Sjogren Syndrome Questions & Answers

Updated: Apr 07, 2020
  • Author: Sriya K Ranatunga, MD, MPH; Chief Editor: Herbert S Diamond, MD  more...
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Questions & Answers

Overview

What is Sjögren syndrome?

What are the extraglandular features of Sjögren syndrome?

What are the dermatologic features of Sjögren syndrome?

How are primary and secondary Sjögren syndrome differentiated?

Which conditions should be included in the differential diagnoses of Sjögren syndrome?

What are the possible complications of Sjögren syndrome?

Which antibodies are most commonly associated with Sjögren syndrome?

What are the treatment options for Sjögren syndrome?

What are systems used to classify Sjögren syndrome?

When are the American-European Consensus Group (AECG) criteria used in the diagnosis of Sjögren syndrome?

What are the AECG diagnostic criteria for primary Sjögren syndrome?

What are the AECG diagnostic criteria for secondary Sjögren syndrome?

How accurate are the AECG diagnostic criteria for Sjögren syndrome?

What are the AECG criteria for exclusion of a diagnosis of Sjögren syndrome?

What is the American College of Rheumatology/European European League Against Rheumatism (ACR/EULAR) classification criteria used in the diagnosis of Sjögren syndrome?

What should be included in patient education information on Sjögren syndrome?

What are the subtypes of Sjögren syndrome?

What parts of the body are affected by Sjögren syndrome?

What is the pathogenesis of Sjögren syndrome?

What is the frequency of HLA-DR52 in patients with primary Sjögren syndrome?

What is the role of genetics in the etiology of Sjögren syndrome?

What is the role of viral infection in the pathogenesis of Sjögren syndrome?

What is the glandular pathology of Sjögren syndrome?

What is the role of neuroendocrine mechanism in the pathogenesis of Sjögren syndrome?

What is the role of apoptotic mechanisms in the pathogenesis of Sjögren syndrome?

What causes extraglandular involvement in the pathogenesis of Sjögren syndrome?

What is the role of sex hormones in the pathogenesis of Sjögren syndrome?

What is the prevalence and other epidemiologic features of Sjögren syndrome?

What is the prognosis of Sjögren syndrome?

What are the signs of antiphospholipid syndrome in Sjögren syndrome?

What morbidity is associated with Sjögren syndrome?

How much is the risk for non-Hodgkin lymphoma increased in patients with Sjögren syndrome?

What is the most common subtype of non-Hodgkin lymphoma in patients with Sjögren syndrome?

What is a biomarker for non-Hodgkin lymphoma in primary Sjögren syndrome?

What are the risk factors for lymphoma in Sjögren syndrome?

What are the possible complications of Sjögren syndrome in pregnant women?

Presentation

What are the types of extraglandular involvement in Sjögren syndrome?

How does Sjögren syndrome affect quality of life?

What is the significance of a finding of parotitis in the evaluation of Sjögren syndrome?

What are the pulmonary symptoms of Sjögren syndrome?

What are the clinical presentations of secondary Sjögren syndrome?

What is the clinical presentation of Sjögren syndrome?

What are the signs and symptoms of Sjögren syndrome?

Which alternative causes of sicca symptoms should be considered in the evaluation of Sjögren syndrome?

Which medications should be considered alternative causes of sicca symptoms in the evaluation of Sjögren syndrome?

How is dry mouth characterized in Sjögren syndrome?

How are dry eyes characterized in Sjögren syndrome?

What are cutaneous symptoms of Sjögren syndrome?

What are the cutaneous vasculitis symptoms in Sjögren syndrome?

What are the GI symptoms of Sjögren syndrome?

What are the cardiac symptoms of Sjögren syndrome?

What are the neurologic symptoms of Sjögren syndrome?

What are the renal symptoms of Sjögren syndrome?

Which symptoms of Sjögren syndrome are related to the presence of antiphospholipid antibodies?

How do the symptoms of primary Sjögren syndrome compare with those of secondary Sjögren syndrome?

What physical exam findings of the parotid glands suggest Sjögren syndrome?

Which cutaneous findings suggest Sjögren syndrome?

Which urinary conditions are associated with Sjögren syndrome?

What are the two groups of physical signs of primary Sjögren syndrome?

What should be included in the ophthalmologic exam for suspected Sjögren syndrome?

What is the role of the Schirmer test in the evaluation of Sjögren syndrome?

What are the oral signs of Sjögren syndrome?

