Facilitating Timely Diagnosis of Sjögren's Syndrome

ARTICLE

CPD: ONE HOUR Facilitating timely diagnosis of

Sjögren’s syndrome CPD questions This article has four CPD

7 questions attached to it Research at the University of Toronto found a neuropathy, which will earn you one hour median delay of four years between onset and hypothyroidism, of verifiable CPD. To access By Linda 5 diagnosis (range 0-28 years). Prompt diagnosis and lymphoma. the free BDA CPD hub, Douglas1 allows access to treatment to relieve symptoms, go to http://bit. minimise complications, and enhance vigilance Sjögren’s ly/2e3G0sv for lymphoma: Sjögren’s patients have a 16 syndrome and times greater risk of developing lymphoma than mental health a healthy individual.6 In common with many chronic medical Introduction Much of the delay between onset and diseases, depression or anxiety might Sjögren’s syndrome is an autoimmune disease diagnosis is related to the diverse symptoms, accompany Sjögren’s syndrome, related to in which intense lymphocytic infiltration of which make diagnosis difficult, plus a lack its adverse effect on the quality of life. This the exocrine glands1 and hyperactivity of B of awareness of the condition on the part of is shown by its score on the Devin’s Illness lymphocytes2 cause inflammation, which various healthcare professionals encountered by Intrusiveness Scale,8 where the negative damages glandular tissue and impairs function. many Sjögren’s patients, who frequently fail to impact on quality of life is comparable with Primary Sjögren’s syndrome is diagnosed in ‘connect the dots’ regarding the numerous signs that of multiple sclerosis, or kidney dialysis.9 the absence of any other connective tissue and symptoms affecting multiple body systems. In addition, mounting evidence indicates that disease, while secondary Sjögren’s syndrome chronic exposure to elevated inflammatory is accompanied by other autoimmune Clinical features of Sjögren’s cytokines causes persistent alterations in diseases, such as rheumatoid arthritis, lupus syndrome neurotransmitter systems, which can lead to erythematosus, Graft Versus Host Disease, or in Sjögren’s syndrome is mainly characterised neuropsychiatric disorders and depression.10 rare cases, Behçet’s disease.3 Secondary Sjögren’s syndrome comprises approximately 60% of ‘ORAL-RELATED COMPLICATIONS INCLUDE cases. In January 2012, the Sjögren’s Syndrome Foundation launched a 5-year Breakthrough SIALOLITHS, SALIVARY GLAND SWELLING, HIGH Goal, to reduce the time between onset and diagnosis of Sjögren’s by 50% in five years. CARIES RISK, PERIODONTAL DISEASE, SOFT When this Goal was launched, research showed the average diagnosis time was nearly six years.4 TISSUE INFECTIONS AND ANGULAR CHEILITIS’

1 RDH, BSc. Linda M. Douglas graduated as a dental hygienist from the Royal by dry eyes and a dry mouth, and may also Prevalence and incidence of Dental Hospital in London, England in affect other mucosal tissues such as the nose, Sjögren’s syndrome 1982. After graduation she worked in larynx, gastro-intestinal tract and vagina. Dry An estimated 221,583 Britons are affected,11 and periodontology, before moving to Toronto, eyes can lead to blurred vision. Oral-related approximately 4 million Americans. Ninety Canada, where she has worked in complications include sialoliths (salivary percent of Sjögren’s patients are women.12 Most private practice since 1990. Linda also stones), salivary gland swelling, high caries risk, are diagnosed in their late 40s, but it can also has a BSc in Oral Health Promotion. particularly at the cusp tips and cervical sites, affect children and males. Males are usually Linda’s desire to support dry mouth periodontal disease, and soft tissue infections diagnosed later than females. patients has instigated an in-depth such as candidiasis, and angular cheilitis. study of xerostomia, and the related Dysphagia might also occur, which can lead to Diagnosis of Sjögren’s syndrome pathologies. She is also Clinical Director aspiration pneumonia. Accurate diagnosis of Sjögren’s syndrome for Hygienetown, an online community The systemic autoimmunity associated with begins with thorough assessment to expedite for dental hygienists, and teaches Sjögren’s syndrome can also result in dry skin, specialist referral. This includes general continuing education courses on fatigue, low grade fever, constipation, myalgia, appraisal, medical history review, and screening xerostomia management, saliva testing, and joint pain. Other conditions which might for symptoms using questionnaires, followed by and caring for dental patients with occur include small vessel vasculitis, Raynaud’s head and neck examination, and comprehensive eating disorders. phenomenon, pulmonary symptoms, nephritis, intra-oral examination.

