Of the 5 papers included in this review, only one study was considered to have low degree of bias. In most of these earlier works, the diagnostic criteria of the primary SS were either not stated or were heterogeneous; the sample size was small and there were no details regarding the baseline data of the participants. Overall, the punctum plugs appeared to be effective for keratoconjunctivitis sicca but they were not regarded as a disease-specific treatment modality.
Other treatment options, including oral lubrication devices, psychodynamic therapy and acupuncture are not proven to be useful in primary SS. A study looking at the efficacy of acupuncture in primary SS is afoot in mainland China. The control group of subjects will receive sham acupuncture, in which the needle will not puncture the skin at all.
The result of the above study is yet to be reported. Artificial tears e. Eye lubricants e. Cyclosporine A and steroid eye drops are used by ophthalmologists as the second line treatment of serious or refractory sicca symptoms of the eyes.
Artificial saliva is the first-line treatment for xerostomia. However, the efficacy of these csDMARDs is only reported in open-label studies but not supported by evidence from large randomized controlled trials. It was concluded that HCQ did not have apparent benefits in the treatment of dry eyes or systemic inflammation in primary SS. The result was different from a previous retrospective open-label study in which HCQ was shown to improve local symptoms, arthralgia, myalgia and erythrocyte sedimentation rate after a mean observation of 3 years. Moreover, the duration of follow-up of this study was much shorter.
Cyclosporine A, on the other hand, is promising for the treatment of primary SS. The reported side effects were consistent with the known toxicity profile of cyclosporine A. Systemic involvement of primary SS is heterogeneous; with virtually any organ system may be involved. Cardiopulmonary, renal and neurological manifestations are usually treated with high-dose glucocorticoids with or without combination with other non-glucocorticoid immunosuppressive agents.
Renal manifestations such as distal renal tubular acidosis have well been described in primary SS. Membranoproliferative glomerulonephritis is the commonest form of glomerulonephritis in primary SS.
Renal tubulo-interstitial disease is also common in primary SS, which is present in approximately two-thirds of patients with kidney dysfunction who have undergone a renal biopsy. As the B cells are central to the pathogenesis of SS, B cell-targeted therapies have been mostly studied in primary SS in the past few years.
A meta-analysis regarding the efficacy rituximab in primary SS was recently published. Among the included studies, only one evaluated lacrimal function, which was measured by lissamine stain test, and ocular symptoms that were reported by a visual analogue scale. Both parameters showed significant improvement after 24 weeks of treatment with rituximab. Sign in. You could not be signed in.
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The Diagnosis and Treatment of Sjögren’s Syndrome ()
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If you believe that this Physiopedia article is the primary source for the information you are refering to, you can use the button below to access a related citation statement. Cite article. Sjogren's syndrome was first described by physician Henrik Sjogren in the early nineteen hundreds to explain the signs and symptoms of a group of women exhibiting chronic arthritis along with extremely dry eyes and dry mouth . It is now understood that Sjogren's syndrome is an autoimmune connective tissue disease in which the body's own immune system attacks moisture producing glands, causing inflammation in addition to reduction in both the quality and quantity of the glands' secretions .
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As observed by Dr. Sjogren, the glands responsible for producing saliva and tears, the salivary and lachrymal glands respectively, are the organs most notably impacted by the disease .
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However, Sjogren's syndrome is a systemic disorder in which many organs may be affected, including kidneys, gastrointestinal tract, blood vessels, respiratory tracts, liver, pancreas, and central nervous system . Additionally, it is considered a rheumatic disease, like rheumatoid arthritis or lupus, and similar to these diseases Sjogren's syndrome can cause inflammation in joints, muscles, skin, and other organs .
Sjogren's syndrome is the second most prevalent autoimmune rheumatic disease. In , it was estimated that 1. Statistics from rheumotology clinics indicate that Sjogren's syndrome affects 0. Although, it can affect individuals of all genders and ages, including children, symptoms usually occur in women between the ages of 45 and 55 years old . In fact, it is estimated that nine times as many women are affected when compared to men . Among individuals with rheumatoid arthritis or systemic sclerosis, thirty percent have histological evidence of Sjogren's syndrome .
While these are the hallmark signs of Sjogren's syndrome, 40 to 60 percent of individuals with primary SS will develop extraglandular problems . A patient may present with a number of other complaints including:. Approximately half of patients who have been diagnosed with Sjogren's Syndrome have also been diagnosed with rheumatoid arthritis, lupus, or some other connective tissue disorder .
Fewer people may also experience Sjogren's in association with lymphoma . As more research is developed on the topic, more and more co-morbities are being associated with SS. Recent studies suggest that clinical depression is also common among individuals diagnosed with this disease . Secondary Sjogren's is a form of the syndrome which develops after the presentation of a primary disease, usually another autoimmune connective tissue disease .
Common primary diseases associated with Sjogren's Syndrome are listed in the table below. There is no cure for Sjogren's syndrome, therefore its symptoms are managed through medications, both prescription and over the counter. This table was created using information from the National Institute of Arthritis and Musculoskeletal and Skin Diseases  and PubMed Health  , see references below.
Because dry eyes and dry mouth are common symptoms and side affects of a number of diseases and treatments, including radiation to the head and neck, the physician will commonly perform a battery of additional tests to confirm no other causes. These tests may include:.
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Many of these criteria for classification involve test explained above. The below tables display the classification critera for Sjogren's syndrome as proposed by the American-European Consensus Group:. The above table was adapted from Table 2 in Vitali C, et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group.
Ann Rheum Dis ; See references below. The above table was adapted from Table 3 in Vitali C, et al. Regulation of the salivary glands by the nervous system is impaired, and the secretory acinar apparatus displays structural abnormalities . The acinus is the terminal end of the exocrine gland where the secretions are produced.
The membrane of this structure lacks the laminin alpha-1 chain, which may explain this organ's inability to induce differentiation of the stem cells into acinar cells . Evidence in the pattern of cases suggest that the cause of Sjogren's syndrome has a genetic component . Recent research has found that the same gene variant commonly present in individuals with other connective tissue diseases, such as RA and lupus is also associated with Sjogren's syndrome .