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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While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacteria. Treatment is directed at the person’s symptoms.
The hallmark symptom of SS is dry mouth and keratoconjunctivitis sicca dry eyes. Skin dryness in some people with Sjogeen may be the result of lymphocytic infiltration into skin glands. 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.
SS can damage vital organs of the body with symptoms that may plateau or worsen, or go gougerit remission as with other autoimmune diseases. Some people may experience only the mild symptoms of dry eyes and mouth, while others have symptoms of severe disease.
Many patients can treat problems symptomatically. Others are forced to cope with blurred visionconstant eye discomfort, recurrent mouth infectionsswollen parotid glandsdysphonia vocal disorders including hoarsenessmwladie difficulty in swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life. Some patients can develop renal kidney involvement autoimmune tubulointerstitial nephritis leading to proteinuria excess protein in urineurinary concentrating defectand distal renal tubular acidosis.
SS is associated with a number of other medical conditions, many of which are autoimmune or rheumatic disorders, such as celiac disease  fibromyalgiaSLE lupusautoimmune thyroiditismultiple sclerosis and spondyloarthropathy and several malignanciesprincipally non-Hodgkin lymphoma. The cause of SS is unknown, but it may be the influence of a combination of genetic, environmental, and other factors—as is the case with many other autoimmune disorders.
The observation of high rates of autoimmune disorders in families of SS is linked with jsogren genetic predisposition to the syndrome. Since SS is associated with a high prevalence in women, sex hormonesespecially estrogenare believed to affect humoral and cell-mediated immune responses affecting susceptibility to gougrrot syndrome. Microchimerism of fetal cells offspring lymphoid cells in maternal circulation may generate autoimmunity in women who have been previously pregnant.
Viral proteinsengulfed moleculesor degraded self-structures may initiate autoimmunity by molecular mimicry and increase the chances of SS development.
The pathogenetic mechanisms of SS have not been fully elucidated, resulting in the lack of pathophysiology knowledge of the management of this autoimmune exocrinopathy. 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 maadie set of pathogenic events that occur prior to the diagnosis of SS.
SS was originally proposed as a specific, self-perpetuating immune system-mediated loss of exocrine glands, specifically acinar and ductal cells. Although this explains sjogrwn more obvious symptoms e. This suggests that the disease begins with increased activity in ce interleukin 1 system, followed sjogrej an autoregulatory up-regulation of IL-1RA to reduce the successful binding of interleukin 1 to its receptors. Interleukin 1 likely is sjogfen marker for fatigue, but increased IL-1RA is observed in the CSF and is associated with increased fatigue through cytokine sjogrne sickness behavior.
Seropositivity for anti-Ro and anti-La is associated with greater severity and longer duration of disease, and findings of their high abundance from the salivary glands of SS patients suggests their imperative role in the pathogenesis of SS. Beyond genetics, epigenetic abnormality related to DNA methylationhistone acetylationor microRNA expression probably have key roles in the pathogenesis of autoimmune diseases, including SS, though research in this area is very limited and minimal.
Environmental factors, such as glandular viral infection re, could prompt epithelial cells to activate the HLA-independent innate immune system through toll-like receptors. 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.
Nonetheless, it remains to be clarified exactly how reactivation of EBV is induced in lesions of patients with SS, and which malade molecular mechanisms are involved in the process of viral reactivation.
Epithelial cells in SS gougetot are active participants in the induction and perpetuation of the inflammatory gouerot.
Environmental and hormonal factors, in concert with an appropriate genetic background, are believed to trigger SS, which dysregulates epithelial cells and allows aberrant homing and activation of dendritic cells DCsT cells, and B cells. Following the migration of lymphocytes into the glands in response to chemokines and specific adhesion moleculesT cells interact with epithelial cells.
The early accumulation of plasmacytoid dendritic cells in the target tissues, which produce high levels of type 1 IFNs, seems important, as these cells can further dysregulate the immune response through abnormal retention of lymphocytes in the tissues and their subsequent activation.
BAFF stimulates aberrant B-cell maturation, leading to the emergence of self-reactive B cells, which locally produce autoantibodies, in a germinal centre -like structure GC-likewhich is also the location of lymphomagenesis origin of lymphoma. 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. Both the Fas and Fas ligand proteins are overexpressed in primary SS patients, while expression of BCL-1which is known to downregulate apoptosis, was found significantly reduced in acinar and ductal epithelial cells of SS patients compared to healthy people.
Reduced apoptosis was also implicated in the accumulation of autoreactive B-cells found in the glands. The relationship of autoantibodies expressed in SS with apoptosis is still being researched. Sex hormones seem to influence humoral and cell-mediated immune response, with estrogen being considered one of the biggest factors responsible for sex- immunologic dimorphism. Diagnosing SS is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms of SS and those of other conditions.
Also, patients with SS symptoms approach different specialities for treatment, which can make diagnosis difficult.
Since dry eyes and dry mouth are very common symptoms, and frequently occur in people over 40, people often think the symptoms are age-related and ignore them. However, some medications can cause gougerott similar to those of SS. The combination of several tests, which can be done in a series, can eventually diagnose SS.