What are mucous membrane findings suggestive of Sjögren syndrome?

What are physical skin findings suggestive of Sjögren syndrome?

Which findings of hair and nail exam suggest Sjögren syndrome?

What are GI findings suggestive of Sjögren syndrome?

What are respiratory findings suggestive of Sjögren syndrome?

What is the significance of a finding of arthritis in the evaluation of Sjögren syndrome?

Which neurologic findings suggest Sjögren syndrome?

How is peripheral neuropathy manifested in Sjögren syndrome?

Which CNS findings suggest Sjögren syndrome?

DDX

What factors contribute to the underdiagnosis or misdiagnosis of Sjögren syndrome?

How is Sjögren syndrome differentiated from other causes of parotid gland enlargement?

The histologic findings of which disorders are consistent with Sjögren syndrome?

What conditions should be included in the differential diagnoses of Sjögren syndrome in patients with sicca symptoms?

What conditions should be included in the differential diagnoses of Sjögren syndrome in patients with parotid enlargement?

Which disorders associated with Sjögren syndrome should be part of the evaluation?

What are the differential diagnoses for Sjogren Syndrome?

Workup

Which autoimmune findings suggest Sjögren syndrome?

What is the role of serum protein electrophoresis in the diagnosis of Sjögren syndrome?

What is the role of lab tests in the diagnosis of Sjögren syndrome?

Which lab test results suggest Sjögren syndrome?

How frequently are autoantibodies found in the evaluation of Sjögren syndrome?

What is the role of the Schirmer test in the diagnosis of Sjögren syndrome?

How frequently is rheumatoid factor present in Sjögren syndrome?

What is the importance of antinuclear antibodies in the diagnosis of Sjögren syndrome?

What is the role of staining in the evaluation of Sjögren syndrome?

What is the role of salivary testing in the diagnosis of Sjögren syndrome?

What is the role of the erythrocyte sedimentation rate (ESR) in the diagnosis of Sjögren syndrome?

What is the role of protein profiling in the diagnosis of Sjögren syndrome?

Which tests may be considered in the diagnosis of Sjögren syndrome?

What is the role of SSA and SSB antibody testing in the diagnosis of Sjögren syndrome?

What is the role of CBC count in the diagnosis of Sjögren syndrome?

How is sialography used in the diagnosis of Sjögren syndrome?

How is scintigraphy used in the diagnosis of Sjögren syndrome?

What is the significance of positive findings on sialography or scintigraphy in the evaluation of Sjögren syndrome?

What is the best test to establish a diagnosis of Sjögren syndrome?

How is a salivary gland biopsy used in the evaluation of Sjögren syndrome?

What are the characteristic findings of minor salivary gland biopsy in Sjögren syndrome?

How are focal scores interpreted in the diagnosis of Sjögren syndrome?

What is the significance of a finding of lymphocytic infiltrates in the evaluation of Sjögren syndrome?

Treatment

What are the treatment options for Sjögren syndrome?

What are treatment options for skin and vaginal dryness in Sjögren syndrome?

What are the treatment options for arthralgia and arthritis in Sjögren syndrome?

What are the treatment options for major organ involvement in Sjögren syndrome?

How are serious manifestations of Sjögren syndrome managed?

When is long-term anticoagulation indicated in the treatment of Sjögren syndrome?

What is the role of mycophenolate sodium in the treatment of Sjögren syndrome?

What is the role of rituximab in the treatment of Sjögren syndrome?

What is the efficacy of rituximab in the treatment of fatigue and oral dryness caused by Sjögren syndrome?

What is the efficacy of rituximab in the treatment of extraglandular symptoms of Sjögren syndrome?

What is the role of TNF inhibitors in the treatment of Sjögren syndrome?

What is the role of immune mediators in the treatment of Sjögren syndrome?

What is included in emergency department (ED) treatment of Sjögren syndrome?

What is the focus of inpatient care of Sjögren syndrome?

What is included in the outpatient care of Sjögren syndrome?

What is included in the monitoring of Sjögren syndrome?

What is the role of surgery in the treatment of Sjögren syndrome?

When are biopsies indicated in the management of Sjögren syndrome?

What is the purpose of a rheumatology consultation for Sjögren syndrome?

What is the purpose of an ophthalmology consultation for Sjögren syndrome?

What is the purpose of an otolaryngology consultation for Sjögren syndrome?

Which specialist consultations may be necessary in the treatment of Sjögren syndrome?

