By being judicious about identifying secondary Sjögren's syndrome, you can grow your practice.
Barbara Caffery, O.D., Ph.D., Toronto, CAN.
Optometry could and should be Rheumatology's best friend. After all, we have the ability to be the first to identify autoimmune rheumatic diseases, such as Sjögren's syndrome, in our eye exam, enabling us to refer these patients to rheumatologists for further diagnostic testing and systemic management of their condition. Once this co-management of patients begins, rheumatologists will reciprocate by referring their patients to us for both primary and secondary Sjögren's syndrome (SS) management. Also, once the rheumatologist sees you can treat SS, he'll be more likely to refer to you for other ocular problems.
Considering an estimated 1.5 million U.S. adults have rheumatoid arthritis (RA), and a recent CDC survey suggests that SS is as common as RA and much more common than systemic lupus erythematosus (SLE), scleroderma and ankylosing spondylitis, it's definitely worth forging such a relationship.1
While I have no doubt some O.D.s and rheumatologists have and continue to work together, I also know that other O.D.s are losing out on this practice-building opportunity because many secondary SS patients remain un-diagnosed for several years. The reasons: SS symptoms may mimic those of menopause, drug side effects, allergies or the aforementioned medical conditions themselves along with fibromyalgia, chronic fatigue syndrome and multiple sclerosis.2 Also, autoimmune diseases have exacerbations and remissions so that all symptoms of the condition are not always present at the same time. In addition, SS can involve many body systems (e.g. the brain, lungs, skin, stomach, liver and extremities) leading doctors to sometimes treat each symptom individually rather than recognize they comprise SS. Finally, diagnosing SS is challenging because those who have it often try to cope with its symptoms on their own. As an example, someone who has dry mouth, may simply drink more water to conquer the problem.3 With the recent media attention surrounding Venus Williams' diagnosis of SS, however, it is hoped that those who have been trying to cope on their own will now seek their optometrist or primary care physician to determine whether they too, have SS.
Here, I provide an overview of SS, how to arrive at a definitive diagnosis and how to treat it, so you can take advantage of the benefits of seeing these patients.
The criterion for objective dry eye in Sjögren's syndrome is Schirmer 1 scores ≤5mm in at least one eye or staining scores of ≥4/9 in at least one eye.
SS is a chronic systemic rheumatologic autoimmune disorder characterized by lymphocytic infiltration and malfunction of the exocrine glands. It can effect the brain (concentration/memoryloss); the nose (nasal dryness, recurrent sinusitis, nose bleeds); the mouth (dry mouth, dental decay, chewing, speech and taste); the digestive tract (difficulty swallowing, heart burn, reflux esophagitis); the lungs (recurrent bronchitis, pneumonia, interstitial lung disease); the liver (abnormal liver function tests, chronic active autoimmune hepatitis, primary biliary cirrhosis); the stomach (gastroparesis, autoimmune pancreatitis); and cause peripheral neuropathy (numbness and tingling in the extremities.)4 It is named for Henrik Sjögren, a Swedish ophthal mologist who first described the condition. (Dr. Sjögren died in 1986 at age 87.)
Nine out of ten SS patients are women, although the condition can occur in both men and in children.2 There is some controversy as to the age of SS onset, as the disease can exist for a long time in subclinical form, making the disease duration of most patients uncertain.5,6 Some report two age peaks in the diagnosis of this disease: 20 to 30 years after menopause and the mid 50 years.7 Others state the age of onset as approximately 45 years.8-10
The symptoms of SS vary but may include: a dry, gritty or burning sensation in the eyes; dry mouth; difficulty talking, chewing or swallowing; a sore or cracked tongue; a dry or burning throat; dry or peeling lips; a change in taste or smell; increased dental decay; joint pain; vaginal and skin dryness; digestive problems; dry nose and fatigue.4 In addition, these patients are also 16 times more likely to develop lymphoma, and they are at high risk for fatigue and depression.11-16 (These whole-body disease characteristics alone emphasize the need for early diagnosis and management. Remember: A grateful patient is your best marketing vehicle.)
Adding fluorescein and grading the amount of corneal staining from 0 to 3 will help you diagnose Sjögren's syndrome.
