We describe a 50yearold woman with recurrent neurologic deficits. Extrapyramidal signs are known to be very rare and unusually discovered on early onset in this pathology. The condition typically develops gradually beginning in middle adulthood, but it can occur at any age. Gougerotsjogren syndrome is an autoimmune disorder in which. Some sort of trigger is also needed, such as a viral or bacterial. Very many true or supposedly autoimmune diseases have been reported in association with the gougerotsjogren syndrome. The syndrome can be primary when occurred as an isolated entity or can be considered secondary when associated with any of a wide range of other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematuos, chronic. Cytokines play a major role in tissue destruction caused by autoimmune dysregulation, in sjogren s syndrome ss patients, salivary glands are the target organs for autoimmune tissue damage, in. It has been the subject of some more recent publication over the last decade,28 some suggesting a relationship to lupus erythematosus,9,10 others connective tissue diseases as well as. In a recent survey of ss patients belonging to the french sjogrens. It is characterized by a lymphocytic infiltration of the exocrine glands, also called epitheliitis, resulting in xerostomia and keratoconjunctivitis sicca. Cognitive dysfunction and dementia in primary sjogrens syndrome.
Sjoegren syndrome an overview sciencedirect topics. It most often affects middleaged females with a prevalence of 0. Syndrome society association francaise du gougerotsjogren et des syndromes secs, 81 %. Chronic erythematous candidiasis on the dorsal surface of the tongue, and bilateral angle cheilitis. In sjogrens syndrome, the bodys immune cells go rogue and start attacking various exocrine glands, which are glands that pour their secretions into a.
We present the case of a 17yearold female requiring psychiatric hospitalization for obsessivecompulsive disorder and major depressive disorder with mixed affective features, who was subsequently diagnosed with primary sjogrens syndrome. Rafai ma, boulaajaj fz, moutawakil f, et al 2009 neurological manifestations revealing primitive gougerotsjogren syndrome. Intraductal infusion of steroids in patients with sjogren syndrome to treat painful salivary swelling. Oct 03, 2016 sjogrens syndrome is an autoimmune condition in which there is lymphocytic infiltration of exocrine glands, producing the main symptoms of xerophthalmia dry eyes, xerostomia dry mouth and enlargement of the parotid glands. The symptom complex of dryness of the eyes and mucous membranes with enlargement of the parotid glands in menopausal women was described by henri gougerot in 1925 1 and has been the subject of numerous studies reported by the swedish ophthalmologist, henrik sjogren 19331952. Gougerotsjogren syndrome is an autoimmune disorder in which immunocytes damage the. Our case is unusual because the patient developed the syndrome long after the beginning of the treatment 3 x 3. To the best of our knowledge, this report is the first to describe the association of granulomatous mastitis with sjogrens syndrome. The liver is a common nonexocrine target in primary. Sjogren syndrome affects t million to 4 million people in the united states most are over 40 years old at the time of diagnosis.
With the growing use of the essdai, some domains appear to be more challenging to rate than others. In addition, sjogren syndrome may cause skin, nose, and vaginal dryness. Brain mr imaging abnormalities in primary sjogren syndrome pss are generally discrete white matter lesions. Sjogrens syndrome disease damage index and disease activity index. Sjogren syndrome ss is a chronic autoimmune disorder characterized by inflammatory. Adoue the association of multiple nuclear dots antinuclear antibodies and anti j01 antibodies in a true sjogren syndrome leads us to discuss about. This dermatosis has been associated with many autoimmune disorders, in particular. Primary sjogren syndrome with tumefactive central nervous. It also may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain. It has been the subject of some more recent publication over the last decade, 28 some suggesting a relationship to lupus erythematosus, 9,10 others connective tissue diseases as well as malignant lymphoma. Sjogren syndrome sjs, ss is a longterm autoimmune disease that affects the bodys moistureproducing glands. Sjogren syndrome genetic and rare diseases information. Gougerothouwersjogren syndrome sicca syn swedish ophthalmologist henrik sjogren who first described it 1933.
The symptom complex of dryness of the eyes and mucous membranes with enlargement of the parotid glands in menopausal women was described by henri gougerot in 19251 and has been the subject of numerous studies reported by the swedish ophthalmologist, henrik sjogren 19331952. Gougerot described a few cases of atrophy of the salivary glands with. It is difficult to determine the exact prevalence because the characteristic features of this disorder, dry eyes and dry mouth, can also be caused by many other conditions. Several different genes appear to affect the risk of developing the condition, but specific genes have not been confirmed. Simply having one of these genes does not cause a person to develop the disease. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Frequent references to sjogrens disease, gougerot s disease, and sjogren gougerot or gougerot sjogren disease. Mikulicz 1888 defined as a systemic autoimmune disease caused by an immunemediated inflammation of exocrine glands, and involves salivary, lacrimal and sweat glands, as sicca. Vitali c, palombi g, baldini c, benucci m, bombardieri s, covelli m, et al. Sjogren syndrome likely results from a combination of genetic and environmental factors it appears to be multifactorial. Cytokines play a major role in tissue destruction caused by autoimmune dysregulation, in sjogrens syndrome ss patients, salivary glands are the target organs for autoimmune tissue damage, in.
