Sjögren’s syndrome—management

Author(s):  
Wan-Fai Ng ◽  
Arjan Vissink ◽  
Elke Theander ◽  
Francisco Figueiredo

Management of Sjögren's syndrome (SS) encompasses confirmation of diagnosis, disease assessment, and treatment of glandular and systemic manifestations including special situations such as pregnancy and SS-related lymphoma. The American European Consensus Group classification criteria 2002 are the current gold standard for the diagnosis of SS. Salivary gland sialometry, sialochemistry, and ultrasound and tear osmolarity may be useful adjuncts. Symptoms of SS are non-specific and must be actively explored. When assessing patients with SS, it is important to consider not only objective parameters such as abnormalities in blood tests and changes in tear and salivary flow, but also patient-reported outcome measures and impact on quality of life. Current management of patients with SS is hampered by the lack of evidence-based strategies. The symptoms experienced by patients with SS are often not fully appreciated by clinicians, which may contribute to the suboptimal management of the condition. Management of fatigue remains a major challenge and a holistic, multidisciplinary approach is recommended. Factors that may contribute to fatigue should be fully addressed. Recent advances in the understanding of the pathogenic mechanisms of SS have informed more targeted therapeutic strategies with some promising data. Optimal management of SS requires expertise from different disciplines. Combined clinics with rheumatology, oral medicine, and ophthalmology input will improve care and communications as well as reduce the number of clinic visits for patients and healthcare-related cost. Effective link between pSS specialists, dentists, opticians, and general practitioners will facilitate early diagnosis and reduce risk of long-term disability of SS.

Author(s):  
Wan-Fai Ng ◽  
Arjan Vissink ◽  
Elke Theander ◽  
Francisco Figueiredo

Management of Sjögren’s syndrome (SS) encompasses confirmation of diagnosis, disease assessment, and treatment of glandular and systemic manifestations including special situations such as pregnancy and SS-related lymphoma. The American European Consensus Group (AECG) classification criteria 2002 are the current gold standard for the diagnosis of SS. Salivary gland sialometry, sialochemistry, and ultrasound and tear osmolarity may be useful adjuncts. Recently, preliminary classification criteria of the American College of Rheumatology have been introduced as an alternative to the AECG criteria. Symptoms of SS are non-specific and must be actively explored. When assessing patients with SS, it is important to consider not only objective parameters such as abnormalities in blood tests and changes in tear and salivary flow, but also patient-reported outcome measures and impact on quality of life. Current management of patients with SS is hampered by the lack of evidence-based strategies. The symptoms experienced by patients with SS are often not fully appreciated by clinicians, which may contribute to the suboptimal management of the condition. Management of fatigue remains a major challenge and a holistic, multidisciplinary approach is recommended. Factors that may contribute to fatigue should be fully addressed. Recent advances in the understanding of the pathogenic mechanisms of SS have informed more targeted therapeutic strategies with some promising data. Optimal management of SS requires expertise from different disciplines. Combined clinics with rheumatology, oral medicine, and ophthalmology input will improve care and communications as well as reduce the number of clinic visits for patients and healthcare-related cost. Effective link between pSS specialists, dentists, opticians, and general practitioners will facilitate early diagnosis and reduce risk of long-term disability of SS.


Author(s):  
Wan-Fai Ng ◽  
Arjan Vissink ◽  
Elke Theander ◽  
Francisco Figueiredo

Management of Sjögren’s syndrome (SS) encompasses confirmation of diagnosis, disease assessment, and treatment of glandular and systemic manifestations including special situations such as pregnancy and SS-related lymphoma. The 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria are the current gold standard for the diagnosis of SS. These criteria replace the 2002 American European Consensus Group (AECG) classification criteria. Salivary gland sialometry, sialochemistry, and ultrasound and tear osmolarity may be useful adjuncts. Symptoms of SS are non-specific and must be actively explored. When assessing patients with SS, it is important to consider not only objective parameters such as abnormalities in blood tests and changes in tear and salivary flow, but also patient-reported outcome measures and impact on quality of life. Current management of patients with SS is hampered by the lack of evidence-based strategies. The symptoms experienced by patients with SS are often not fully appreciated by clinicians, which may contribute to the suboptimal management of the condition. Management of fatigue remains a major challenge and a holistic, multidisciplinary approach is recommended. Factors that may contribute to fatigue should be fully addressed. Recent advances in the understanding of the pathogenic mechanisms of SS have informed more targeted therapeutic strategies with some promising data. Optimal management of SS requires expertise from different disciplines. Combined clinics with rheumatology, oral medicine, and ophthalmology input will improve care and communications as well as reduce the number of clinic visits for patients and healthcare-related cost. Effective link between pSS specialists, dentists, opticians, and general practitioners will facilitate early diagnosis and reduce risk of long-term disability of SS.


Author(s):  
Wan-Fai Ng ◽  
Arjan Vissink ◽  
Elke Theander ◽  
Francisco Figueiredo

Management of Sjögren’s syndrome (SS) encompasses confirmation of diagnosis, disease assessment, and treatment of glandular and systemic manifestations including special situations such as pregnancy and SS-related lymphoma. The American European Consensus Group (AECG) classification criteria 2002 are the current gold standard for the diagnosis of SS. Salivary gland sialometry, sialochemistry, and ultrasound and tear osmolarity may be useful adjuncts. Recently, preliminary classification criteria of the American College of Rheumatology have been introduced as an alternative to the AECG criteria. Symptoms of SS are non-specific and must be actively explored. When assessing patients with SS, it is important to consider not only objective parameters such as abnormalities in blood tests and changes in tear and salivary flow, but also patient-reported outcome measures and impact on quality of life. Current management of patients with SS is hampered by the lack of evidence-based strategies. The symptoms experienced by patients with SS are often not fully appreciated by clinicians, which may contribute to the suboptimal management of the condition. Management of fatigue remains a major challenge and a holistic, multidisciplinary approach is recommended. Factors that may contribute to fatigue should be fully addressed. Recent advances in the understanding of the pathogenic mechanisms of SS have informed more targeted therapeutic strategies with some promising data. Optimal management of SS requires expertise from different disciplines. Combined clinics with rheumatology, oral medicine, and ophthalmology input will improve care and communications as well as reduce the number of clinic visits for patients and healthcare-related cost. Effective link between pSS specialists, dentists, opticians, and general practitioners will facilitate early diagnosis and reduce risk of long-term disability of SS.


Author(s):  
Mirjana Sijan Gobeljic ◽  
Vera Milic ◽  
Nada Pejnovic ◽  
Nemanja Damjanov

Abstract Sjogren’s syndrome (SS) is a complex, chronic, systemic, autoimmune disease that mainly affects the exocrine glands, especially the salivary and lacrimal glands, leading to the dryness of the mouth and eyes, along with fatigue, joint and muscle pain. The prevalence of SS is estimated to be between 0.05% and 1% in European population. Diagnosis of SS is based on the revised criteria of the American-European consensus group (AECG). Sjogren’s syndrome can be subclassified into primary disease (primary Sjogren syndrome, pSS) and a secondary disease (secondary Sjogren syndrome, sSS) when present with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis. The decrease in salivary flow and qualitative alterations in saliva could explain many of the oral manifestations frequently present in patients with SS. Low salivary flow may affect chewing, swallowing, speech and sleeping in pSS patients. Oral manifestations include dental erosion, dental caries, mucosal infection, ulcers and oral candidiasis. Recent studies reveal that pSS patients experience impaired olfactory and gustatory functions and have higher occurrence of oral complications such as dysgeusia, burning sensation in the tongue (BST) and halitosis. The exocrine manifestations and systemic involvement in SS significantly impact the patient’s perception of oral healthrelated quality of life (OHRQoL).


2020 ◽  
pp. annrheumdis-2020-218599
Author(s):  
Alan N Baer ◽  
Jacques-Eric Gottenberg ◽  
E William St Clair ◽  
Takayuki Sumida ◽  
Tsutomu Takeuchi ◽  
...  

ObjectivesTo evaluate efficacy and safety of abatacept in adults with active primary Sjögren’s syndrome (pSS) in a phase III, randomised, double-blind, placebo-controlled trial.MethodsEligible patients (moderate-to-severe pSS [2016 ACR/European League Against Rheumatism (EULAR) criteria], EULAR Sjögren’s Syndrome Disease Activity Index [ESSDAI] ≥5, anti-SS-related antigen A/anti-Ro antibody positive) received weekly subcutaneous abatacept 125 mg or placebo for 169 days followed by an open-label extension to day 365. Primary endpoint was mean change from baseline in ESSDAI at day 169. Key secondary endpoints were mean change from baseline in EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) and stimulated whole salivary flow (SWSF) at day 169. Other secondary clinical endpoints included glandular functions and patient-reported outcomes. Selected biomarkers and immune cell phenotypes were examined. Safety was monitored.ResultsOf 187 patients randomised, 168 completed double-blind period and 165 continued into open-label period. Mean (SD) baseline ESSDAI and ESSPRI total scores were 9.4 (4.3) and 6.5 (2.0), respectively. Statistical significance was not reached for primary (ESSDAI −3.2 abatacept vs −3.7 placebo, p=0.442) or key secondary endpoints (ESSPRI, p=0.337; SWSF, p=0.584). No clinical benefit of abatacept over placebo at day 169 was seen with other clinical and PRO endpoints. Relative to baseline, abatacept was associated with significant differences vs placebo in some disease-relevant biomarkers (including IgG, IgA, IgM-rheumatoid factor) and pathogenic cell subpopulations (post hoc analyses). No new safety signals were identified.ConclusionsAbatacept treatment did not result in significant clinical efficacy compared with placebo in patients with moderate-to-severe pSS, despite evidence of biological activity.


1974 ◽  
Vol 83 (3) ◽  
pp. 370-378 ◽  
Author(s):  
Alfred E. Jones ◽  
Alvin L. Larson ◽  
Ralph D. Powell ◽  
Gerald S. Johnston ◽  
Robert I. Henkin

Patients with Sjögren's syndrome accumulated abnormal amounts of 99mtechnetium pertechnetate in the region of the nose during isotopic salivary flow studies. It was concurrently and independently observed that many patients with Sjögren's syndrome had hyposmia and pathological changes in the nasal mucous membranes. Fourteen patients with Sjögren's syndrome were studied for the relationship of the above observations and the nasal accumulation of radionuclide was compared with a control group of 16 subjects. Eleven of 14 patients with Sjögren's syndrome (78%) had nasal accumulation of the radionuclide; 14 had hyposmia and 13 of 14 had chronic inflammation of the nasal mucous membrane. One of 16 controls (6%) localized radionuclide in the nasal region. Results suggest that hyposmia, inflammatory changes in the nasal mucous membrane, and nasal accumulation of 99mtechnetium pertechnetate are interrelated aspects of Sjögren's syndrome.


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