SS is usually classified as either ‘primary’ or ‘secondary’. Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as antinuclear antibody ANA and rheumatoid factor because SS frequently occurs secondary to rheumatoid arthritiswhich are associated with autoimmune diseases.
The rose bengal test uses a dr that measures state and function of the lacrimal glands. This test involves placing the non-toxic dye rose bengal on the eyes. Any distinctive colour change can indicate SS, but confirming the condition requires many related diagnostic tools.
Schirmer’s test measures the production vougerot tears: This measurement analysis varies among people depending on other eye-related conditions and medications in use when the test is taken.
Ds of dry mouth and dryness in the oral malarie are caused by the reduced production of saliva from the salivary glands parotid glandmalqdie glandand sublingual gland. 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.
This test’s results can determine whether the salivary glands are functioning adequately. Not enough saliva produced could mean the person has SS. A resultant collection of less than 1. In addition, a sialograma special X-ray test, is performed to see if any blockage is present in the salivary gland ducts i. Also, a radiological procedure is available as a reliable and accurate test for SS. A contrast agent is injected into the parotid duct, which opens wjogren the cheek into the vestibule of the mouth opposite the neck of the upper second molar tooth.
Histopathology studies should show focal lymphocytic sialadenitis. SS can be excluded from people with past head and neck radiation therapyacquired immunodeficiency syndrome AIDS dde, pre-existing lymphomasarcoidosisgraft-versus-host diseaseand use of anticholinergic drugs.
There is no prevention mechanism for SS due to its complexity as an autoimmune disorder. However, lifestyle changes can reduce the risk factors of getting SS or reduce the severity of the condition with patients who have already been diagnosed. Diet is strongly associated with inflammation that is mostly seen in many autoimmune related diseases including SS. An ogugerot study concludes that SS patients show high sensitivity to gluten that directly relates to inflammation.
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Neither a cure for SS nor a specific treatment is known to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes.
Some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the ocular surface for a longer time.
Additionally, cyclosporine Restasis is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion. Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline Evoxac and pilocarpine. Salagena manufactured form of pilocarpinecan be used to help produce tears, as well as saliva in the mouth and intestines.
Orphanet: Syndrome de Gougerot Sjogren
It is derived from the jaborandi plant. In women with SS, vaginal dryness, vulvodynia and dyspareunia painful sexual intercourse are often reported; personal lubricants are recommended to help lessen irritation or pain that may result from dryness in the vaginal and vulva areas.
For individuals with severe complicationscorticosteroids or immunosuppressive drugs may be prescribed, and sometimes IVIG intravenous immunoglobulin. Hydroxychloroquine Plaquenil is another option and is generally considered safer than methotrexate. Also, people who take drugs to suppress the immune system are more likely to develop cancer later.
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 less toxic profiles than traditional immunosuppressive regimens.
CENTRE MALADIES RARE COCHIN
Preventive dental treatment is also necessary and often overlooked by the patientas the lack of saliva associated with xerostomia creates an ideal environment for the proliferation of bacteria that cause cavities.
Existing cavities must also be treated, as cavities that extend into the tooth can not be effectively treated through teeth cleaning alone, and are at a high risk of spreading into the pulp of the toothleading to the loss of vitality and need for extraction or root canal therapy.
This treatment regimen is the same as for all xerostomia patients—such as those undergoing head and neck radiation therapy, which often damages the salivary glands, which are more susceptible to radiation than other body tissues. Published studies on the survival of SS patients are limited in varied respects, perhaps owing to the relatively small sample sizes, and secondary SS is associated with other autoimmune diseases.
However, results from a number of studies indicated, compared to other autoimmune diseases, SS is associated with a notably high incidence of malignant non-Hodgkin lymphoma NHL.
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.
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. Apart from this notably higher incidence of malignant NHL, SS patients show only modest or mqladie insignificant deterioration in specific organ-related function, which explains the only slight increases in mortality rates of SS patients in comparison with the remainder of the population.
SS is the second most common rheumatic autoimmune disorder, behind rheumatoid arthritis RA and systemic lupus erythematosus SLE.
Nine out of ten SS patients are women. Although SS occurs in all gougero groups, the average age of onset is between ages 40 and 60, although experts note that up to half of all cases may be left undiagnosed or unreported. Jan Mikulicz-Radecki — is generally credited with the first description of SS. Inhe described a year-old man with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy.
Inhe published his doctoral thesisdescribing 19 females, most of whom were postmenopausal and had arthritis, showing clinical and pathological manifestations of the syndrome.
Research on multifactorial autoimmune diseases such as SS focuses on expanding the knowledge surrounding the siogren, improving diagnostic tools and finding ways to prevent, manage, and cure the disorder. As an autoimmune diseasesusceptibility to SS is greatly influenced by the human leukocyte antigen. Some research showed that the lack of vitamin A and vitamin D are associated with this disease.
On the other hand, vitamin A levels were inversely associated with extra-glandular manifestations of the disease. Saliva is a potential diagnostic tool of SS because the salivary component is changed after onset of the disease.
With regard to therapeutics, multiple monoclonal antibodies were under investigation in From Wikipedia, the free encyclopedia. Archived from the original on