ARTICLE

Questionnaires to assess symptoms Table 1 Classification criteria for Sjögren’s syndrome Oral symptoms A positive response to one or more of the Diagnostic Criteria Item Scoring following questions is suggestive of Sjögren’s syndrome: Anti-SSA/Ro antibody positivity 3 points 1. Have you had a daily feeling of dry mouth for more than three months? Focal lymphocytic sialadenitis with a focus score of 1 foci/4 mm² or greater 3 points 2. Have you had recurrently or persistently An abnormal ocular staining score of 5 or greater (or van Bijsterveld score of swollen salivary glands as an adult? 1 point 3. Do you frequently drink liquids to aid in 4 or more) swallowing dry food? A Schirmer’s test result of 5 mm/5 minutes or less 1 point Ocular symptoms An unstimulated salivary flow rate of 0.1 ml/minute, or less 1 point A positive response to at least one of the following questions is suggestive of Sjögren’s syndrome: 1. Have you had daily, persistent troublesome established for use in individuals with signs and/ Management of the dry mouth needs dry eyes for more than three months? or symptoms suggestive of SS. to be multifaceted, to address the multiple 2. Do you have a recurrent sensation of sand or These single set classification criteria oral symptoms and prevent complications. gravel in the eyes? performed well in validation analyses, This includes stimulation of salivary flow, 3. Do you use tear substitutes more than three demonstrating 96% sensitivity and 95% conservation of functional salivary gland times a day? specificity, and are also suitable criteria tissue, and saliva substitutes as required to protect and lubricate, and facilitate speech, mastication and swallowing. Supplementation ‘SWELLING OF THE PAROTID AND with Omega 3 1,000 mg has been shown to improve the lubricating quality of the saliva, 15 SUBMANDIBULAR SALIVARY GLANDS CAN BE and the tears. Topical fluoride applications, and calcium and phosphate remineralisation preparations REDUCED BY APPLYING HEAT AND MASSAGE reduce caries risk.16 Swelling of the parotid and submandibular salivary glands (Fig. 2) can be TO AID SALIVARY FLOW THROUGH THE DUCTS.’ reduced by application of heat and massage to aid salivary flow through the ducts.

Conclusion Diagnostic techniques include blood testing for enrollment in clinical trials. The final We are getting closer to our goal of early for anti-SSA/Ro antibody, biopsy to assess the classification criteria are based on the weighted diagnosis. In 2016, the Sjögren’s Syndrome minor salivary glands inside the lower lip for sum of five items.13 Foundation annual survey of newly diagnosed focal lymphocytic sialadenitis, ocular staining Each objective criterion is allocated points: patients revealed that for those diagnosed with Rose Bengal, or the van Bijsterveld score individuals with signs and/or symptoms in 2015, it took an average of three years to to assess surface damage of the conjunctiva and suggestive of Sjögren’s syndrome and a total receive an accurate diagnosis,17 compared with cornea, Schirmer’s test for lachrymal flow, and score of at least 4 points meet the criteria for almost six years in 2012. This was achieved by measurement of unstimulated salivary flow. diagnosis of primary SS (Table 1). increasing public awareness, and increasing Other conditions which cause dry eyes and education and awareness among healthcare dry mouth should be considered in differential Management of the Sjögren’s professionals. diagnosis, and excluded. For example: pre- patient in the dental practice setting Increased awareness of Sjögren’s syndrome in existing lymphoma, hepatitis C infection, HIV Extra-oral and intra-oral examination needs the medical and dental communities facilitates infection, sarcoidosis, use of anti-cholinergic to be performed on a regular basis, to assess improved outcomes for the Sjögren’s patient, drugs, or a history of head and neck radiation for complications of Sjögren’s syndrome. The by enhancing inter-professional collaboration therapy. Challacombe Scale of Clinical Oral Dryness14 and vigilance. For example: screening (Fig. 1) is a valuable tool for clinically assessing questionnaires utilised by both medical and Current diagnostic criteria and quantifying the severity of oral dryness, dental professionals could include questions on The diagnostic criteria for Sjögren’s syndrome is and for evaluating the outcomes of our ocular, oral and systemic symptoms of Sjögren’s evolving, and has been revised numerous times interventions. This scale is based on a Clinical syndrome. since 1965. Oral Dryness Score (CODS), and lists ten key Raising public awareness with more widely In 2016, the American College of features of dry mouth; one point is allocated disseminated information on Sjögren’s Rheumatology (ACR) and the European League for each feature, and the patient’s additive score syndrome, and increased availability of Against Rheumatism (EULAR) developed indicates the severity of the dry mouth. There screening questionnaires for self-assessment and validated an international consensus is an inverse relationship between salivary flow could be valuable. Knowledge also empowers of data-driven classification criteria for rates, and CODS: a high CODS is related to patients to advocate for themselves as they primary Sjögren’s syndrome (SS), which were hyposalivation. navigate the healthcare system. www.nature.com/BDJTeam BDJ Team 18 ©2018 British Dental Associati on. All ri ghts reserved.

