Sjogren's syndrome-Sjögren's syndrome

Sjogren's syndrome/Sjögren's syndrome-What is Sjogren's syndrome?

Sjogren's syndrome is a violation of lacrimal glands, salivary glands and other exocrine glands, chronic autoimmune disease, also known as autoimmune exocrine disease.

Sjogren's syndrome /Sjögren's syndrome- IntroductionSjogren's syndrome is a violation of lacrimal glands, salivary glands and other exocrine glands, chronic autoimmune disease, also known as autoimmune exocrine disease. Sjogren's syndrome mainly dry keratoconjunctivitis, xerostomia associated with rheumatoid arthritis and other rheumatic diseases, it can affect other systems such as the respiratory system, digestive, urinary, blood Department, nervous system and muscles, joints, caused by multi-system, multi-organ damage.Sjogren's syndrome can exist alone, may also occur in other autoimmune diseases, stand-alone primary Sjogren syndrome secondary to rheumatoid arthritis, systemic scleroderma, systemic lupus erythematosus and other autoimmune disease is secondary to Sjogren's syndrome. The incidence of this disease, more often in women over the age of 40. The pathological mechanism is mainly due to excessive autoimmune response, causing the exocrine glands are a large number of lymphocytes, plasma cells, the gland cell destruction, loss of function, and thus give rise to a series of clinical symptoms and performance.Sjogren's syndrome is a global disease, more than 90% female, age of onset is mostly 40 - to 60-year-old middle-aged, children less. Because so far the lack of uniform diagnostic criteria, so the prevalence of the disease is far from accurate. Is generally estimated at 0.1% - 0.7%. In the U.S., the incidence of Sjogren's syndrome is second only to rheumatoid arthritis.Sjögren's syndrome is a chronic autoimmune connective tissue disease can occur at any age, but incidence of the majority, middle-aged women account for more than 90% occurrence of the disease with genetic, endocrine, viral infection may be related to the exact cause of not yet clear. Currently no effective treatment for drugs of modern medicine, public symptomatic treatment, such as dry eyes with artificial tears in their eyes, dry mouth artificial saliva. Unexpected performance of patients for dry eyes, dry mouth and nose, severe cases, no tears, no saliva, the consumption of water delivery, part of the female patients, vaginal dryness, pain and abnormal. Lesions often involving the lungs, liver, kidney, gastrointestinal, skin, joints and lymph, etc., often secondary to rheumatoid arthritis, lupus erythematosus, polymyositis, nodular artery go far, and may be complicated by chronic active hepatitis reticular cell tumor, malignant lymphoma, and allergic vasculitis. This disease to the mouth, dry eye and rheumatoid arthritis Triad of two exist can be diagnosed patients with laboratory tests often humoral and cellular immune abnormalities, such as erythrocyte sedimentation rate growth, serum gamma globulin, immunoglobulin G significantly l high, low complement, the beginning of the illness is easily overlooked, early treatment is key. Sjögren's syndrome belongs to the Chinese medicine "dry disease" category, symptom manifestations within dry outside dry performance. And more due to the heat-chun, injury or loss within a long illness fine blood, or excessive bleeding, or sweat, spit, next after the injured body fluid. We the Ziyinqingre, nourishing and moistening as the main treatment principle.


Sjogren's syndrome - DiagnosisProposed five diagnostic criteria such as the Copenhagen criteria, Fox standards in the international literature of Sjögren's syndrome, due to poor sensitivity, is rarely used, Again the Manthorpe diagnostic criteria (1981): ① dry eye syndrome; 2 xerostomia [J]; another connective tissue disease. With ① and / or ② and ③ those with secondary SS. The standards in the pilot process is the specificity is poor work. 1992 Dong Yi diagnostic criteria: (1) keratoconjunctivitis sicca; ② The xerostomia; ③ serum one of the following antibodies: anti-SS-A anti-SS-B, ANA> 1:20, the RF> 1:20 3 above, and except for other connective tissue disease and lymphoma, AIDS, and GVH disease can be diagnosed; only, except for the above two and other diseases for possible cases, the specificity and sensitivity of the standard by the clinical trial The results are relatively high.Sjögren's syndrome no special treatment. Attention to the health of the eye and mouth, drops to 0.5% methyl cellulose; often gargle citrate solution, 2% methyl cellulose, pre-dinner smear the mouth even to stimulate the salivary gland secretory function and replace the part of the saliva, can improve symptoms. Severe functional changes and a wide range of systems involved as well as with other connective tissue disease, can be used corticosteroids, immunosuppressants, or Thunder and Chun preparation, plasma conversion the rule fuufurui treatment, can inhibit the parotid gland enlargement and improvement of exocrine function.

