Sjögren's syndrome
Introduction
Introduction to Sjogren Sjögrensyndrome (SS) is a chronic autoimmune disease that invades the exocrine glands, especially the salivary glands and lacrimal glands. Mainly manifested as dry mouth and eyes, but also multi-organ, multi-system damage. A large number of lymphocytes infiltrate in the affected organs, and various autoantibodies in the serum are positive. This syndrome is also known as autoimmune exocrine disease, autologous syndrome, and Sjogren's syndrome. Often overlap with other rheumatism or autoimmune diseases. basic knowledge The proportion of illness: 0.012% Susceptible people: no specific population Mode of infection: non-infectious Complications: chronic bronchitis, atrophic gastritis, epilepsy, lymphoma
Cause
Cause of Sjogren's syndrome
Genetic factors (30%):
(1) Histocompatibility antigen: In recent years, the frequency of some major histocompatibility complex (MHC) genes has increased in patients with Sjogren's syndrome, and human MHC is also called human. Human leucocyte antigen (HLA), associated with Sjogren's syndrome, is HLA-DR3, HLA-B8. This correlation may vary from race to race and is reported to be associated with HLA-B8 in Western European Sjogren's syndrome. Related to DR3, DRW52, HLA-DR5 in Greece, HLA-DR53 in Japan, HLA-DR8 in China, and DRW52 in male patients, HLA gene and Sjogren's syndrome itself There are also correlations between antibody production and clinical manifestations. For example, Sjogren's syndrome with HLA-DR3 and DQ1/DQ2 has high titers of anti-SSA, SSB antibodies, clinical symptoms, and vasculitis. These results suggest that some class II HLA genes may be genetic markers of susceptibility to Sjogren's syndrome. They play an important role in the pathogenesis, clinical manifestations and disease persistence of Sjogren's syndrome, but in non-dry synthesis A large number of healthy people in the levy also have HLA-DR3 or DR8. Therefore, the genetic basis is only one of the causes of Sjogren's syndrome. It is necessary to have other factors involved in the disease to cause the onset of Sjogren's syndrome.
(2) Family history: Sjogren's syndrome patients have sisters, mothers and daughters at the same time, which also suggests that there are genetic factors in the cause of the disease.
Virus infection (25%):
It is believed that a variety of viruses are associated with the onset of Sjogren's syndrome and the persistence of the disease, such as Epstein-Barr virus, herpesvirus type 6, cytomegalovirus, retrovirus, and the like.
(1) Epstein-Barr (EB) virus: It is a common herpesvirus that infects humans. It has the property of activating B cells with high proliferation. It is characterized by hyperglobulinemia, which can be converted into B lymphocyte tumors. In patients with syndrome, the early antigen (EA) and DNA of Epstein-Barr virus in the lacrimal gland, salivary gland, and renal tubular epithelial cells indicate that Epstein-Barr virus is actively replicating in Sjogren's syndrome patients, which continuously activates B cell proliferation and differentiation. The disease continues to linger; other scholars have also detected the DNA of Epstein-Barr virus from normal human salivary gland biopsy specimens, so it is suspected that Epstein-Barr virus is the cause of this disease.
(2) retrovirus: human immunodeficiency virus (HIV) infected with retrovirus may have dry syndrome, parotid gland swelling and other symptoms of Sjogren's syndrome, and 30% of patients with Sjögren's syndrome in serum An antibody to the HIV component P24gag protein was detected; in HTLV-1 (human T lymphocytic leukemia virus, another retrovirus), Sjogren's syndrome-like symptoms also appeared, so some people think that HIV infection may be Sjogren's syndrome. However, although HIV patients have mouth and dry eyes, they do not have anti-SSA and SSB antibodies in serum. They are not related to HLA-DR3. In addition, some SLE patients also have anti-P24 antibodies in their serum, so they cannot explain HIV or HTLV. -1 is the direct cause of Sjogren's syndrome.
(3) Hepatitis C virus: The US literature reports that hepatitis C virus has no significance in Sjogren's syndrome, unless accompanied by mixed cryoglobulinemia or other diseases, such as membrane proliferative nephritis, a report in France found in the original In the Sjogren's syndrome, hepatitis C virus infection is about 10%. These viruses can occur in the salivary glands of the lips, and it is considered that hepatitis C virus infection is one of the causes of Sjogren's syndrome. Italian scholars believe that Hepatitis C virus is dry. There is no effect on the autonomic organ damage of the syndrome. The form of lymphocyte lesion damage in chronic hepatitis C is different from that of primary Sjogren's syndrome.
Endocrine factors (25%):
Estrogen levels in patients with Sjogren's syndrome are elevated, and most patients with Sjogren's syndrome are women, presumably associated with elevated estrogen. The disease can be found in the mouth, eyes, etc., and then the muscles and joints of the extremities, followed by Internal organs are dirty; muscle and joint symptoms and visceral damage can also occur first, followed by signs of dry eyes.
