A few months ago a patient with arthralgia of the lower extremities was brought to the musculoskeletal system. When discussing her medical history, she mentioned a diagnosis of Sjogren's Syndrome (SS) and added, "I bet you have no idea what it is?" She was right, I wasn't.
Although this lack of knowledge was of course not the be-all and end-all, I knew that at that moment I had missed one of the many early opportunities for assessment to gain patient trust . I later reflected on how our first meeting could have gone if I had an idea of her condition, its connections to musculoskeletal practice, and the potential strain on her life.
Sjogren's research helped in our subsequent treatment sessions, and it is perhaps not surprising that the first UK guidelines for dealing with adults with Sjogren's were not published until 2017.
What is Sjogren's Syndrome (SS)?
SS takes its name from the Swedish ophthalmologist Henrik Samuel Sjögren (1899 – 1986). Briefly mentioned in my post on extra-articular manifestations, SS is an autoimmune rheumatic disorder described under the British Society for Rheumatology (BSR) Long Term Conditions.
Extra-articular manifestations are the key to early detection
Sjögrens (Sho-Grens) is a complex disease that is most often associated with dry mouth and eyes, tiredness and pain. It is known that it predominantly affects the moisture-producing glands of the body and that it has neurological, respiratory and systemic components. Chronic dry cough and dry mouth (xerostomia) are characteristic of SS and patients suffer from decreased salivary flow rate, increased salivary viscosity, and dry mucous membranes. Lung manifestations include interstitial lung disease and lung cysts.
Systemic features that were observed in around 70% of patients relate to the joints, lungs, skin as well as peripheral and central nerves. The frequency of the neurological components varies in SS. Alegria et al. (2016) found that peripheral and central neurological manifestations were around 15% and 5%, respectively. They believed that these manifestations were associated with higher disease activity and were more common in patients with prior neurological involvement. The authors recommended that clinicians pay more attention to neurological symptoms in SS in general. Pure sensory and sensorimotor neuropathies were the most common manifestations of the peripheral nervous system in patients with SS while the involvement of the cranial nerve V (trigeminal nerve) was also described in Sjögren.
In addition, it is known that SS is associated with both fibromyalgia and systemic lupus erythematosus (SLE), an increased risk of developing lymphoma and a higher prevalence in women.
Chronic dry cough and dry mouth (xerostomia) are characteristic of Sjogren's syndrome
There are also more overlapping conditions, particularly thyroid disease, primary biliary cirrhosis, and celiac disease; Once again the need to be aware of the wider context proves necessary for assessment and management.
As with several rheumatological diseases, there is often a delay between the onset of symptoms and diagnosis in SS. Shiboski and colleagues (2017) developed an international set of diagnostic classification criteria for the primary SS using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) that are worth further review for those interested.
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Published Guidelines
There is currently no known cure for SS. The treatment recommendations are therefore aimed at treating symptoms, preventing further damage, improving quality of life and helping doctors to select the appropriate treatment for patients.
Carsons et al. (2017) have published treatment guidelines for rheumatological manifestations in SS. The guidelines were based on a review of published studies, case reports, and contributions from doctors and patients. Unfortunately, they found a lack of well-designed, controlled studies in the literature. They examined a number of immunosuppressive treatments as well as non-pharmacological interventions in their review, and while pharmacological management does not fall within the scope of this article, it is interesting to know that some biologics, such as anti-TNF therapy, are not recommended in the Treatment of SS.
Of importance for physiotherapists was the focus of Carson et al. For tiredness. The causes of fatigue in SS are numerous, and the only highly recommended treatment for fatigue has been exercise, which "provides the same benefit for SS patients as is seen in patients with rheumatoid arthritis (RA), SLE, or multiple sclerosis (MS)".
More recently, the BSR published its UK guidelines for the management of SS, including non-pharmacological and pharmacological treatments. Their guidelines are accredited by the National Institute for Excellence in Health and Care (NICE).
The importance of holistic management and the emphasis on non-pharmacological therapies and general support are of paramount importance to physical therapists. The BSR guideline is an easy-to-read document that covers the treatment of ocular and oral manifestations, as well as systemic (extra-glandular), pulmonary, and neurological manifestations. Lifestyle management strategies can benefit SS patients when fatigue severely affects their ability to perform daily activities. However, there does not appear to be a “gold standard” by which to accurately measure the multifactorial nature of fatigue. Strategies recommended by the NICE guidelines for fatigue include sleep and activity management, relaxation techniques, cognitive behavioral therapy, and graded exercise therapy.
Exercise "strongly recommended" in Sjögrens
It is great to see exercise is highly recommended as a treatment for another rheumatoid condition, but unfortunately the exercise recommendations are lacking in detail because they are based on inconclusive, rare evidence. That being said, both Carsons et al. (2017) and Price et al. (2017) found that patients with SS would benefit from “moderate to high-intensity training”, both guidelines cited the same author from about 10 years earlier to support these recommendations. It is certainly a useful starting point, but it leaves many unanswered questions about exercising in the SS.