ARTICLE

Fig. 1 The Challacombe Scale of Clinical Oral Dryness, courtesy of Professor Stephen Challacombe

ARTICLE

1. Sjögren’s Society of Canada. What is Sjögren’s? Available at: http:// sjogrenscanada.org/ (accessed January 2018). 2. Pers J-O, Youinou P. B Lymphocytes in Primary Sjögren’s syndrome. 2016; 217- 228. 10.1016/B978-0-12-803604-4.00014-9. 3. Erten S, Erkol E, Perçinel S, Ölmez U, Ensari A, Düzgün N. A case with Sjogren’s syndrome and Behçet’s disease developing secondary amyloidosis. Turkiye Klinikleri J Med Sci 2012; 32: 222-225. 4. Sjögren’s Syndrome Foundation. SSF launches 5-year breakthrough goal. (Article from The Moisture Seekers January 2012). Available at: http://www.sjogrens.org/ home/about-the-foundation/breakthrough- goal- (accessed January 2018). 5. Hauck T S, Douglas S C, Bookman A A M. Sjogren’s syndrome in Canada: diagnosis, treatment and patient perspectives. Connections 2013; 7 (1). 6. Solans-Laqué R, López-Hernandez A, Bosch-Gil J A, Palacios A, Campillo M, Vilardell-Tarres M. Risk, predictors, and clinical characteristics of lymphoma development in primary Sjögren’s syndrome. Semin Arthritis Rheum 2011; 41: 415-423.

‘INCREASED AWARENESS OF SJÖGREN’S

SYNDROME IN THE MEDICAL AND

DENTAL COMMUNITIES FACILITATES IMPROVED Fig. 2 Chronic salivary gland enlargement OUTCOMES FOR THE SJÖGREN’S PATIENT’ Available at: http://www.sjogrens.org/ home/research-programs/clinical-practice- guidelines (accessed January 2018). 7. Grant I A, Hunder G G, Homburger H A, Last revision 16 June 2003. Available at: 17. Sjögren’s Syndrome Foundation. SSF Dyck P J. Peripheral neuropathy associated http://www.cureresearch.com/s/sjogrens_ 5-Year Breakthrough Goal Update: with sicca complex. Neurology 1997; 48: syndrome/stats-country_printer.htm The Average Sjögren’s Diagnosis Time 855-862. (accessed January 2018). Decreases to 3 Years! Available at: 8. Devins G M. Illness intrusiveness and the 12. Sjogren’s syndrome statistics. From http:// http://www.sjogrens.org/home/about- psychosocial impact of lifestyle disruptions www.sjogrens.org. the-foundation/breakthrough-goal- in chronic life-threatening disease. Adv Ren 13. Shiboski C H, Shiboski S C, Seror R et al. /5yearupdate (accessed January 2018). Replace Ther1994; 1: 251-263. 2016 American College of Rheumatology/ 9. Bell M, Askari A, Bookman A et al. European League Against Rheumatism Sjögren’s syndrome: a critical review of classification criteria for primary Sjögren’s clinical management. J Rheumatol 1999; 26: syndrome. Ann Rheum Dis 2017; 76: 9-16. CPD questions 2051-2061. 14. Dry mouth scale launched. Br Dent J 2011; This article has four CPD questions 10. Felger J C, Lotrich F E. Inflammatory 211: 351. attached to it which will earn you one hour of verifiable CPD. To access cytokines in depression: neurobiological 15. Papas A, Singh M, Singh M. The effect of a the free BDA CPD hub, go to http:// mechanisms and therapeutic implications. unique Omega-3 supplement on dry mouth bit.ly/2e3G0sv Neuroscience 2013; 246: 199-229. and dry eye in Sjögren’s patients. Invest 11. Statistics by Country for Sjogren’s Ophthalmol Vis Sci 2007; 48: 377. syndrome. Extrapolations of prevalence and 16. Sjögren’s Syndrome Foundation. Clinical incidence statistics for Sjogren’s syndrome. Practice Guideline Resource Sheets. bdjteam201826