Saturday, March 5, 2016

Dr. Sarah Schafer: A Sjogren's Bibliography for Your Physician

An enormous thank you to Dr. Sarah Schafer for sharing this excellent resource: a bibliography to share with your physician regarding all things Sjogren's.

She says,

     "I have been teaching PCPs about Sjogren’s.  Very gratifying—and horrifying how little they know.  They still do not get taught in medical school, and very little in residency.  Most of them are still stuck in the “sicca only” world.
     Here is the annotated bibliography I created for a handout.   It would be useful for patients who need to educate their MDs."  
Watch for other upcoming excellent posts from Dr. Schafer.

Feel free to print this bibliography to take to your next appointment if you feel that your doctor's education has been lacking regarding Sjogren's syndrome.

Sjogren's for Primary Care: More than Dry
Sarah Schafer, MD

Sjogren’s Prevalence
  1. 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 prevalence than SLE, MS.  Lack of clear diagnostic criteria and provider awareness results in underestimation of Sjogren’s cases, resulting in widely varied estimates.   In US, 1 million are currently diagnosed; estimated 3-4 million adults total in US have primary or secondary Sjogren’s, mostly undiagnosed.

Recommended Review Article 
  1. 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   
Excellent clinical review, although unrealistically low disease prevalence stated, based on diagnosed cases vs. extrapolation to include undiagnosed cases.  See Helmick article.

Fatigue in Sjogren’s
  1. Ng, Wan-Fai. Primary Sj√∂gren’s syndrome: Too dry and too tired. Rheumatology. 2010;49:844-853.
70 % of PSS suffer from disabling fatigue.  Pathogenesis poorly understood and effective management strategies yet to be identified.  

Pain in Sjogren’s
  1. 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. 

Functional status and Quality of Life  
  1. 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 of impact on QOL                                     
  1. 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 PSS as great as SLE.
  1. 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.  Ann Rheum Dis. 2004;63(6): 723–729. 
HRQOL was decreased in PSS, and comparable to levels of women with RA and fibromyalgia

Mortality from Sjogren’s Complications
  1. 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
  1. Brito-Zeron, P, Ramos-Casals M.   Systemic Sjogrens: More than a sicca disease.

Sjogren’s Neurologic manifestations
  1. Birnbaum, J.   Peripheral nervous system manifestations of Sjogren Syndrome: Clinical Peripheral Nervous System Manifestations of Sj√∂gren Syndrome: Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies.  Neurologist 2010;16(5):287-297
  1. 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 %. 

  1. 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.  
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. (see Grubb, below) 
  1. 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. 
  1. Tiensvoll 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

Other Helpful articles and resources 
Up-to-date excellent resource for patients and providers

Schafer, S.  Speak Out Rheum:  Under the Radar:  A physician-patient’s experience with Sjogren’s syndrome.  The Rheumatologist, Nov. 2013
Analysis of clinical, education and research neglect of Sjogren’s, along with personal story.

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.  (See Neurologic manifestations


Nicole said...

Thank you Julia and dr. Schaefer! What a great resource.

Fajar Optimasi said...
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Anonymous said...

Thanks! This could prove helpful.