Sjogren's syndrome - etiology
Autoimmunity: Sjogren's syndrome patient's body to detect a variety of autoantibodies such as antinuclear antibodies, rheumatoid factor, anti-RNP antibody, anti-SSA antibody, anti-SSB antibodies and hypergammaglobulinemia, reflecting a B lymphocyte the function of a high degree of hyperactivity and inhibition of T lymphocyte dysfunction. In addition, changes in the disease of T lymphocyte subsets that inhibit T cells decreased to reflect the cellular immune abnormalities.

Genetic basis: the determination of the genetic research on the immune, medical scientists found that human leukocyte antigen, HLA-DR3, B8 and Sjogren's syndrome is closely related to, and this because of race vary, such as Western Europeans and of HLA-B8, DR3, DW52-related, the Japanese with the HLA-DR53 related to Sjogren's syndrome have a genetic predisposition.

3 virus infection: There are at least three viruses such as Epstein-Barr virus, cytomegalovirus, HIV virus and Sjogren's syndrome related to Epstein-Barr virus can stimulate B cell proliferation and production of immunoglobulin. Physician in patients with primary Sjogren's syndrome salivary gland, lacrimal gland, kidneys were detected Epstein-Barr virus and its DNA gene.

Dry syndrome - PathogenesisSjögren's syndrome is not clear, some people believe that this disease for one's own immune diseases. The primary port is willing to xerosis and HLA-B8-DRW3 genetic phenotype associated with rheumatoid arthritis patients with HLA-DRW4, the literature reports the incidence of family. Serum anti-CMV IgM antibody titer increased mountain, there is some evidence to support the Epstein-Barr virus in SS merger play a role in the pathogenesis of RA. Certain weeks of age of NZB / NZW mice salivary duct, a large number of lymphocytic infiltration, and there was a series of autoimmune phenomena, that the primary β-cells of these mice there is a over-reaction, for the polyclonal nature, directed against a variety of itself and the non-self antigen epitope, resulting in a variety of autoantibodies and immune complexes, including the generation of suppressor T cell antibodies, functional defects, and continuation of the excessive reaction of the beta cell and the incidence of Human SS may have a similar pathogenesis. Has proven to small lymphocytic foci in minor salivary glands of human SS patients, mainly composed of β-cells and plasma cells, while the larger lesions of the central T cell infiltration, and the surrounding area of ​​beta cells and plasma cells. TH subsets of T cells dominated the early lesions of the tissue damage caused by the antigen recognition and / or migration to the lesion beta cells and plasma cells. The possible mechanism is antibody-dependent cell-mediated cytotoxicity reaction or a cytotoxic antibody, lymphotactin, etc. so that the accumulation of T cells in the lesions.Sjogren's syndrome - EpidemiologyThe incidence of this disease, the prevalence in natural populations is unknown, has been reported population prevalence rate of 0.4% to 0.7%, up to 3 to 4% in the elderly. The incidence is second only to rheumatoid arthritis. The incidence of any age can be more common in middle-aged women.

