Diagnosis and management of sjogren syndrome paul kruszka, lcdr, usphs, u. Sjogrens syndrome is a chronic autoimmune disease in which the bodys immune system inappropriately attacks selftissues, particularly the glands that produce moisture in the eyes, the mouth, and elsewhere in the body. Aetiology, epigenetics, genetics, immunological factors, nonimmunological factors, pathogenesis, sjogren s syndrome. Update on pathogenesis of sjogrens syndrome request pdf. Sjogrens syndrome ss is a chronic autoimmune disorder. Sjogren s syndrome is a common autoimmune disease that presents with sicca symptoms and extraglandular features.
Many systemic aspects of ss are described, including b cell lymphoma in addition to pulmonary and renal pathoses 24. We examined whether mast cells play a critical role in immunemediated inflammation and fibrosis in patients with sjogrens syndrome. Similar to other autoimmune disorders, a strong interferon ifn signature is present among subsets of pss patients. Most often, the cooccurring autoimmune disease is rheumatoid arthritis. Sjogrens syndrome can be classified into two forms. Most individuals with sjogren syndrome present with sicca symptoms, such as xerophthalmia dry eyes, xerostomia dry mouth, and parotid gland enlargement, which is seen in the image below. Dryness may also involve the nose, pharynx, larynx, and tracheobronchial tree. A clinical picture of dry eye and dry mouth with the histological counterpart of focal lymphocytic sialoadenitis, usually detected in minor salivary glands, is considered the hallmark of sjogrens syndrome. Examples of these other autoimmune diseases that commonly can coassociate with sjogren s are rheumatoid arthritis, primary biliary cirrhosis, and lupus. Involvement of aquaporins in the pathogenesis, diagnosis and.
Primary sjogrens syndrome pss is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. Aug 30, 2011 the pathogenesis and natural history of sjogren s syndrome the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Request pdf pathogenesis of sjogrens syndrome to summarize recent developments in our understanding of the pathogenesis of sjogrens syndrome with a focus on the relationship between. Other symptoms can include dry skin, vaginal dryness, a chronic cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. It is the second most common multisystem autoimmune disease after rheumatoid arthritis, and is characterised by eye and mouth dryness and lymphocytic infiltration of the salivary glands, which are easily accessible and thus facilitate. Sjogren syndrome sjs, ss is a longterm autoimmune disease that affects the bodys moistureproducing glands. The pathophysiology of ss has not yet been fully deciphered. Sjogrens syndrome, chronic inflammatory disorder characterized by severe dryness of the eyes and the mouth that results from a diminution in secretion of tears and saliva. Classically it has been postulated that sicca symptoms in ss patients are a double step process whereby lymphocytic infiltration of lacrimal and. It mainly affects the glands that produce saliva and tears, leading to dry eyes and dry mouth. Involvement of mast cells in the pathogenesis of sjogrens.
The vascularity and secretory function of exocrine glands affected in sjogrens syndrome are innervated by the sympathetic and parasympathetic branches of the ans. Recent advances in understanding molecular mechanisms in the pathogenesis and antibody profile of sjogrens syndrome. The purpose of this study is to learn more about sjogren s syndrome. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx andor vagina. Sjogrens syndrome and systemic lupus erythematosus. Despite extensive study of the underlying cause of sjogrens syndrome, the pathogenesis remains obscure. Hypofunction of the salivary glands is the major cause underlying symptoms of a dry mouth xerostomia, occurring in over 90%. As a result of the ease and safety of minor salivary gland biopsy, sjogren s syndrome serves as a prototype model to study the immunopathogenic features of a human organspecific autoimmune disease. This suggests that the immune system, which normally functions to protect the body against cancers and infections, is reacting against its own tissue. It is characterized by dysfunction and destruction of the exocrine glands associated with lymphocytic infiltrates and immunological. Klinefelter s syndrome 47,xxy is in excess among men with sjogren s syndrome. Sjogrens syndrome and lupus hospital for special surgery. Sjogrens syndrome ss is a systemic autoimmune disease affecting primarily the exocrine glands.
Request pdf update on pathogenesis of sjogren s syndrome sjogrens syndrome is a common autoimmune disease that presents with sicca symptoms and extraglandular features. Pathophysiology of sjogrens syndrome sciencedirect. Sjogrens syndrome ss is a systemic autoimmune disease that has several oral manifestations, with reduced salivary flow being the most prevalent. The oral cavity is the microbial habitat closest to the salivary glands the most affected tissue in pss. Sjogrens syndrome is a disorder of the immune system the bodys defence system against infection. Pulmonary manifestations of sjogrens syndrome european. The immune factors involved in the pathogenesis, diagnosis, and. There is an emerging view that mast cells may play a pivotal role in several inflammatory and autoimmune diseases.
