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Autoimmune epithelial inflammation characterized by lymphocytic infiltration of exocrine glands and epithelial cells in multiple sites.
A systemic chronic inflammatory disorder with lymphocyte infiltrates in exocrine organs.
Characterized by an inflammatory infiltrate of exocrine glands, particularly the lachrymal and salivary glands.
Primary Sjogren’s syndrome is characterized by dryness, pain and fatigue.
A systemic autoimmune disease, that presents mainly with dry mouth and dry eyes.
Associated with marked fatigue and widespread pain affecting the quality of life of patients.
Systems are non specific and can occur with many other illnesses.
Systemic manifestations occur in approximately one third of patients.
A systemic autoimmune disease characterized mainly by the presence of keratoconjunctivitis sicca and xerostomia.
Patients also may experience inflammation in any other organ and include: joints, muscles, nerves, lungs, gastrointestinal tract, thyroid, and lymph nodes.
Patients may experience fatigue, disturbed sleep, and memory problems.
Autoimmune epithelial inflammation characterized by lymphocytic infiltration of exocrine glands and epithelial cells in multiple sites.
A systemic chronic inflammatory disorder with lymphocyte infiltrates in exocrine organs.
Characterized by an inflammatory infiltrate of exocrine glands, particularly the lachrymal and salivary glands.
The key features include lymphocytic infiltration of the exocrine glands,predominantly the lacrimal and salivary glands, the release of inflammatory mediators and cytokines, and the production of autoantibodies.
Primary Sjogren syndrome is characterized by dryness, pain and fatigue.
A systemic autoimmune disease, that presents mainly with dry mouth and dry eyes.
Associated with marked fatigue and widespread pain affecting the quality of life of patients.
Has distressing physical, emotional, and financial impacts on patients.
In a 2017 survey revealed that SS adds significant burdens to patients� lives, relationships with friends, families, and sexual partners, and 28% of patients had to stop work, reduce their working hours, or take a lighter job due to their disease.
Systems are non specific and can occur with many other illnesses.
Systemic manifestations occur in approximately one third of patients.
Up to one-third of patients experience extraglandular inflammation, including polysynovitis, neuropathy and inflammtory lung disease.
Extra glandular manifestations are common and lymphoma is a recognized complication.
Estimated 0.5 million-3 million affected people in the United States.
The mean age of onset is in the 4th or 5th decade.
Prevalence of approximately 0.5% in the general population.
Affects as many as 3% of women aged 50 years or older.
There is a female to male ratio of 9 to 1.
Males present with a more severe form of the disease.
Primarily, affects perimenopausal women.
Primary Sjogren syndrome refers to the onset of sicca symptoms in a previously healthy individual.
Management includes controlling the sicca symptoms using substitute topical agents, and utilizing corticosteroids and immunosuppressive agents for extra glandular manifestations of the disease.
Has many extra glandular features including arthralgias, arthritis, myalgias, gastrointestinal disease, Raynaud’s disease, anemia, leukopenia, lymphadenopathy, neuropathy, vasculitis renal tubular acidosis and lymphoma.
Primary disease occurs in the absence of an underlying rheumatic disease, and can occur as a solitary condition.
Secondary syndrome reflects the presence of an underlying rheumatic disease, such as rheumatoid arthritis, systemic lupus erythematous or scleroderma.
Chronic disorder characterized by dry mouth and dry eyes.
Typically 8-10 years from initiation of symptoms to diagnosis.
Dental complaints are common.
Llife-threatening manifestations are infrequent.
Anti-Ro/SS-A antibodies are detected in 90% and anti-SS-B antibodies are present in 60% of patients with Sjogren syndrome.
20% have a MALT lymphoma and chronic parotid enlargement.
Rituximab has been shown to improve some extra glandular features with vasculitis, neuropathy, glomerulonephritis and arthritis and should be considered in refractory patients.