This inflammation causes dryness of the eyes, xerophthalmia. Sjogren syndrome ss is a chronic autoimmune disorder characterized by inflammatory destruction of the bodys exocrine glands and may be considered primary or secondary, in association with other autoimmune disorders, such as collagen vascular diseases. A diagnosis of pss was made on the basis of clinical features, positive antiro and antila antibodies, abnormal schirmer test findings, and salivary gland. The symptom complex of dryness of the eyes and mucous membranes with enlargement of the parotid glands in menopausal women was described by henri. Sjogrens syndrome ss is a chronic autoimmune disorder. Sjogren syndrome sjogren syndrome keratoconjunctivitis sicca, dryness of mucous membranes, telangiectasias or purpuric spots on the face, and bilateral parotid enlargement, seen in menopausal women. Genetically, some people have an increased risk of developing sjogrens, but scientists believe another factor, like being infected by a certain virus or strain of bacteria, is also required to set the syndrome in motion. 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.
The syndrome of keratroconjunctivitis sicca, xerophthalmia, xerostomia and chronic arthritis is called gougerotsjogrens syndrome 3. In this study, we aimed to explore the cognitive impairment and the correlations with brain mri. Gougerotsjogren syndrome, connective tissue diseases and. Alternatively, you can download the file locally and open with any standalone pdf reader. Primary sjogrens syndrome associated neuropathy canadian. None of these reports describe the incidence of proper gougerot sjogren like syndrome, the time course of the events onset and resolution, or its management. The syndrome can be primary when occurred as an isolated entity or can be considered secondary when associated with any of a wide range of other autoimmune diseases, such as. Pdf first report of granulomatous mastitis associated with. Joint pain and sjogrens syndrome johns hopkins sjogrens center.
Oct 10, 2015 rafai ma, boulaajaj fz, moutawakil f, et al 2009 neurological manifestations revealing primitive gougerotsjogren syndrome. Sjogrens syndrome can also occur as a secondary condition with underlying systemic lupus erythematosus sle or rheumatoid arthritis ra. Gougerotsjogren disease definition of gougerotsjogren. Sjogren 1 first reported a syndrome of xerostomia, keratoconjunctivitis sicca, and rheumatoid arthritis in 1933. There is an increasing awareness that autoimmune diseases can present with neuropsychiatric manifestations. A 46yearold woman with a history of progressive parkinsonism for 6 years and a normal brain magnetic resonance imaging was partially improved. Sjogren syndrome definition of sjogren syndrome by medical. Frontiers primary sjogrens syndrome associated with. Gougerotsjogrenlike syndrome under pd1 inhibitor treatment. A 46yearold woman with a history of progressive parkinsonism for 6 years and a normal brain magnetic resonance imaging was partially improved with levodopa therapy. A 66yearold woman with sjogren syndrome presented with a 10year history of intermittent bilateral preauricular swelling and pain, dry eyes, and dry mouth. Abstract primary sjogrens syndrome is an autoimmune disorder with. For language access assistance, contact the ncats public information officer. Mar 01, 2006 gougerotsjogren syndrome gss is a chronic heterogeneous non.
Sjogrens syndrome diagnosis and management km mahendranath sjogrens syndrome ss is a chronic inflammatory autoimmune disease characterized by mixed cellular infiltration of exocrine glands, notably the lachrymal glands and salivary glands. Genetically, some people have an increased risk of developing sjogrens, but scientists believe another factor, like being infected by a certain virus or strain of bacteria, is also required to. Association francaise du gougerot sjogren home facebook. Primary sjogrens syndrome presented with sensory ataxia. If you have problems viewing pdf files, download the latest version of adobe reader. Sjogrens syndrome an overview sciencedirect topics. It is characterized by dysfunction and destruction of the exocrine glands associated with lymphocytic infiltrates and immunological. Adolescent sjogrens syndrome presenting as psychosis.
Sjogren syndrome definition of sjogren syndrome by. None of these reports describe the incidence of proper gougerotsjogrenlike syndrome, the time course of the events onset and resolution, or its management. Association francaise du gougerot sjogren, ostwald, basrhin. Lacrimal and salivary gland infiltration results in the classic sicca syndrome of dry eyes and dry mouth in 90% of patients. Sjogrens syndrome causes increased levels of il1ra in csf suggesting increased activity in the interleukin 1. Studies of immune cpis describe dryeye syndrome with an incidence ranging from 1. The involvement of these two glands results in dry mouth and dry eyes also known as sicca complex. 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. Diagnosis and management of sjogren syndrome paul kruszka, lcdr, usphs, u. The essdai is now in use as a gold standard to measure disease activity in clinical studies, and as an outcome. Sjogrens syndrome is a chronic, systemic autoimmune disease that predominantly affects women and targets the salivary glands in the mouth and lacrimal glands in the eye. Preliminary criteria for the classification of sjogrens syndrome. The disease is called primary if it develops in isolation and secondary if it occurs with other autoimmune diseases. Those affected are also at an increased risk 5% of lymphoma.
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