How are dry eyes graded in Sjögren syndrome?

What new therapeutic strategies are under investigation for the treatment of Sjögren syndrome?

What are the treatment guidelines for dry eye in Sjögren syndrome?

What are the treatment options for dry eyes in Sjögren syndrome?

What are treatments for level 1 dry eye symptoms in Sjögren syndrome?

What are treatments for level 2 dry eye symptoms in Sjögren syndrome?

Which drugs are used in the treatment of level 2 dry eye disease in Sjögren syndrome?

What are the treatments for level 3 dry eye disease in Sjögren syndrome?

What are the treatments for level 4 dry eye disease in Sjögren syndrome?

How is dry mouth due to Sjögren syndrome treated?

What is the role of toothpaste in the treatment of dry mouth in Sjögren syndrome?

How is oral candidiasis treated in Sjögren syndrome?

How is sinusitis and sinus blockade treated in Sjögren syndrome?

Guidelines

According to the Sjögren’s Syndrome Foundation (SSF) guidelines, what are the treatment goals for Sjögren syndrome?

What are the Sjögren’s Syndrome Foundation (SSF) treatment guidelines for dry mouth in Sjögren syndrome?

What is the basis for the SSF recommendations for the treatment of dry eye in Sjögren syndrome?

What are the SSF treatment recommendations for severity 1 dry eye without meibomian gland disease in Sjögren syndrome?

What are the SSF treatment recommendations for severity 1 dry eye with meibomian gland disease in Sjögren syndrome?

What are the SSF treatment recommendations for severity 2 dry eye without meibomian gland disease in Sjögren syndrome?

What are the SSF treatment recommendations for severity 2 dry eye with meibomian gland disease in Sjögren syndrome?

What are the SSF treatment recommendations for severity 3 dry eye without meibomian gland disease in Sjögren syndrome?

What are the SSF treatment recommendations for severity 3 dry eye with meibomian gland disease in Sjögren syndrome?

What are the SSF treatment recommendations for severity 4 dry eye without meibomian gland disease in Sjögren syndrome?

What are the SSF treatment recommendations for severity 4 dry eye with meibomian gland disease in Sjögren syndrome?

How were the Sjögren’s Syndrome Foundation (SSF) guidelines formulated for the treatment of inflammatory musculoskeletal pain in Sjögren syndrome?

What are the Sjögren’s Syndrome Foundation (SSF) treatment guidelines for inflammatory musculoskeletal pain in Sjögren syndrome?

What is the SSF recommended treatment for fatigue in Sjögren syndrome?

Which treatments for fatigue in patients with Sjögren syndrome are not recommended by SSF?

According to SSF guidelines, which treatments have insufficient evidence for their use in treating fatigue in Sjögren syndrome?

What are the SSF guidelines for the use of tumor necrosis factor–alpha (TNF-?) inhibitors in the treatment of Sjögren syndrome?

What are the SSF guidelines indications for the use of rituximab in the treatment of Sjögren syndrome?

For which systemic manifestations of Sjögren syndrome should treatment with rituximab be considered?

What are possible adverse effects of rituximab in the treatment of Sjögren syndrome?

What should be considered when using rituximab to treat Sjögren syndrome?

According to the EULAR guidelines, how should patients with Sjögren syndrome be managed?

What are the EULAR treatment guidelines for dry mouth in Sjögren syndrome?

What are the EULAR Guidelines for the management of Sjogren syndrome?

What are the EULAR treatment guidelines for dry eyes in Sjögren syndrome?

What are the EULAR treatment guidelines for fatigue and/or pain in Sjögren syndrome?

What are the EULAR treatment guidelines for systemic disease in Sjögren syndrome?

Medications

Which medications are used in the treatment of Sjögren syndrome?

Which medications in the drug class Antineoplastics, Monoclonal Antibody are used in the treatment of Sjogren Syndrome?

Which medications in the drug class Corticosteroids are used in the treatment of Sjogren Syndrome?

Which medications in the drug class Immunomodulators are used in the treatment of Sjogren Syndrome?

Which medications in the drug class Antineoplastics, Alkylating are used in the treatment of Sjogren Syndrome?

Which medications in the drug class Antimalarials are used in the treatment of Sjogren Syndrome?

Which medications in the drug class Ophthalmic Lubricants are used in the treatment of Sjogren Syndrome?

Which medications in the drug class Gastrointestinal Agents, Other are used in the treatment of Sjogren Syndrome?