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. Many systemic aspects of ss are described, including b cell lymphoma in addition to pulmonary and renal pathoses 24. 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. 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. Aetiology, epigenetics, genetics, immunological factors, nonimmunological factors, pathogenesis, sjogrens syndrome. Research article open access impact of interleukin21 in. And even from the first reports, the hallmark, which maintains true to today, is an infiltration of target exocrine glands by lymphocytes. Sjogren s syndrome is a common autoimmune disease that presents with sicca symptoms and extraglandular features. Laboratory of immunology, brest university medical school hospital, bp 824, f 29609 brest, france. Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies, and lymphomas. We examined whether mast cells play a critical role in immunemediated inflammation and fibrosis in patients with sjogrens syndrome. Many patients develop sjogrens syndrome as a complication of another autoimmune disease, such as rheumatoid arthritis or lupus. Classically it has been postulated that sicca symptoms in ss patients are a double step process whereby lymphocytic infiltration of. In 1936, duke elder honored sjogren by naming the disease sjogrens syndrome.
Blymphocytes govern the pathogenesis of sjogrens syndrome volume. It mainly affects the glands that produce saliva and tears, leading to dry eyes and dry mouth. 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. Pdf sjogrens syndrome is a chronic inflammatory disease of the lacrimal and salivary gland with subsequent keratoconjunctivitis sicca and. Update on pathogenesis of sjogrens syndrome request pdf. Involvement of aquaporins in the pathogenesis, diagnosis and. Sjogrens syndrome is a relatively common condition that mainly affects the eyes and salivary glands, but can affect different parts of the body. 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. Is the oral microbiome involved in the pathogenesis of. Klinefelter s syndrome 47,xxy is in excess among men with sjogren s syndrome. Listing a study does not mean it has been evaluated by the u.
However, the role of mast cells in sjogrens syndrome remains unclear. The vascularity and secretory function of exocrine glands affected in sjogrens syndrome are innervated by the sympathetic and parasympathetic branches of the ans. Pathophysiology of sjogrens syndrome sciencedirect. The cause of sjogren s syndrome is unknown, but inflammation plays an important role. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx andor vagina. Diagnosis and management of sjogren syndrome paul kruszka, lcdr, usphs, u.
Sjogren syndrome is a systemic autoimmune disorder nocturne g, mariette x. It is characterized by dysfunction and destruction of the exocrine glands associated with lymphocytic infiltrates and immunological. Patients with secondary sjogren s syndrome also have signs and symptoms associated with rheumatic disorder. The immune factors involved in the pathogenesis, diagnosis. Involvement of aquaporins in the pathogenesis, diagnosis. Sjogrens syndrome ss is a chronic, multisystem inflammatory disorder characterized by diminished lacrimal and salivary gland function. Pdf what have we learned from clinical trials in primary.
Hypofunction of the salivary glands is the major cause underlying symptoms of a dry mouth xerostomia, occurring in over 90%. Sjogrens syndrome can be classified into two forms. Sjogrens syndrome is an autoimmune condition, which means it occurs as a result of a malfunctioning immune system. This suggests that the immune system, which normally functions to protect the body against cancers and infections, is reacting against its own tissue. Impact of interleukin21 in the pathogenesis of primary sjogrens syndrome. Sjogrens syndrome ss is a systemic, autoimmune disorder characterized by salivary insufficiency and lymphocytic infiltration of the exocrine glands. Sjogren s syndrome ss is a systemic autoimmune disease that has several oral manifestations, with reduced salivary flow being the most prevalent. Sjogren syndrome sjs, ss is a longterm autoimmune disease that affects the bodys moistureproducing glands. B lymphocyte, sjogrens syndrome, b cell activating factor, ectopic germinal centers, t cells, pathogenesis. 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. Recent advances in understanding molecular mechanisms in the pathogenesis and antibody profile of sjogrens syndrome.
Dryness may also involve the nose, pharynx, larynx, and tracheobronchial tree. 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. Aetiology, epigenetics, genetics, immunological factors, nonimmunological factors, pathogenesis, sjogren s syndrome. 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. Primary sjogrens syndrome pss is a systemic autoimmune disease, characterized by. Sjogrens syndrome ss is a chronic autoimmune disorder. Primary sjogrens syndrome was originally described in 1926 by g ougerot. Pulmonary manifestations of sjogrens syndrome european. 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. The purpose of this study is to learn more about sjogren s syndrome. Apr 26, 2012 immunopathologic differences of sjogrens syndrome versus sicca syndrome in hcv and hiv infection. Environmental factors involved in the pathogenesis of primary sjogrens syndrome pss are still largely unknown.
Sjogrens syndrome and lupus hospital for special surgery. Sjogrens syndrome and systemic lupus erythematosus. Similar to other autoimmune disorders, a strong interferon ifn signature is present among subsets of pss patients. Sjogrens syndrome is an autoimmune condition that can occur at any age, but is most common in older women. It was first described by henrik sjogren in 1933, who was an ophthalmologist. Request pdf update on pathogenesis of sjogren s syndrome sjogrens syndrome is a common autoimmune disease that presents with sicca symptoms and extraglandular features. 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. Those affected are also at an increased risk 5% of lymphoma. Classically it has been postulated that sicca symptoms in ss patients are a double step process whereby lymphocytic infiltration of lacrimal and.