The syndrome can present as secondary SS, which by definition is associated with rheumatoid arthritis, SLE or scleroderma, or as primary SS, which presents absent of an underlying rheumatological disease.
The Toronto Sjögren's Syndrome Clinic is now investigating the prevalence of secondary SS in other autoimmune diseases, such as CREST (calcinosis, Raynaud's phenomenon, esophageal dys-function, sclerodactyly and telangiectasia) syndrome.
I suspect that in the future, more autoimmune diseases will appear on the list of those associated with SS as well. It is my suspicion that the involvement of dry eye in these other autoimmune diseases has been ignored in the past, as dry eye is considered a very minor aspect of systemic autoimmune disease. Patients with dry eye will be quick to tell you otherwise.
Making the diagnosis
To determine whether the patient has SS:
► Inquire about the presence of systemic disease. On the patient's history form, include check boxes next to rheumatoid arthritis, systemic lupus erythematosus and scleroderma for the aforementioned reasons.
► Inquire about dry eye symptoms. On the patient's history form, ask whether he/she is experiencing ocular dryness. This question is crucial because as mentioned above, many SS patients are stoic individuals. In fact, a recent survey of SS patients revealed a lack of patient-physician dialogue about symptoms, such as dry mouth, contributed to a delayed diagnosis.3
► Ask about dry mouth. Include this question on your patient history form: “Can you eat five soda crackers without drinking water?” If the patient writes “no,” you have a SS suspect.
If the patient has a diagnosis of RA, SLE or scleroderma or if either dry mouth or dry eye symptoms are reported, move on to the following tests:
► Measure the ocular dryness level. Use Schirmer 1 testing. The criterion for objective dry eye in SS is: Schirmer 1 scores ≤5mm in at least one eye or staining scores of ≥4/9 in at least one eye.
► Grade corneal staining. Add fluorescein, and grade the amount of corneal staining from 0 to 3.
► Grade the temporal and nasal sections of the bulbar conjunctiva. Employ lissamine green stain, and grade from 0 to 3. Please be aware that the presence of lissamine green staining of the temporal bulbar conjunctiva is the most important variable (of more than 90 variables measured at our SS clinic) in suspicious SS and in differentiating it from other forms of aqueous deficient dry eye.17
Now, total your scores out of nine.
If there is no underlying systemic diagnosis but symptoms of dry eye and dry mouth are present, as are the objective signs of dry eye, you are well on your way toward a diagnosis of Primary SS (PSS). Although the complete diagnosis is not in your hands, you are often the first healthcare provider to entertain the possibility. (In this case, you can garner reciprocal referrals from primary care physicians.) In PSS, testing is required by several disciplines to determine each of the following six criteria:
1. Symptoms of dry eye for at least three months.
2. Symptoms of dry mouth for at least three months.
3. Signs of dry eye: Schirmer 1 scores of ≤ 5mm in five minutes and/or Rose Bengal or fluorescein staining scores of ≥ 4/9 in at least one eye. This scoring is a sum of the cornea, nasal and temporal conjunctiva graded 0 to 3.
4. Signs of dry mouth: salivary flow by unstimulated spitting in a cup of ≤ 1.5ml in 15 minutes.
5. Positive blood serum findings of autoantibodies to Ro and/or La.
6. Positive salivary gland biopsy score: ≥ 1 focus score in 4mm of tissue.
A minimum of four of these six criteria must be met, including one of serum-positive or biopsy-positive results to garner a PSS diagnosis, according to the gold standard American European-Consensus Criterion (AECC) for SS.
A referral letter to a family practice with specific instructions to test the serum for ANA, RA, anti-ro and anti-la moves the PSS diagnosis forward. If anti-ro or anti-la is present, the diagnosis is confirmed. If not, a minor salivary gland biopsy is required.
The current standard diagnostic criterion for secondary SS is also a part of the AECC.18 In the presence of an associated autoimmune disease (RA, SLE, etc.), the presence of ocular symptoms or oral symptoms combined with any two of the objective signs of dry eye, objective signs of dry mouth and histopathology define secondary SS. Thus, the diagnosis of secondary SS is completely in your hands.