This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx andor vagina 1. Listing a study does not mean it has been evaluated by the u. What have we learned from clinical trials in primary sjogrens syndrome about pathogenesis. Aetiology, epigenetics, genetics, immunological factors, nonimmunological factors, pathogenesis, sjogrens syndrome. Laboratory of immunology, brest university medical school hospital, bp 824, f 29609 brest, france.
Many patients develop sjogrens syndrome as a complication of another autoimmune disease, such as rheumatoid arthritis or lupus. Apr 26, 2012 immunopathologic differences of sjogrens syndrome versus sicca syndrome in hcv and hiv infection. Despite this, its exact aetiology and pathogenesis is still unclear. Involvement of aquaporins in the pathogenesis, diagnosis. Is the oral microbiome involved in the pathogenesis of. Those affected are also at an increased risk 5% of lymphoma. Sjogren syndrome is a systemic autoimmune disorder nocturne g, mariette x. Abstract sjogrens syndrome is a chronic autoimmune disease that affects many. Symptoms vary in type and intensity, but many people with sjogrens are able to live normal lives. Sjogren s syndrome is a disease that affects about 14 million americans. Patients with secondary sjogren s syndrome also have signs and symptoms associated with rheumatic disorder. Even though the mechanism of its pathology and progression has been researched ever since its discovery, the roles of different parts of immune system remain inconclusive. Pathogenesis the pathogenesis of sjogren syndrome is obscure.
Introduction sjogrens syndrome ss is a relatively common systemic autoimmune disease characterized by lymphocytic infiltration of the secretory glands. In 1936, duke elder honored sjogren by naming the disease sjogrens syndrome. Incidence has recently been estimated at between 3. Primary sjogrens syndrome was originally described in 1926 by g ougerot. There is no straightforward and simple theory for the pathogenesis and. Sjogrens syndrome american college of rheumatology. Other individuals with secondary sjogrens may have lupus, scleroderma.
Sjogrens syndrome causes increased levels of il1ra in csf suggesting increased activity in the interleukin 1 system and that this is associated with increased fatigue through cytokine induced sickness behavior2. The condition often accompanies other immune system disorders, such as rheumatoid arthritis and lupus. Epidemiology and pathogenesis of sjogrens syndrome request pdf. Impact of interleukin21 in the pathogenesis of primary sjogrens syndrome. B lymphocyte, sjogrens syndrome, b cell activating factor, ectopic germinal centers, t cells, pathogenesis. White blood cells attack the bodys tear and saliva glands, which.
Environmental factors involved in the pathogenesis of primary sjogrens syndrome pss are still largely unknown. However, patients often have evidence of systemic autoimmunity, and they are at markedly increased risk for the development of non hodgkins lymphoma. As a result of the ease and safety of minor salivary gland biopsy, sjogrens syndrome serves as a prototype model to study the immunopathogenic features of a human organspecific autoimmune disease. People with this disease have abnormal proteins in their blood. Maehara et al present an interesting study on interleukin21 contributes to germinal centre formation and immunoglobulin g4 production in igg4related dacroadenitis and sialoadenitis, socalled mickuliczs disease md. Reviewing primary sjogrens syndrome international journal of. Blymphocytes govern the pathogenesis of sjogrens syndrome volume.
Sjogren syndrome ss is a systemic autoimmune disease that initially targets primarily the lacrimal and salivary glands, resulting in keratoconjunctivitis sicca kcs dry eye disease andor stomatitis sicca dry mouth disease. Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies, and lymphomas. Sjogrens syndrome ss is an autoimmune disorder characterised by. Sjogren s syndrome ss is a systemic autoimmune disease that has several oral manifestations, with reduced salivary flow being the most prevalent. The cause of sjogren s syndrome is unknown, but inflammation plays an important role. Ectopic expression of the b cellattracting chemokine bca1 cxcl on endothelial cells and within lymphoid follicles contributes to the establishment of germinal centerlike structures in sjogrens syndrome. Autonomic nervous system ans abnormalities are common in sjogrens syndrome 28,29,30,31 and may play an etiologic role in its pathogenesis.
