Can you die from sjogrens

The average time from onset of symptoms to diagnosis is 3 years. However, treatments may improve various symptoms and prevent complications. Prescription medicines for dry eye and dry mouth are available. A number of over-the-counter products may also be used to alleviate different types of dryness. Immunosuppressive medications are also used to treat the serious internal organ manifestations. Although no one knows exactly how damage occurs, damaged glands can no longer produce tears and saliva, and eye and mouth dryness result.

In Sjogren's syndrome, the mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first — resulting in decreased tears and saliva. Although you can develop Sjogren's syndrome at any age, most people are older than 40 at the time of diagnosis.

The condition is much more common in women. Treatment focuses on relieving symptoms. You have three pairs of major salivary glands — parotid, sublingual and submandibular. Each gland has its own tube duct leading from the gland to the mouth. Sjogren's syndrome is an autoimmune disorder. Your immune system mistakenly attacks your body's own cells and tissues.

Scientists aren't certain why some people develop Sjogren's syndrome. Certain genes put people at higher risk of the disorder, but it appears that a triggering mechanism — such as infection with a particular virus or strain of bacteria — is also necessary. While these are predictors of the development of lymphoid malignancies [ 22 ], these were independently associated with increased mortality.

Third, cardiovascular diseases, solid-organ and lymphoid malignancies and infections are leading causes of mortality. However, two studies observed an increased mortality risk in patients with pSS, and both studies were potentially at risk of selecting patients with severe disease. In their multicentre prospective cohort study, Brito-Zeron et al. Likewise, in their population-based Scottish study, Thomas et al.

Through pooled analysis, we identified several consistent clinical and serological factors associated with increased mortality. The identification of these high-risk baseline factors may be useful in identifying patients who require closer follow-up. Several of these factors, including parotid enlargement, anti-SSA and SSB antibodies and low C3 and C4, have also been associated with increased risk of lymphoma [ 22 ].

In a single-centre retrospective cohort study, Ramos-Casals et al. Hypocomplementaemia was associated with lower 10 year cumulative survival vs normal complement levels; More recently, Brito-Zeron et al. While these variables were independently associated with a 1. We also observed that, in contrast to previous observations, lymphoid malignancies are not the leading cause of mortality in these patients.

Cardiovascular diseases, infections and solid-organ malignancies were leading causes of mortality. However, it is unclear whether pSS confers an increased risk of these or whether these were representative of usual causes of death in an ageing population particularly cardiovascular diseases and malignancies , regardless of pSS disease status. The strengths of this systematic review include a comprehensive and systematic literature search with well-defined inclusion criteria; quantitatively and qualitatively studying all aspects of mortality in pSS, including rate, risk factors and causes of death; and subgroup and sensitivity analyses to evaluate the stability of findings and identify potential factors responsible for inconsistencies.

Our study has several limitations, both at the meta-analysis level and individual study level. At a meta-analysis level, significant heterogeneity was observed in the pooled estimate of risk of mortality. This was explained by two studies with a high risk of selection bias [ 5 , 7 ]. Second, in our attempt to quantify risk factors associated with mortality in pSS, we used available adjusted and unadjusted data from individual studies for pooling.

Unfortunately, multivariate analysis was inconsistently reported with adjustment for different confounding variables across studies, and this somewhat limits the inferences that can be drawn from these observations. We acknowledge that pooling unadjusted estimates is unable to account for confounding factors.

Third, there were insufficient data to pool causes of mortality in patients with pSS, due to inconsistent reporting. It is unclear whether pSS itself increases the risk of cardiovascular and infection-related mortality, based on our analysis.

Fourth, we are unable to exclude publication bias even though there was no funnel plot asymmetry, given the small number of published studies. It is possible that small negative studies are not published. However, this would only bias results towards null and is unlikely to change observations in our analysis. At an individual study level, single referral-centre studies were at higher risk of selection bias, although rates of attrition were low. Finally, there was limited information on the treatment of pSS, and it is unclear how treatment may modify disease course.

In conclusion, patients with pSS are not at increased mortality risk as compared with the general population. However, as others have also observed, a subset of patients with high-risk clinical older males, parotid enlargement, extraglandular involvement or vasculitis and serological markers hypocomplementaemia, cryoglobulinaemia warrant close follow-up [ 25 ].

In addition, cardiovascular diseases, infections and solid-organ and lymphoid malignancies are leading causes of mortality. Although we were unable to examine this issue, it is likely that high inflammatory disease burden and poor disease control may be associated with increased risk of some of these outcomes. Future studies should focus on clinical and serological scoring indices to identify high-risk patients for risk stratification and inclusion in clinical trials of potential disease-modifying therapy.

We wish to thank Larry Prokop, Medical Librarian at the Mayo Clinic Library, for helping in the literature search for this systematic review and meta-analysis. Study concept and design: A. Funding : No specific funding was received from any funding bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article. Disclosure statement : The authors have declared no conflicts of interest.

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Sign In. Advanced Search. Search Menu. Article Navigation. Close mobile search navigation Article Navigation. Volume Article Contents Abstract. Singh , Abha G. Oxford Academic. Siddharth Singh. Eric L. Revision received:.