Pathogenesis of sjogrens syndrome pubmed central pmc. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx andor vagina 1. Despite this, its exact aetiology and pathogenesis is still unclear. Patients with secondary sjogrens syndrome also have signs and symptoms associated with rheumatic disorder. Epidemiology and pathogenesis of sjogrens syndrome. Advances in understanding the pathogenesis of primary sjogrens syndrome. The oral cavity is the microbial habitat closest to the salivary glands the most affected tissue in pss. Sjogrens syndrome american college of rheumatology. Pathogenesis of sjogrens syndrome is believed to be multifactorial. What have we learned from clinical trials in primary sjogrens syndrome about pathogenesis. Sjogrens disease epidemiology, etiology, pathogenesis. 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.
Klinefelters syndrome 47,xxy is in excess among men with sjogrens syndrome. Sjogrens syndrome is a disorder of the immune system the bodys defence system against infection. Sjogrens syndrome ss is a systemic autoimmune disease that has several oral manifestations, with reduced salivary flow being the most prevalent. The labial salivary biopsies lsb of 15 ss patients. Autonomic nervous system ans abnormalities are common in sjogrens syndrome 28,29,30,31 and may play an etiologic role in its pathogenesis. Primary sjogrens syndrome pss is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. People with this disease have abnormal proteins in their blood. Reviewing primary sjogrens syndrome international journal of. These lymphocytes can infiltrate a wide range of glands and target organs not just the exocrine glands. Incidence has recently been estimated at between 3. Involvement of mast cells in the pathogenesis of sjogrens. Symptoms vary in type and intensity, but many people with sjogrens are able to live normal lives.
Abstract sjogrens syndrome is a chronic autoimmune disease that affects many. Introduction sjogrens syndrome ss is a relatively common systemic autoimmune disease characterized by lymphocytic infiltration of the secretory glands. 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. Unraveling the pathophysiology of sjogren syndrome. The immune factors involved in the pathogenesis, diagnosis, and. Sjogren s syndrome is a disease that affects about 14 million americans. Populationbased and samplebased studies estimate its prevalence at 43 and 282 per 100 000 inhabitants respectively, the former probably being. Despite extensive study of the underlying cause of sjogrens syndrome, the pathogenesis remains obscure. Another study found an excess of klinefelter men in sjogren syndrome compared with klinefelter in normal control men and men with rheumatoid arthritis. Sjogrens syndrome ss is an autoimmune disorder characterised by. The condition often accompanies other immune system disorders, such as rheumatoid arthritis and lupus. Sjogrens syndrome ss is a systemic autoimmune disease affecting primarily the exocrine glands.
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. There is no straightforward and simple theory for the pathogenesis and. You may have dryness in other places that need moisture, such as your nose, throat, and skin. Sjogrens syndrome is considered an autoimmune disease characterized by chronic inflammation involving the exocrine glands. 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. 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. Other individuals with secondary sjogrens may have lupus, scleroderma. In sjogrens syndrome, the mucous membranes and moisturesecreting glands of your eyes and mouth are usually affected first resulting in decreased tears and saliva. Pathogenesis the pathogenesis of sjogren syndrome is obscure. This means that your immune system attacks parts of your own body by mistake. Epidemiology and pathogenesis of sjogrens syndrome request pdf.
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. Blymphocytes govern the pathogenesis of sjogrens syndrome. Sjogren syndrome symptoms, diagnosis and treatment bmj. The cause of sjogrens syndrome is not known, but it is an autoimmune disorder. Apr 07, 2020 sjogren syndrome is a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs. Examples of these other autoimmune diseases that commonly can coassociate with sjogren s are rheumatoid arthritis, primary biliary cirrhosis, and lupus. The pathophysiology of ss has not yet been fully deciphered. The pathogenesis and natural history of sjogren s syndrome. 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.
There is an emerging view that mast cells may play a pivotal role in several inflammatory and autoimmune diseases. However, patients often have evidence of systemic autoimmunity, and they are at markedly increased risk for the development of non hodgkins lymphoma. 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. 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.
Sjogren s syndrome ss is a systemic, autoimmune disorder characterized by salivary insufficiency and lymphocytic infiltration of the exocrine glands. 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. 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. Most often, the cooccurring autoimmune disease is rheumatoid arthritis. In sjogrens syndrome, it attacks the glands that make tears and saliva. 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.
White blood cells attack the bodys tear and saliva glands, which. The distinct pathogenesis of igg4 mickuliczs disease and. Sjogren s can occur alone and then is referred to as primary sjogren s or concomitant with another autoimmune disease, then referred to as secondary sjogren s. Pierre youinou, alain saraux and jacquesolivier pers affiliation. 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. Novel insights in pathogenic, clinical and therapeutic aspects.
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