Corticosteroids and cyclophosphamide, possibly with plasma exchange should be utilized in patients with a rapidly progressive glomerulonephritis, neuropathy, interstitial lung disease, or myelitis, or with severe systemic vasculitis.
Use of preservative-free substitutes recommended for xerophthalmia.
Ocular lubricating ointments are suggested for nocturnal use.
Topical use of 0.5% cyclosporine BID is suggested for patients with moderate to severe dry eye disease.
Refractory dry eye may respond to topical nonsteroidal anti-inflammatory drugs or glucocorticoids.
For mild-moderate, dry mouth saliva replacement therapy and sugar-free gum may be helpful.
Oral pilocarpine (5 mg every six hours) and cervimeline (30 mg every eight hours) are treatments of choice for patients with residual salivary gland function.
N-acetylcysteine is an alternative therapy for dry mouth.
Hydroxychloroquine is an immunomodulatory drug most frequently used for primary Sjogren’s syndrome for patients with fatigue, arthralgia, and myalgia rather than severe systemic manifestations.
Hydroxychloroquine use in primary Sjogren’s syndrome compared to placebo does not improve symptoms during 24 weeks of treatment (JOQUER Trial), suggesting previous trials have overestimated the therapeutic efficacy of the drug.
Systemic symptoms are usually treated with corticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil or rituximab.
Etiology and pathophysiology are complex and are not well understood.
Genetic, environmental, and hormonal factors have been postulated to establish the condition
The second most prevalent chronic rheumatic autoimmune disease, after rheumatoid arthritis.
Clinical manifestations of the disease can be divided into glandular and extraglandular features.
In a retrospective study of 80 patients with primary SS followed for a median of 7.5 years all patients experienced dry eyes and dry mouth, and this was the only manifestation in 31% of the patients, while extraglandular involvement occurred in 25% of the patients.
In this study 2.5% developed non-Hodgkin lymphoma (NHL).
Glandular manifestations include: dry mouth, dry eyes, salivary and lacrimal gland enlargement, vaginal dryness and associated dyspareunia.
Multiple extraglandular organs may be affected in patients with SS, including the skin, joints, blood vessels, lungs, kidneys, gynecologic system, and nervous system.
Glandular manifestations occurs in approximately 50% of patients during the course of the disease.
Keratoconjunctivitis sicca patients complain of of a sensation of foreign body and burning in the eyes.
Xerostomia can cause in difficulty swallowing and talking.
When oral health is significantly affected; they are 10 times prone to dental caries and recurrent oral Candida albicans infection compared with the general population.
Approximately 5% to 10% of all primary SS patients eventually develop NHL of B-cell lineage, arising from mucosal associated lymphoid tissue, lymph nodes, and exocrine glands, particularly the parotid gland.
Patients experience fatigue and sleep disorders due to the disruption caused by xerostomia stimulating polydipsia with resultant polyuria.
Depression occurs 5 times more often compared with healthy control patients.
33% to 49% have depression, and 22% to 33% of patients with primary SS have fibromyalgia.
No single diagnostic test for SS exists.
The diagnosis is made by the presence of complemental clinical and laboratory findings after the exclusion of all the other possible diagnoses.
Many patients do not require further tests beyond basic blood investigations and an ocular examination.
The diagnostic evaluation starts with a thorough history and physical examination of patients with suspected cases: includes those with persistent dry eyes and/or dry mouth on a daily basis for 3 or more months, parotid gland enlargement, and recurrent dental caries.
Laboratory might reveal anemia, leukopenia, and thrombocytopenia an elevated erythrocyte sedimentation rate; hyperglobulinemia, and urinalysis with hematuria and proteinuria.
All patients should be evaluated for systemic autoimmune abnormalities.
All patients are evaluated for an objective marker of dry eyes.
Sjogren’s syndrome treated with drugs compared to placebo does not improve symptoms during 24 weeks of treatment (JOQUER Trial), suggesting previous trials have overestimated the therapeutic efficacy of the drug.
Systemic symptoms are usually treated with corticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil or rituximab.