Sjogren's syndrome - clinical manifestationsThe majority of Sjögren's syndrome onset was insidious and chronic progress, a small number of acute and progress faster. Department of the disease a multisystem disease.(A) patients with oral mild lesions are often ignored, heavier less saliva, food stimulation and chewing can not be a corresponding increase in the secretion of saliva, red, chapped, or ulcers, difficulty moving, tongue tie at the bottom of saliva accumulation, chewing and swallowing difficulties . Common dental cavities and gingivitis, teeth were powder or small piece of broken fall, dry and chapped lips and mouth, bad breath. About half of patients with recurrent cheek swelling and severe when the formation of a squirrel-like face, submandibular gland may also swollen.(B) the eye was keratoconjunctivitis sicca eyes feel dry, itching, foreign body or burning sensation, blurred vision, like screen tilting, photophobia, the cornea may be cloudy, erosion or ulcers, vascular proliferation, perforation can be serious. Can be combined the iris Choroiditis; conjunctiva inflammation, bulbar conjunctival blood vessels to dilate; tear a small number of lacrimal gland enlargement, complicated by bacterial, fungal and viral infections. Occasionally there is exophthalmos as the initial symptom.(C) respiratory nasal gland invasion cause nasal dryness, nose callus formation, often epistaxis and nasal septal inflammation, the Eustachian tube blockage can occur in serous otitis media, conductive deafness; dry throat, hoarseness, sputum sticky, complicated by tracheitis, bronchitis, pleurisy, interstitial pneumonia and atelectasis, patients without significant lung disease patients may have a restrictive ventilation disorder and gases diffuse functional decline.(D) drying to the digestive tract of the pharynx and esophagus can make swallowing difficult, and occasionally cricoid esophageal stricture, gastric mucosal lymphocyte infiltration increased due to gland, gastric mucosa wrinkled arm thick gastric acid secretion reduce the formation of cobblestone false cancer; with atrophic gastritis the incidence can be as high as 70.5%, intestinal absorption function can be impaired the response to gastrin and promote PZ impaired, suggesting that subclinical pancreatitis is more common. The hepatosplenomegaly accounted for 1/5 of cases.(V) urinary tract kidney disease changed accounting for 1/3, interstitial nephritis, renal tubular function defects was renal tubular acidosis, renal glucosuria, amino acids, urine, and uric acid in reducing the absorption, also concurrent glomerulonephritis, Department of IgM and complement in the glomerular deposition.(F) the level of the nervous system nerve tissue can be damaged central nervous system involving the 25%, 10% to 43% of peripheral nerve. The former can be involved from the meninges into the brain parenchyma and all parts of the spinal cord, extensive parts of the peripheral nerves, including nerve root, axon, myelin, sensory and motor branch can be involved; diverse clinical manifestations, including mental disorders, seizures, hemianopia , aphasia, hemiplegia, paraplegia, ataxia, and so on. Mechanism for inflammatory vascular disease of the nerve tissue to cause ischemic or hemorrhagic changes or due to the infiltration of mononuclear cells.(G) involving the muscles about 2%, myalgia, muscle weakness, caused by interstitial myositis interstitial perivascular infiltration of lymphocytes and monocytes, also can appear green in the renal tubular acidosis caused by low blood strive periodic paralysis.(H) off to save 10% of cases involving the joints, showing swelling and pain, often for non-invasive joint.(I) dry skin and mucous membranes, such as fish-like phosphorus disease, erythema nodosum, purpura, Raynaud's phenomenon and skin ulcers; vaginal mucosa can also be drying and shrinking.(10) lymph nodes, local or systemic lymph node enlargement.With secondary SS with the most common connective tissue disease, rheumatoid arthritis (35 ~ 55%). Other partners with SLE, PSS, of MCTD, nodules nodosa, Hashimoto's thyroiditis, primary biliary cirrhosis, chronic active hepatitis. Pseudolymphoma usually SS lymphocyte infiltration limited to the salivary glands and lacrimal gland was benign process of progressive dry eye and mouth, there are obvious gland outside the lymphoid cell infiltration and clinical manifestations of lymph nodes, lung, kidney, liver , spleen, muscle involving blood leukopenia, γ-globulin and macroglobulin increased, Histopathology showed lesions of the infiltrating cells were pleomorphic, the size of lymphocytes, plasma cells and the Gang woven cells, for several years , the benign course of the disease, known as pseudolymphoma. These cases some of which can be transformed into a malignant lymphoma, the infiltrating cells were highly undifferentiated, the organizational structure destruction, infiltrates more than envelope. A significant increase in the prevalence of malignant lymphoma in patients with SS compared with the control population.

