Summary
Definition
History and exam
Key diagnostic factors
- fatigue
- dry eyes
- dry mouth
Other diagnostic factors
- vasculitis
- dental caries
- increased oral fungal and bacterial infections
- musculoskeletal pain
- thyroid disease
- urogenital disease
- pulmonary disease
- gastrointestinal disease
- peripheral neuropathy
- corneal ulceration
- no saliva pool
- enlarged salivary glands
- facial pain
- burning mouth syndrome
Risk factors
- female
- systemic lupus erythematosus (SLE)
- rheumatoid arthritis
- systemic sclerosis (scleroderma)
- HLA class II markers
- age 40 to 70
- genetic inheritance
Diagnostic tests
1st tests to order
- Schirmer test
- anti-60 kD (SS-A) Ro and anti-La (SS-B)
Tests to consider
- sialometry
- minor salivary gland biopsy
- lissamine green test
- fluorescein corneal staining test
- parotid sialography
- salivary gland technetium-99m pertechnetate scintigraphy
- skin biopsy
- angiography
- urinalysis
- serum electrolytes
Emerging tests
- MRI salivary glands
- ultrasound salivary glands
Treatment algorithm
dry eyes
dry mouth
with fatigue
with musculoskeletal manifestations
with vasculitis
with renal tubular acidosis
with neuropathy
Contributors
Authors
Biji T. Kurien, PhD
Staff Scientist
Arthritis and Clinical Immunology Research Program
Oklahoma Medical Research Foundation
Research Associate
Department of Veterans Affairs
Oklahoma City
OK
Disclosures
BTK is an author of several articles cited in this topic.
R. Hal Scofield, MD
Professor
Section of Endocrinology and Diabetes
Department of Medicine
University of Oklahoma Health Sciences Center
Member
Arthritis and Clinical Immunology Research Program
Oklahoma Medical Research Foundation
Associate Chief of Staff for Research and Staff Physician
Department of Veterans Affairs Medical Center
Oklahoma City
OK
Disclosures
RHS has received compensation from Jessen Pharmaceuticals as a member of a clinical trial advisory board, and honorarium from Merck and Company for consultation on an investigational agent for Sjogren’s syndrome. RHS is the principal investigator for grants from the US National Institute of Health and the US Department of Veteran Affairs for institutions studying Sjorgren’s syndrome. RHS is an author of several articles cited in this topic.
Peer reviewers
Nora Sandorfi, MD
Associate Professor of Medicine
University of Pennsylvania
Philadelphia
PA
Disclosures
NS declares she has no competing interests.
References
Key articles
Foulks GN, Forstot SL, Donshik PC, et al. The Sjogren's Syndrome Foundation clinical practice guidelines. Ocular management in Sjogren's patients. 2015 [internet publication].Full text
Vivino FB, Zero D, Brennan M, et al. Sjogren's Syndrome Foundation's clinical practice guidelines. Oral management: caries prevention in Sjogren's patients. 2015 [internet publication].Full text
Carsons SE, Vivino FB, Parke A, et al. Treatment guidelines for rheumatologic manifestations of Sjögren's syndrome: use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain. Arthritis Care Res (Hoboken). 2017 Apr;69(4):517-27.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Systemic lupus erythematosus (SLE)
- Rheumatoid arthritis
- Hepatitis C
More DifferentialsGuidelines
- 2023 American College of Rheumatology (ACR) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic disease
- 2022 American College of Rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal disease
More Guidelines
Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer