Rate, risk factors and causes of mortality in patients with Sjögren’s syndrome: a systematic review and meta-analysis of cohort studies

Singh, Anshu; Singh, Siddharth; Matteson, Eric L.

doi:10.1093/rheumatology/kev354

Cited by 86 publications

(85 citation statements)

References 26 publications

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“…It is not characterized by the types of disease flares seen in systemic lupus or rheumatoid arthritis, but patients may report periods of worsening sicca or fatigue. There is no increase in overall mortality according to a recent meta-analysis, but patients with specific extra-glandular manifestations, including those with vasculitis, cryoglobulinemia, pulmonary disease and lymphoma, have been identified as having higher mortality rates [45, 46]. …”

Section: Sjögren Syndromementioning

confidence: 99%

Sjögren Syndrome and Other Causes of Sicca in Older Adults

Baer

2017

Clinics in Geriatric Medicine

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Synopsis Dry eye and dry mouth symptoms are each reported by up to 30% of persons over the age of 65 years, particularly in women. Multiple factors contribute to these symptoms, but medication side effects are the most common. The evaluation of these symptoms requires measures of ocular and oral dryness, some of which can be done by the geriatrician in a clinic setting. Sjögren syndrome is the prototypic disease associated with dryness of the eyes and mouth and predominantly affects women in their peri- and postmenopausal years of life. The diagnosis requires substantive evidence of autoimmune-induced inflammation targeting the salivary or lacrimal glands. With current classification criteria, the diagnosis can only be established if the patient has antibodies to the SSA (Ro) and/or SSB (La) antigens or a labial gland biopsy showing a characteristic pattern and severity of inflammation, in concert with at least one measure of ocular or oral dryness. A diagnosis is important, since this disease is associated with a substantial increased risk of lymphoma and other forms of morbidity. In addition to topical treatment of the mucosal dryness, patients with Sjögren's syndrome may require treatment with systemic immunomodulatory and immunosuppressive agents to manage a variety of “extraglandular” manifestations.

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Section: Sjögren Syndromementioning

confidence: 99%

Sjögren Syndrome and Other Causes of Sicca in Older Adults

Baer

2017

Clinics in Geriatric Medicine

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“…Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in the ambit of almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure - in most cases requiring ventilatory support - as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event [1,2,3,4,5,6,7,8,9,10]. …”

Section: Introductionmentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

Respiration

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Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure requiring ventilatory support as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event. In CTDs, acute respiratory events may be related to any respiratory compartment including the airways, lung parenchyma, alveolar capillaries, lung vessels, pleura, and ventilatory muscles. Acute respiratory events may also precipitate disease-specific extrapulmonary organ involvement such as aspiration pneumonia and lead to digestive tract involvement and heart-related respiratory events. Finally, antirheumatic drug-related acute respiratory toxicity as well as lung infections related to the rheumatic disease and/or to immunosuppression complete the spectrum of acute respiratory events. Overall, in CTDs the lungs significantly contribute to morbidity and mortality, since they constitute a common site of disease involvement; a major site of infections related to the ‘mater' disease; a major site of drug-related toxicity, and a common site of treatment-related infectious complications. The extreme spectrum of the abovementioned events, as well as the ‘vicious' coexistence of most of the aforementioned manifestations, requires skills, specific diagnostic and therapeutic means, and most of all a multidisciplinary approach of adequately prepared and expert scientists. Avoiding lung disease might represent a major concern for future advancements in the treatment of autoimmune disorders.

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“…All patients achieved clinical remission after IBCTD and no flare were observed during the one-year follow-up. IBCDT resulted in a decrease of median global SLEDAI from 14.6 [11-23] to 4 [1][2][3][4][5] at 12 months (p<0.01) Conclusions: Our results suggest a phenotypic change of peripheral T lymphocytes as a result of B cells depletion obtained with IBCTD. Treg considered being essential in the maintenance of peripheral self-tolerance, progressively increased after B cell depletion.…”

mentioning

confidence: 62%

“…Elevated levels of IP-10 levels were found in patients with pSS but did not show a difference between glandular and extraglandular manifestation nor did it correlate with the disease activity. Conclusions: SIGLEC1 is up-regulated in pSS patients with a known higher mortality and morbidity (5,6), which therefore may be of value for a risk stratification, differential therapeutic approaches and guiding clinical decision making in pSS.…”

Section: Discussionmentioning

confidence: 99%

FRI0348 Feasibility and Validity of Patient Reported Outcome Measurement Information System (PROMIS) in Systemic Lupus Erythematosus

Kasturi¹,

Burket

Berman³

et al. 2016

Ann Rheum Dis

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BackgroundAccurate measurement of patient reported outcomes (PROs) is important in systemic lupus erythematosus (SLE), a heterogeneous disease in which similar symptoms can have disparate impact across patients. PROMIS offers dynamic computer adaptive tests (CATs) to precisely and efficiently measure PROs in a variety of relevant domains.ObjectivesThe aims of this study were to: 1) assess the feasibility of administering PROMIS CATS serially to SLE outpatients 2) correlate PROMIS CATs with legacy PRO measures, SLE disease activity and organ damage 3) assess retest reliability of PROMIS CATs.MethodsAdults meeting American College of Rheumatology SLE classification criteria were recruited from a SLE Center of Excellence. Subjects completed the Short Form-36 (SF-36), LupusQoL-US, and selected PROMIS CATs. SLE disease activity, flare, and damage were evaluated with the SELENA-SLEDAI and SLICC-ACR damage index. PROMIS domains were compared with disease activity, damage, and similar domains in legacy instruments using Spearman correlations. Retest reliability was evaluated among subjects reporting stable SLE activity at two assessments one week apart using intraclass correlation coefficients (ICC).ResultsOf 198 patients approached, 163 (82%) completed at least one assessment, 130 (80%) completing it remotely. 138 (85%) completed a retest. Most PROMIS domains showed moderate to strong correlations with similar domains in both legacy instruments (Figure 1). However, correlations between PROMIS and the physician global assessment, SELENA-SLEDAI, and SLICC-ACR damage index were generally weak and statistically insignificant. PROMIS CAT retest ICCs ranged from 0.74 to 0.89.ConclusionsTo our knowledge, these data are the first to show that PROMIS CATs can be successfully administered to a diverse cohort of SLE patients at the point of care or remotely, and are valid and reliable for many SLE relevant domains. Importantly, PROMIS scores did not correlate well with physician-derived measures. This disconnect between objective signs and symptoms and the subjective patient disease experience underscores the crucial need to integrate PROs into clinical care to ensure optimal disease management.AcknowledgementFunding for this study was provided by the Rheumatology Research Foundation Scientist Development Award.Disclosure of InterestNone declared

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Rate, risk factors and causes of mortality in patients with Sjögren’s syndrome: a systematic review and meta-analysis of cohort studies

Abstract: pSS is not associated with an increase in all-cause mortality as compared with the general population. However, a subset of patients with extraglandular involvement, vasculitis, hypocomplementaemia and cryoglobulinaemia may be at increased risk of mortality and require close follow-up.

Cited by 86 publications

References 26 publications

Sjögren Syndrome and Other Causes of Sicca in Older Adults

Sjögren Syndrome and Other Causes of Sicca in Older Adults

Acute Respiratory Events in Connective Tissue Disorders

FRI0348 Feasibility and Validity of Patient Reported Outcome Measurement Information System (PROMIS) in Systemic Lupus Erythematosus

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