Sjogren's syndrome - assistant examinationSjögren's syndrome than the degree of positive cells are hypochromic anemia (25%), leukopenia (6% ~ 33%), increased eosinophils (5% ~ 25%) can also occur with mild thrombocytopenia. ESR (80% -94%). About half of cases of albumin to reduce and strain peak globulin, the main part of the γ-globulin, may also have increased α and β ball high protein. Immunoglobulin IgG and IgM increased about 3/4 patients with rheumatoid factor positive, and often IgM type; antinuclear antibodies (17% ~ 68%), rare anti-dsDNA antibody, macroglobulin, cryoglobulin positive hyperviscosity syndrome; anti-thyroglobulin and anti-gastric parietal cell antibody-positive (30%), antiglobulin test and anti-mitochondrial antibody-positive (10%); anti-SS-A antibodies in primary SS sex-positive rate of 70% to 75%, 48% to 60% of anti-SS-B antibodies, and SS merger with rheumatoid arthritis were 9% and 3%, the anti-salivary duct epithelial cell antibodies (ASDA) in primary SS 25% positive, while the SS with rheumatoid arthritis up to 70% ~ 80% higher in serum and saliva of β2-microglobulin (beta2-M), serum concentrations can be used to observe the disease activity index. IgG concentration in saliva, there are high levels of IgA and IgMRF. The DIF show IgG calm next to the epidermal basal layer and basal layer. Ts were significantly lower in peripheral blood T lymphocytes decreased, the increase in Ia-positive T-lymphocyte population, lymphocyte transformation test and the activity of petal formation test low. Circulating immune complexes increased; of CH50 and C3 increased or normal can reduce the occurrence of vasculitis. About 2/3 patients with reticuloendothelial system Fc receptor functional defects, when SS benign lymphoproliferative into malignant lymphoma, high γ hypergammaglobulinemia variable thought γ-globulinemia, autoantibody titers decreased or negative.(A) the lacrimal gland function tests are the Schirmer test (with filter paper to the determination of tear flow bend at 5mm × 35mm filter paper, placed under the conjunctiva of the observed 5 'tear moist filter paper length <10mm lower than normal) ; tear film breakup time (BUT test, <10 〃 not normal), the cornea 2% fluorescein, or 1% Congo red or 1% Rose Bengal red vital staining (foci <10 is normal). The above modern two positive keratoconjunctivitis sicca.(B) of the salivary glands detect the amount of saliva secretion assay (sugar test pressure into a piece of sucrose per tablet 800mg, was placed in the middle of the back of the tongue, the record is completely dissolved the time required, normal <30 '; saliva flow rate measurement: hollow catheter connected to the suction cup, vacuum adsorption of the unilateral parotid duct opening to collect saliva secretion, normal> 0.5ml/min). Parotid Glands 40% lipiodol to observe any damage and atrophy of the contrast agent in the residence time within the parotid gland, parotid duct stenosis or dilatation the parotid isotope iodine 131 or technetium 99m scan, observe the radioactivity distribution excretion and concentration whether slow or reduce in order to understand the function of secretion. Biopsy observation from Chune or nasal glands of pathological changes, more than two positive xerosis.(C) organization pathological lacrimal gland, parotid and submandibular gland in vivo was infiltration of lymphocytes, mainly β-cells, severe cases of β-cell infiltration may be similar to the germinal centers of lymph nodes, glandular atrophy, proliferation of epithelial cells of the catheter to the formation of epithelial - myoepithelial cells of birds, duct stenosis or expansion of late connective tissue substitute. Gland outside the lymphoid infiltrates involving the lung, kidney or skeletal muscle caused by its dysfunction.

Sjogren's syndrome - to the attention of
Diet: Avoid eating spicy and hot drinks and food, in case help to dry the disability allowance, and aggravate the condition. Not eat spicy, fragrant dry, warm the goods, such as wine, tea, coffee, all kinds of fried food, lamb, dog meat, venison, as well as ginger, onion, garlic, chili, pepper, pepper, fennel, etc., and are prohibited from smoking. Treat Taboo, nor too strict taboos, such as the diet is too strict, over many years, but the impact of nutrition absorption in the condition unfavorable. In short, the food should be fresh, with meat and vegetables, eat much food, diet to suit the taste is appropriate, and to ensure adequate nutrition. Sjögren's syndrome is a common immunodeficiency disease predominantly affects the exocrine glands, including lacrimal and salivary glands, causing the eyes dry mouth. The basic principles of diet for patients with Sjögren's syndrome should eat with Yin and nutrient solution, the fluid moistening effect of food, should eat a light, juicy, multi-vitamins, fresh fruits and vegetables, should eat the sweet and sour food.