Sjogrens syndrome can present in a heterogeneous manner with symptoms varying from systemic features such as unexplained fever, weight loss and neurological manifestations to the more typical symptoms of dry mucus membranes. Advances in understanding the pathogenesis of primary sjogrens syndrome. You may have dryness in other places that need moisture, such as your nose, throat, and skin. Pdf what have we learned from clinical trials in primary. Pathogenesis of sjogrens syndrome is believed to be multifactorial. In sjogrens syndrome, the mucous membranes and moisturesecreting glands of your eyes and mouth are usually affected first resulting in decreased tears and saliva. However, the role of mast cells in sjogrens syndrome remains unclear. Klinefelters syndrome 47,xxy is in excess among men with sjogrens syndrome. The labial salivary biopsies lsb of 15 ss patients. Pdf sjogrens syndrome is a chronic inflammatory disease of the lacrimal and salivary gland with subsequent keratoconjunctivitis sicca and. Sjogrens disease epidemiology, etiology, pathogenesis. Highlights sjogrens syndrome is an autoimmune disease characterized by dysfunction and inflammation of the salivary and lacrimal glands the pathogenesis of sjogrens syndrome has not been elucidated, but the role of viruses has been suggested.
The immune factors involved in the pathogenesis, diagnosis. Sjogrens syndrome ss is a systemic, autoimmune disorder characterized by salivary insufficiency and lymphocytic infiltration of the exocrine glands. Sjogren syndrome symptoms, diagnosis and treatment bmj. Sjogrens syndrome is a relatively common condition that mainly affects the eyes and salivary glands, but can affect different parts of the body. Sjogrens syndrome is an autoimmune condition that can occur at any age, but is most common in older women. Sjogren s syndrome ss is a systemic, autoimmune disorder characterized by salivary insufficiency and lymphocytic infiltration of the exocrine glands. Populationbased and samplebased studies estimate its prevalence at 43 and 282 per 100 000 inhabitants respectively, the former probably being. In sjogrens syndrome, it attacks the glands that make tears and saliva. The pathogenesis and natural history of sjogren s syndrome the safety and scientific validity of this study is the responsibility of the study sponsor and investigators.
Sjogren s syndrome causes increased levels of il1ra in csf suggesting increased activity in the interleukin 1 system and that this is associated with increased fatigue through cytokine induced sickness behavior2. Sjogrens syndrome is an autoimmune condition, which means it occurs as a result of a malfunctioning immune system. Sjogrens syndrome is a common autoimmune disease that presents with sicca symptoms and extraglandular features. Ss is only one of the many dry eyedry mouth diseases, but it is considered to be among the most severe. Patients with secondary sjogrens syndrome also have signs and symptoms associated with rheumatic disorder. The pathogenesis and natural history of sjogren s syndrome. It was first described by henrik sjogren in 1933, who was an ophthalmologist. Pathogenesis of sjogrens syndrome pubmed central pmc. Unraveling the pathophysiology of sjogren syndrome.
Sjogrens syndrome ss is a chronic, multisystem inflammatory disorder characterized by diminished lacrimal and salivary gland function. This means that your immune system attacks parts of your own body by mistake. Sjogrens syndrome was in 1933 by swedish eye specialist henrik sjogren the condition causes antibodies to attack the bodys moistureproducing glands, leading to dryness due to lack of secretions. Classically it has been postulated that sicca symptoms in ss patients are a double step process whereby lymphocytic infiltration of. Blymphocytes govern the pathogenesis of sjogrens syndrome.
Another study found an excess of klinefelter men in sjogren syndrome compared with klinefelter in normal control men and men with rheumatoid arthritis. Research article open access impact of interleukin21 in. The distinct pathogenesis of igg4 mickuliczs disease and. Primary sjogrens syndrome pss is a systemic autoimmune disease, characterized by. Sjogrens syndrome is considered an autoimmune disease characterized by chronic inflammation involving the exocrine glands.
Hlab8 dr3,although dr4 is more closely associated with syndrome occuring with raynauds phenomenon antibodies to the ro antigen occur in excess in relatives of pts with sjogrens syndrome. These lymphocytes can infiltrate a wide range of glands and target organs not just the exocrine glands. In 1932, the danish ophthalmologist henrik sjogren reported the triad of keratoconjunctivitis sicca kcs, xerostomia, and rheumatoid arthritis describing clinical and histological findings in 19 women, and then sjogren introduced the term keratoconjunctivitis sicca for this syndrome, to distinguish it from dry eyes caused by lack of vitamin a xerophthalmia. Epidemiology and pathogenesis of sjogrens syndrome. The cause of sjogrens syndrome is not known, but it is an autoimmune disorder. May 20, 2019 sjogrens syndrome ss is a chronic autoimmune disease that classically affects the lacrimal and salivary glands resulting in dry eyes and dry mouth and can affect almost any organ system in the body including the lungs, kidney, and central nervous system.
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