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