Difficulty quantifying exercise and defining fatigue in the SS
Strömbeck et al. (2007) examined the effects of a 12-week training program on women with primary SS. Their treatment group did a “high-intensity” Nordic walking exercise for 45 minutes for 3 minutes per week. The control group did "low-intensity movement exercises" 3 times a week at home without a fixed period of time. The authors found a decrease in fatigue and an increase in aerobic capacity after the 12-week exercise program, and therefore concluded that high-intensity aerobic exercise may be beneficial for patients with SS. The small sample size in the study cannot be ignored as a limitation. There was significant wear and tear on the recruited participants (94-19).
Fatigue is common in many rheumatological diseases and often has a major impact on the patient. “Segal et al. (2008) focused their research on the effects of fatigue on quality of life in SS. They used a range of different fatigue measures. The main purpose of this study was to examine the relative contributions of disease status, behavioral and immunological factors to fatigue in SS. Her main finding was that psychological factors are determinants of fatigue and that depression correlates with the severity of fatigue, although it is not a cause of fatigue.
Summary
Sjogren's syndrome (SS) is a complex, autoimmune, rheumatic disease that can be treated by a wide variety of healthcare professionals. As with too many rheumatological diseases, there can be significant delays in diagnosis so that awareness of these diseases must become commonplace. Signs of dry mouth and eyes are no longer the only salient features on Sjogren's. The disease also includes respiratory, neurological, and systemic components. Fatigue is widespread in the SS, but manifests itself in a multifactorial nature that is not yet fully understood or defined.
Aerobic training with moderate to high intensity is strongly recommended for the treatment of fatigue in patients with SS. However, there is little evidence to support training recommendations and more thorough research is needed to determine training specifications such as type, duration, and frequency.
With two comprehensive guides published in 2017, I would hope that SS would become more visible to physiotherapists. Raising awareness of Sjögren and rheumatology as a whole helps empower patients, their families and multidisciplinary doctors. Today you may be asked: "Have you heard of Sjogrens?"
This post was originally published in August 2017 and written by Chris Martey . The page has now been updated for freshness, accuracy and completeness.
References
Alegria, GC, Guellec, D., Mariette, X., Gottenberg, JE, Dernis, E., Dubost, JJ, Trouvin, AP, Hachulla, E., Larroche, C., Le Guern, V. and Cornec, D .., (2016). Epidemiology of neurological manifestations in Sjogren's syndrome: data from the French ASSESS cohort. RMD open, 2 (1).
Carsons, SE, Vivino, FB, Parke, A., Carteron, N., Sankar, V., Brasington, R., Brennan, MT, Ehlers, W., Fox, R., Scofield, H., Hammitt, KM, Birnbaum, J., Kassan, S. and Mandel, S. (2017) Treatment guidelines for rheumatological manifestations of Sjogren's syndrome: use of biological agents, treatment of fatigue and inflammatory musculoskeletal pain. Journal of Arthritis Care Research, 69, pp. 517-527.
Ng, W & Bowman, S. (2010) Primary Sjogren's Syndrome: Too Dry and Too Tired, Journal of Rheumatology, 49 (5), pp. 844-853.
Price, EJ, Rauz, S., Tappuni, AR, Sutcliffe, N., Hackett, KL, Barone, F., Granata, G., Ng, WF, Fisher, BA, Bombardieri, M. & Astorri, E. (2017). The British Society for Rheumatology guideline for the treatment of adults with primary Sjogren's syndrome. Journal of Rheumatology, p.166.
Segal, B., Thomas, W., Rogers, T., Leon, JM, Hughes, P., Patel, D.,… Moser, K. (2008) Prevalence, severity and predictors of fatigue in primary Sjogren Syndrome. Journal of Arthritis and Rheumatism, 59 (12), pp. 1780-1787.
Shiboski, CH, Shiboski, SC, Seror, R., Criswell, LA, Labetoulle, M., Lietman, TM, Rasmussen, A., Scofield, H., Vitali, C., Bowman, SJ, Mariette, X. (2017) 2016 American College of Rheumatology / European League Against Rheumatism Classification Criteria for Primary Sjogren's Syndrome: A Consensus and a Data-Driven Methodology with Three International Patient Cohorts. Journal of Arthritis & Rheumatology, 69: pp. 35-45.
Strömbeck, E., Theander, &, L., Jacobsson (2007) Effects of Exercise on Aerobic Capacity and Fatigue in Women with Primary Sjogren's Syndrome, Journal of Rheumatology, 46 (5), pp. 868-871 .
