Review Articles

1. Vivino, F. B. (2017). Sjogren’s syndrome: Clinical aspects. Clinical Immunology, 182: 48–54.   KEY ARTICLE!

Succinct clinical review of presentation, diagnosis and treatment considerations. Illustrates the multisystem nature of the disease, and why Sjogren’s remains a clinical diagnosis that cannot be excluded by diagnostic testing.  

https://doi.org/10.1016/j.clim.2017.04.005    
 

2. Brito-Zeron P, Theader E, et al.  Early Diagnosis of primary Sjogren’s syndrome: EULAR-SS task force clinical recommendations. Expert Rev Clin Immunol 2015 Dec. 1-20  

Emphasizes the key role of systemic involvement  Great article other than the unrealistically low estimate of disease prevalence. Low estimates are based on diagnosed, reported cases vs. estimates that reflect the reality that most cases remain undiagnosed. See Helmick article (# 3) regarding estimated prevalence in the U.S.

https://www.ncbi.nlm.nih.gov/pubmed/26691952

 

 

 

 

 

 

Sjogren’s Prevalence  

3. Helmick CG, Felson DT, Lawrence RC, et al. National Arthritis Data Workgroup. Estimates of prevalence of arthritis and other rheumatic conditions in the United States, Part I. Arthritis Rheum. 2008;58:15-25.

Primary Sjogren’s (PSS) has a comparable prevalence to RA, and much higher than SLE or MS. Lack of clear diagnostic tools and provider awareness results in underestimation of Sjogren’s cases. In US, 1 million are currently diagnosed (2008); an estimated 3-4 million adults in US have Sjogren’s.

https://onlinelibrary.wiley.com/doi/full/10.1002/art.23177

 

Pain and Fatigue in Sjogren’s

4, Ng, Wan-Fai. Primary Sjögren’s syndrome: Too dry and too tired. Rheumatology. 2010;49:844-853.

70 % of PSS patients suffer from disabling fatigue. This is a direct manifestation of the disease. Pathogenesis is poorly understood and effective management strategies yet to be identified. 

 https://www.ncbi.nlm.nih.gov/pubmed/20147445

 

5. Vitali,C, Del Papa, N. Pain in primary Sjogren’s syndrome. Best Practice & Research Clinical Rheumatology, 2015;29: 63-70.

Articular, neuropathic and widespread musculoskeletal pain are discussed. These are common Sjogren's features.
https://www.ncbi.nlm.nih.gov/pubmed/26267000

 

6. Omdal R, Mallegren SI, Norheim KB.  Pain and fatigue in primary Sjogren's syndrome. Rheumatology, 2019. (epub pages 1-8). 

Pain and fatigue in Sjogren's have a biologic basis. Describes the fundamental role of the immune system in generating Sjogren's fatigue.  Numerous pain manifestations are reviewed, as well as the profound impact of fatigue.This article is especially helpful for countering outdated ideas that lead to psychologizing and dismissing of fatigue and pain. 

https://www.ncbi.nlm.nih.gov/pubmed/30815693

 

 7.  Mengshoel AM, Norheim KB, Omdal R.  Primary Sjogren's syndrome: fatigue is an ever present, fluctuating, and uncontrollable lack of energy.  Arthritis Care Res 2014;66:1227-32.

Small qualitative study: In depth interviews of nine Sjogren's patients illuminate the reality of fatigue and its impact on wellbeing and function.

https://www.ncbi.nlm.nih.gov/pubmed/22263
 

 

Functional Status and Quality of Life 

8. Segal G, Bowman S et al. Primary Sjogren’s Syndrome: health experiences and predictors of health quality among patients in the United States. Health Qual Life Outcomes. 2009;7:46.

Excellent review. Documents that profound impact that Sjogren's has on QOL is mainly due to fatigue and pain, not    just dryness. 
https://hqlo.biomedcentral.com/articles/10.1186/1477-7525-7-46             
 

9. Sutcliffe N, Stoll T et al. Functional disability and end-organ damage in patients with systemic lupus and Sjogren’s syndrome. J Rheumatol 1998;25(1):63-68.

Functional disability in Primary SS as great as active SLE.
https://www.ncbi.nlm.nih.gov/pubmed/9458204

 

10. Strombeck G, Ekhdahl C et al.Health-related quality of life in primary Sjogren’s syndrome, rheumatoid arthritis and fibromyalgia compared to normal population data using SF-36.

HRQOL was decreased in PSS, and comparable to RA patients who fail TNF inhibitors. Note this was written prior the widespread use of other biologics for RA.

https://www.ncbi.nlm.nih.gov/pubmed/10722254


Mortality from Sjogren's Complications

11. Brito-Zeron P, Ramos-Casals M, et al. Predicting adverse outcomes in primary Sjogren’s syndrome: identification of prognostic factors. Rheumatology 2007;46:1359-1362.

https://www.ncbi.nlm.nih.gov/pubmed/17569749

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12. Brito-Zeron, P, Ramos-Casals M. Systemic Sjogrens: More than a sicca disease. 

