Syndrome de Gougerot-Sjögren et maladie de Waldenström avec atteinte pulmonaire et hypercalcémieGougerot-Sjögren’s syndrome and Waldenström disease. La pathogénie des LMNH au cours du syndrome de Gougerot-Sjögren se fait en thérapeutique en fonction de l’extension et de l’évolution de la maladie. Le syndrome de Sjögren (SS) est une maladie immunitaire caractérisée par une dégénérescence progressive des glandes exocrines, aboutissant à un.

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Medication side effect, anxietysarcoidosisamyloidosis [5]. Foreign Allergic contact dermatitis Mantoux test. A study’s finding has concluded the continuous stimulation of autoimmune B cells, leading to subtle germinal abnormalities in genes having specific consequences in B cells, which underlies the susceptibility to lymphoma. On the other hand, vitamin A levels were inversely associated with extra-glandular manifestations of the disease.

Sjögren syndrome

An Orphanet summary for this disease is currently under development. Nonetheless, it remains to be clarified exactly how reactivation of EBV is induced in lesions of patients with SS, and which specific molecular mechanisms are involved in the process of viral reactivation.

This indicates viral reactivation and inability of lymphoid infiltrates to control EBV replication in SS, leading to the initiation or perpetuation of an immune response in target organs. Health care resources for this gougerpt Expert centres 0 Diagnostic tests 0 Patient organisations 0 Orphan drug s 0.

Maladie de Crohn associée à un syndrome de gougerot sjogren – EM|consulte

Eagle syndrome Hemifacial hypertrophy Facial hemiatrophy Oral manifestations of systemic disease. Seropositivity for anti-Ro and anti-La is associated with greater severity and longer duration of malaxie, and findings of their high abundance from the salivary glands of SS patients suggests gougrrot imperative role in the pathogenesis of SS. Dysregulation of apoptosis programmed cell death is believed to play a role in the pathogenesis of a variety of autoimmune diseases, though its role in SS is controversial.

Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline Evoxac and pilocarpine. The symptoms may develop insidiously, with the diagnosis often not considered for several years, because the complaints of sicca may be otherwise attributed to medications, a dry environment, agingor may be regarded as not of severity warranting the level of investigation necessary to establish the presence of the specific underlying autoimmune disorder.

A contrast agent is injected into the parotid duct, which opens from the cheek into the vestibule of the mouth opposite the neck of the upper second molar tooth. Retrieved from ” https: SS can damage vital organs of the body with symptoms that may plateau or worsen, or go into remission as with other autoimmune diseases.


Basal cell adenoma Canalicular adenoma Ductal papilloma Monomorphic adenoma Myoepithelioma Oncocytoma Papillary cystadenoma lymphomatosum Pleomorphic adenoma Sebaceous adenoma Malignant: Nasopalatine duct Median mandibular Median palatal Traumatic bone Osteoma Osteomyelitis Osteonecrosis Bisphosphonate-associated Neuralgia-inducing cavitational osteonecrosis Osteoradionecrosis Osteoporotic bone marrow defect Paget’s disease of bone Periapical abscess Phoenix abscess Periapical periodontitis Stafne defect Torus mandibularis.

The rose bengal test uses a stain that measures state and function of the lacrimal glands. Check this box if you wish to receive a copy of your message. Some research showed that the lack of vitamin A and vitamin D are associated with this disease. Inhe published his doctoral thesisdescribing 19 females, most of whom were postmenopausal and had arthritis, showing clinical and pathological manifestations of the syndrome. The documents contained in this web site are presented for information purposes only.

D ICD – However, some medications can cause symptoms similar to those of SS. Graves’ disease Myasthenia gravis Pernicious anemia.

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The relationship of autoantibodies expressed in SS with apoptosis is still being researched. Specialised Social Services Eurordis directory. Any distinctive colour change can indicate SS, but confirming the condition requires many related diagnostic tools. Additional information Further information on this disease Classification s 0 Gene s 0 Other website s 0. Although the numerous factors contributing to the progression of this disease have made it difficult to find out the exact origin and cause, major advances over the past decade have contributed to a proposed set of pathogenic events that occur prior to the diagnosis of SS.

Lymphomagenesis in primary SS patients is considered as a multistep process, with the first step being chronic stimulation of autoimmune B cells, especially B cells that produce rheumatoid factor at sites targeted by the disease.

This test’s results can determine whether the salivary glands are functioning adequately. Benign lymphoepithelial lesion Ectopic salivary gland tissue Frey’s syndrome HIV salivary gland disease Necrotizing sialometaplasia Mucocele Ranula Pneumoparotitis Salivary duct stricture Salivary gland aplasia Salivary gland atresia Salivary gland diverticulum Salivary gland fistula Salivary gland hyperplasia Salivary gland hypoplasia Salivary gland neoplasms Benign: Orofacial soft tissues — Soft tissues around the mouth Actinomycosis Angioedema Basal cell carcinoma Cutaneous sinus of dental origin Cystic hygroma Gnathophyma Ludwig’s angina Macrostomia Melkersson—Rosenthal syndrome Microstomia Noma Oral Crohn’s disease Orofacial granulomatosis Perioral dermatitis Pyostomatitis vegetans.


Archived from the original on 4 July SS can be excluded from people with past head and neck radiation therapyacquired immunodeficiency syndrome AIDSpre-existing lymphomasarcoidosisgraft-versus-host diseaseand use of anticholinergic drugs. Autoimmune disease unknown cause [4]. Other search option s Alphabetical list. Hypersensitivity and autoimmune diseases Temporomandibular jointsmuscles of mastication and malocclusions — Jaw joints, chewing muscles and bite abnormalities Bruxism Condylar resorption Mandibular dislocation Malocclusion Crossbite Open bite Overbite Overeruption Overjet Prognathia Retrognathia Scissor bite Maxillary hypoplasia Temporomandibular joint dysfunction.

Others are forced to cope with blurred visionconstant eye discomfort, recurrent mouth infectionsswollen parotid glandsdysphonia vocal disorders including hoarsenessand difficulty in swallowing and eating.

Skin dryness in some people with SS may be the result of lymphocytic infiltration into skin ejogren. For systemic symptoms, including fatigue, joint pain, myositis and neuropathybiologic immunosuppressant drugs such as rituximab and belimumab that work via B-cell pathology are often used and have gougrrot toxic profiles than traditional immunosuppressive regimens.

Ann N Y Acad Sci. SS is usually classified as either ‘primary’ or ‘secondary’. If you want to subscribe to this journal, see our rates You can purchase this item in Xe Per View: Journal page Archives Contents list.

To check the status of salivary glands and the production of saliva, a salivary flow-rate test is performed, in which the person is asked to spit as much as they can into a cup, and the resulting saliva sample is collected and weighed.

Sjögren syndrome – Wikipedia

Environmental factors, such as glandular viral infectioncould prompt epithelial cells to activate the HLA-independent innate immune system through toll-like receptors. Current Opinion in Rheumatology. In other projects Wikimedia Commons. By using this site, you agree to the Terms of Use and Privacy Policy. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.

Following the migration of lymphocytes into the glands in response to chemokines and specific adhesion moleculesT cells interact with epithelial cells.