Specifically, pay special attention to the diet of patients with Sjögren's syndrome
Yichi: Warou, clam, oyster, Xi Shi tongue, squid, fish, eel, donkey-hide gelatin, honey, royal jelly, herring, mullet, carp, eggs, bean soup, bean soup, bean paste, lily, lotus seeds , radishes, carrots, green vegetables, yellow bean sprouts, shepherd's purse, black garland chrysanthemum, chrysanthemum brain, lettuce, head of wolfberry, Kalimeris, water chestnuts, cucumber, sponge gourd, snake melon, melon, banana, grape, strawberry, tangerine, Luo Han Kuo, ground yellow, yellow fine , Dendrobium, Radix et al.

2, not to eat: It is worth noting: the dog meat, fried peanuts, fried rice, ginger, pepper, cinnamon, ginseng, fried beans, fried beans, chili, pepper, fennel, sparrow meat, longan, litchi, kumquat, betel nuts, Cordyceps, Astragalus, Atractylodes, and other food is not suitable for consumption in patients with Sjögren's syndrome.

Sjogren's syndrome - prognosisSlow course of the disease, depending on the lesions involving the scope and other diseases associated with the cases of pseudolymphoma need to closely observe the outcome, the poor prognosis of malignant lymphoma.How to prevention and treatment of Sjogren's syndrome?

The experts pointed out that due to the relatively dry spring weather, will be metabolized in the human body more moisture, so prone to Sjogren's syndrome. The main symptoms are irritability, dry mouth, thirst and dry moss, dry eye tears, sometimes accompanied by fever, joint pain. Prevention of Sjogren's syndrome in addition to enhance health and improve disease resistance, the best way is to drink plenty of water, the fall of the elderly day complement of water should be 2000-2400 ml, morning human blood concentration is very high, blood clots, so the morning get up to drink 700 ml of water, this glass of water can play the role of diluted blood, cleaning the intestinal tract. In this special day, these patients eating light, avoid eating to lit foods, fried foods, usually drink more water, eat more fruits, eat more vegetables, to ensure smooth bowel movements. Tonic and should not eat deer antler, cinnamon and other dry food. Also home to the indoor humidification with a humidifier.Expert advice: avoid lit three measures, as soon as possible to a regular hospital treatment.Avoid angry, first of all have good living habits, time and rest, to avoid staying up late, regular meals. Secondly, we must eat Qinghuo food such as fresh green leafy vegetables, cucumber, orange, green tea; also eat some carrots, add the body of essential vitamins B, to avoid chapped lips. Finally, to maintain peace of mind. Peace of mind conducive to blood smooth, to avoid the angry emotions are stimulated. In addition, Sjogren's syndrome is a systemic disease, as soon as possible to a regular hospital immune outpatients.


[Sjögren's syndrome care]And psychological care: Because of the longer course of Sjögren's syndrome, patients are often depressed, do psychological counseling of the patient while doing basic care, to improve their fears, the elimination of pessimistic psychological and emotional burden, a positive attitude towards the disease . In addition, patient health education is also very important to advocate a healthy lifestyle and to learn self-care is an important factor to improve patients' quality of life., Respiratory care: care of dry syndrome, mainly refers to the indoor humidity control in 50% -60%, the temperature is kept at 18 ℃ -21 ℃, can ease the respiratory mucosa dry dry cough and other symptoms, and can prevent infection. Difficult to cough up thick phlegm, patients with Sjögren's syndrome do inhalation.Skin care: on sweat gland involvement lead to dry skin, scaling and itching less with or without alkaline soap, use a neutral soap. Frequently changes underwear, blankets, keep the skin dry.