"Systemic involvement plays a key role in the prognosis of PSS, and three recent studies, including more than 2,500 patients, have confirmed that pSS is a systemic autoimmune disease."
http://www.the-rheumatologist.org/article/systemic-sjgrens-more-than-a-sicca-disease/  

 

Clinical Practice Guidelines

13.  https://www.sjogrens.org/home/research-programs/clinical-practice-guidelines     KEY REFERENCE

      PCPs should be familiar with the systemic treatment guidelines!                                      

 

http://www.sjogrens.org/files/research/CPGSystemic.pdf

Guidelines for treating general systemic features

 

http://www.sjogrens.org/files/research/OralCPG.pdf

For dental providers

http://www.sjogrens.org/files/research/OcularCPG.pdf

For eye providers
 

14. Price, Rauz, S, Tappuni, A et al.The British Society for Rheumatology guideline for management of adults with primary Sjogren’s Syndrome .Oxford University Press 2017

          See p.16 for management of pregnancy
         https://doi.org/10.1093/rheumatology/kex166

 

 

Sjogren’s Neurologic Manifestations

 

15.  Moreira I, Teixeira F et al.  Frequent involvement of central nervous system in primary Sjogren syndrome.  Rheumatol Int 2015 35(2):289-94.

15 % of 93 patients with PSS had CNS involvement. Neuro symptoms preceded Sjogren’s diagnosis in 64 %. My comment:  Neurologic involvement is often not recognized, and is therefore underdiagnosed.

https://www.ncbi.nlm.nih.gov/pubmed/25056402

 

16. Birnbaum, J.  Clinical peripheral nervous system manifestations of Sjogren syndrome: clinical patterns, diagnostic paradigms, etiopathogenesis and therapeutic strategies.  Neurologist 2010;16(5):287-297.

A 2010 article, but most currently applies. Does not include much about dysautonomia, but covers many other      important features. See blog posts about neuropathy and dysautonomias for more links to excellent resources.
https://www.ncbi.nlm.nih.gov/pubmed/20827117

 

 

 

 

17. Newton JL, Frith J, Powell D et al.Autonomic Symptoms are common are associated with overall symptom burden and disease activity index in primary Sjogren’s syndrome.  Ann Rheum Dis 2012;71: 1973-1979.

Prospective, controlled study of with 317 PSS patients using Composite Autonomic Symptom Scale. Nearly 55 % had evidence of autonomic dysfunction vs. 20 % of controls. Authors recommend routinely assessing Sjogren’s patients for orthostatic intolerance. (Also see Grubb, below)

https://www.ncbi.nlm.nih.gov/pubmed/22562982 
 

18. Kovacs, L, Paprika, D et al.Cardiovascular autonomic dysfunction in primary Sjogren’s syndrome. Rheumatology 2004;43:95-99.

Cardiovascular autonomic nervous system dysfunction seen in majority of 51 PSS patients studied. HR, BP variability, baroreflex sensitivity and CV reflex tests were performed.
https://www.ncbi.nlm.nih.gov/pubmed/12949253

 

19. Grubb BP, Kanjwal Y, Kosinski DJ.  The Postural Tachycardia Syndrome:  A concise guide to diagnosis and management.   J Cardiovasc Electrophysiol 2006;17:1-5

Describes diagnosis and treatment of various forms of orthostatic intolerance, including POTS. POTS and other cardiovascular dysautonomias may be seen in Sjogren’s. 

https://www.ncbi.nlm.nih.gov/pubmed/16426415

 

2o. Tjensvoll AB, Goransson LG.  High headache-related disability in patients with systemic lupus erythematosis and primary Sjogren's syndrome. Eur J Neruol 2014;21(8):1124-30.

SLE and PSS patients have higher burden of headache and more severe headache than headache sufferers without these diseases. This is another overlooked feature in Sjogren's patients.

https://tinyurl.com/y5hpjra2
 

 

Other Helpful Articles and Resources

 

The website of the Sjogren's Foundation-  http://www.sjogrens.org

Highlights include links to Clinical Practice Guidelines, provider resources, patient education brochures and audio CDs from the national conferences.

The Sjogren's Foundation is a reliable resource that draws on the expertise of medical and scientific advisors from around the world.

  

My comment:  Many other websites, including the ACR's, downplay the serious systemic nature of Sjogren's.

 

Schafer, S.  Speak Out Rheum:  Under the Radar:  A physician-patient’s experience with Sjogren’s syndrome.  The Rheumatologist, Nov. 2013

Learn about my story, and read my 2013 analysis of clinical, medical education, and research neglect of Sjogren’s Disease. Note that this was written prior to publication of Clinical Practice Guidelines.

http://www.the-rheumatologist.org/article/speak-out-rheum-under-the-radar/

 

Schafer, S.  Why doesn't my doctor understand Sjogren's?  Blog post in Moisture Seekers newsletter, 1-30-17.

Many factors contribute to Sjogren's Neglect.  More recent analysis in "Sjogren's Neglect I-IV" blog posts, August and September 2019.

http://info.sjogrens.org/conquering-sjogrens/why-doesnt-my-doctor-understand-sjogrens