Be aware that the presence of lissamine green staining of the temporal bulbar conjunctiva is the most important variable (of more than 90 variables measured at our Sjögren's syndrome clinic) in suspicious Sjögren's syndrome and in differentiating it from other forms of aqueous deficient dry eye.
Currently, SS has no cure. That said, you can offer your patients symptomatic relief in the form of lubrication, lid scrubs and hot soaks, punctal occlusion, anti-inflammatory topical therapy, such as cyclosporine ophthalmic emulsion 0.05% (Restasis, Allergan), and/or pulsed steroids and scleral contact lenses (the fluid reservoir acts as a liquid bandage and provides dryness relief). Always reassure these patients that their dry eye is manageable but not curable and that they will not lose their vision. The latter is an important bit of information for those who are really frightened because of their chronic symptoms.
Don't underestimate the personal and practice-building rewards of diagnosing and managing SS. You'll build a network of referring rheumatologists and primary care physicians, who will likely also refer their patients to you for other ocular issues, and you'll foster patient loyalty and referrals, as you've given a name to their symptoms, reassurance they're not “going crazy” and therapeutic interventions that can provide relief. OM
1. Helmick C Felson D, Lawrence R, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Arthritis Rheum. 2008;Jan;58(1):15-25.
2. Sjögren's Syndrome Foundation: Sjögren's FAQS. www.sjogrens.org/home/about-sjogrens-syndrome/sjoe grens-faqs. (Accessed October 19, 2011.)
3. Sjögren's Syndrome Foundation: A New Resource for Sjögren's Patients - LivingWithDryness.com. www.sjogrens.org/component/content/article/25-news/222-a-new-resource-for-sjoegrens-patients-livingwithdrynesscom. (Accessed October 19, 2012.)
4. Sjögren's Syndrome Foundation: Symptoms. /www.sjogrens.org/home/about-sjogrens-syndrome/symptoms (Accessed October 19, 2011.)
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9. Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ. Sjoegren's syndrome. A clinical, pathological and serological study of sixty-two cases. Medicine (Baltimore). 1965 May;44:187-231.
10. Bjerrum K, Prause JU. Primary Sjögren's syndrome: a subjective description of the disease. Clin Exp Rheumatol. 1990; May-Jun;8(3):283-8.
11. Theander E, Henriksson G, Ljungberg O, et al. Lymphoma and other malignancies in primary Sjögren's syndrome. A cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006 Jun;65(6):796-803.
12. Barendregt PJ, Visser MR, Smets EM, et al. Fatigue in primary Sjögren's syndrome. Ann Rheum Dis. 1998 May; 57(5):291-295.
13. Bowman SJ, Booth DA, Platts RG; UK Sjögren's Interest Group. Measurement of fatigue and discomfort in primary Sjögren's syndrome using a new questionnaire tool. Rheumatology (Oxford) 2004 Jun;43(6):758-64.
14. Godaert GL, Hartkamp A, Greenen R, et al. Fatigue in daily life in patients with primary Sjögren's syndrome and systemic lupus erythematosus. Ann NY Acad Sci. 2002 Jun;966:320-6.
15. Stevenson H, Jones M, Rostron J, Longman L, Field E. UK patients with primary Sjögren's syndrome are at increased risk from clinical depression. Gerodontology. 2004 Sep;21(3):141-5.
16. Valtýsdóttir ST, Gudbjörnsson B, Lindqvist U, et al. Anxiety and depression in patients with primary Sjögren's syndrome. J Rheumatol 2000 Jan;27(1): 165-9.
17. Caffery B, Simpson T, Wang S, et al. Rose bengal staining of the temporal conjunctiva differentiates Sjögren's syndrome from keratoconjunctivitis sicca. Invest Ophthalmol Vis Sci 2010 May;51(5):2381-7.
18. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos H, Alexander E, Carsons S, et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8.
||Dr. Caffery works in a multidisciplinary downtown practice in Toronto Canada, where she does general care with an emphasis on dry eye and contact lenses. She is also a member of the Sjögren's Syndrome Clinic of the University Health Network, where rheumatology, dentistry, ophthalmology/optometry, ENT and pathology work together in the diagnosis and management of Sjogren's syndrome. E-mail her at firstname.lastname@example.org, or send comments to email@example.com.
Optometric Management, Issue: November 2011