Primary Sjogren syndrome

Sjögren Syndrome Research Group, Department of Systemic Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi i Sunyer, ICMiD, Hospital Clinic, Barcelona, Spain.
BMJ (online) (Impact Factor: 17.45). 06/2012; 344(jun14 1):e3821. DOI: 10.1358/dof.2008.033.09.1249789
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Available from: Antoni Sisó Almirall, Oct 09, 2015
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    • "The main complication of SS is hematological neoplasia. Compared to healthy individuals, patients with SS have a 10 to 50 times higher risk of lymphoma and, according to a large case series, 2% to 9% of patients with SS develop lymphoma [7]. "
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    ABSTRACT: Sjögren's syndrome (SS) is a systemic progressive autoimmune disease characterized by a complex pathogenesis requiring a predisposing genetic background and involving immune cell activation and autoantibody production. The immune response is directed to the exocrine glands, causing the typical 'sicca syndrome', but major organ involvement is also often seen. The etiology of the disease is unknown. Infections could play a pivotal role: compared to normal subjects, patients with SS displayed higher titers of anti-Epstein-Barr virus (EBV) early antigens, but lower titers of other infectious agent antibodies such as rubella and cytomegalovirus (CMV) suggest that some infections may have a protective role against the development of autoimmune disease. Recent findings seem to show that low vitamin D levels in patients with SS could be associated with severe complications such as lymphoma and peripheral neuropathy. This could open new insights into the disease etiology. The current treatments for SS range from symptomatic therapies to systemic immunosuppressive drugs, especially B cell-targeted drugs in cases of organ involvement. Vitamin D supplementation may be an additional tool for optimization of SS treatment.
    BMC Medicine 04/2013; 11(1):93. DOI:10.1186/1741-7015-11-93 · 7.25 Impact Factor
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    ABSTRACT: Sjögren syndrome (SS) is a systemic autoimmune disease that presents with sicca symptomatology of the major mucosal surfaces. The clinical spectrum of this condition often extends to systemic involvement (extraglandular manifestations) and may be complicated by the development of lymphoma. SS is one of the most prevalent autoimmune diseases (with an estimated 0.5–3 million sufferers in the United States), and primarily affects perimenopausal women. When sicca symptoms appear in a previously healthy ­person, the syndrome is classified as primary SS. This chapter summarizes recent work focused on extending and characterizing the acute and/or complex clinical presentations of patients with primary SS, including a wide variety of symptoms and the involvement of internal organs. As a general rule, the management of extraglandular features in primary SS should be targeted to the specific organ involved. The mainstays of such treatment regimens remain glucocorticoids and immunosuppressive agents. Such therapies are more likely to affect favorably the extraglandular manifestations of SS, even though the patients’ most intense complaints often pertain to sicca features. Severe, life-threatening involvement has rarely been reported in primary SS. There are now substantially more data on the outcome of patients with primary SS, which indicate that patients with a predominantly extraepithelial expression (often associated with cryoglobulinemia) should be monitored and managed differently from patients with a predominantly periepithelial or sicca-limited disease. KeywordsCryoglobulinemia •-Interstitial nephritis •-Lymphoma •-Myelitis •-Neuropathy •-Primary biliary cirrhosis •-Pulmonary fibrosis •-Sjögren syndrome •-Vasculitis
    05/2011: pages 45-65;
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    ABSTRACT: Introduction: Primary Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by sicca features and systemic manifestations, and requires a multidisciplinary therapeutic approach. Areas covered: Treatment of sicca manifestations is symptomatic and is based on the administration of topical therapies (saliva substitutes and preservative-free artificial tears). In severe cases of keratoconjunctivitis sicca, topical cyclosporine A may be used. For patients with residual salivary gland function, stimulation of salivary flow with a sialogogue (pilocarpine or cevimeline) is the treatment of choice. The management of extraglandular features must be tailored to the specific organ(s) involved. Hydroxychloroquine may be appropriate for patients with fatigue, arthralgia and myalgia, while glucocorticoids and immunosuppressive agents should be reserved for severe systemic involvement (although no controlled trials in primary SS guide their use). RCTs have demonstrated the lack of efficacy of antitumor necrosis factor agents and promising results for B-cell depleting agents. Expert opinion: The overall low level of evidence in therapeutic studies in primary SS suggests that much larger trials of the most promising therapies are necessary. The use of drugs targeting molecules and receptors involved in the etiopathogenesis of primary SS may open up a new era in the therapeutic management of the disease, but the potential risks and benefits of these agents must be weighed carefully.
    Expert Opinion on Pharmacotherapy 01/2013; 14(3). DOI:10.1517/14656566.2013.767333 · 3.53 Impact Factor
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