15:438–510; Jones L, et al. Ocul Surf. 2017;15:575–628.