Pathogenesis
Cellular immunity
(1) Lymphocytes:
1 Peripheral blood: the total number of lymphocytes and the number of CD4 and CD8 subpopulations of T lymphocytes, the ratio is 70% to 80% of Sjogren's syndrome patients are normal, but their functions may be abnormal, it has also been reported that CD8 is reduced The number of T cells with inhibitory function in CD8 is reduced, resulting in a large proliferation of B cells. CD5 B-labeled lymphocytes increase by up to 30% to 40% in dryness syndrome (normally only 15% to 25%). The cells belong to poorly differentiated or incompletely mature cells, which spontaneously secrete IgM-RF and anti-single-stranded DNA antibodies (anti-SS-DNA antibodies), so there is a significant differentiation of T and B lymphocytes in the blood surrounding Sjogren's syndrome. Mature and dysfunctional.
2 In the labial gland: a large number of lymphocyte clusters can be seen in the interstitial tissue of the parotid gland, which is mainly composed of T cells, and T cells in the T cells with the activated marker 4B4 /CD45RO predominate. 4B4 cells are activated T cells with complementary memory function. Most of the cells in the labial gland and ductal epithelial cells express HLA-DR molecules, further indicating T and B cells in the labial gland of Sjögren's syndrome. Both ductal epithelial cells were activated, and the same changes were seen in the small lacrimal gland tissue of the conjunctiva of Sjogren's syndrome.
(2) Natural killer cells: Natural killer cells are a group of extremely large lymphocytes. The number of natural killer cells in the peripheral blood of Sjogren's syndrome is normal, but the function is decreased, and monocytes in their exocrine glands (lip glands) Such cells are absent from infiltrating lesions.
2. Humoral immunity Sjogren's syndrome patients are characterized by hyperglobulinemia and a variety of autoantibodies, reflecting the high hyperfunction of B lymphocyte function and the inhibition of T lymphocyte suppression.
(1) Hyperglobulinemia: gamma globulin in 95% of patients has increased to varying degrees, most of which are polyclonal, immunoglobulin IgG, IgM, IgA can be increased, with IgG increasing the most See, IgM or IgA is also not uncommon, it can also be increased in monoclonality, a small number of patients can appear or chain fragments in urine, hyperglobulinemia in Sjogren's syndrome compared with other connective tissue diseases such as systemic lupus erythematosus and Rheumatoid arthritis is more prominent, and B lymphocytes also have the function of synthesizing a large number of immunoglobulins in the local tissues of the labial glands.
(2) Autoantibodies: Due to the proliferation of B lymphocytes, this disease produces a variety of autoantibodies.
1 anti-SSA (Ro) antibody and anti-SSB (La) antibody: they are closely related to Sjogren's syndrome, and can also occur in some patients with systemic lupus erythematosus, all of which are of IgG type. The antigen recognized by anti-SSA antibody is intracytoplasmic. The small RNA (hy5RNA) is linked to the 60kD and 52kD protein components, both of which belong to the ribonucleoprotein complex. 80% of the antibodies that act on 52kD are from Sjogren's syndrome serum, and the antibodies that act on 60kD are often from SLE. About 85% of Sjogren's syndrome has antibodies against 52kD and 60kD. The anti-SSB antibody recognizes 47kD protein, which is different from the 60kD and 52kD proteins. Anyone with anti-SSB antibody has anti-antibiotics. SSA antibody, because the SSB molecule and SSA are linked together, the SSB RNA may be linked to a variety of proteins, including 70kD, SnRNP A and B/B proteins, SSA 60kD protein, anti-SSA, SSB antibody to Sjogren's syndrome The diagnosis has played a big role.
Two types of rheumatoid factor (RF): RF is an autoantibody that binds to the IgG-FC segment. It occurs in various autoimmune diseases such as Sjogren's syndrome, rheumatoid arthritis, and systemic lupus erythematosus. The positive rate of IgM-RF in Sjogren's syndrome is about 50%, lower than RA (70%), higher than SLE (30%), and the RF positive rate accounts for more than 90% in our Sjogren's syndrome cases. IgM-RF also It can occur in some tumors, infections and other non-rheumatic diseases, where it only represents the body's immune disorder, but no specificity.
3 Others: anticardiolipin antibody (ACA) (25%), anti-mitochondrial antibody (20%), low titer anti-double-stranded DNA antibody (anti-ds-DNA antibody) (10%), anti-RNP antibody, and the like.
4 anti-organ antibodies: patients with Sjogren's syndrome can also appear anti-thyroid duct antibody, anti-thyroid antibodies, anti-corporeal cell antibodies.
(3) immune complex: about 80% of Sjogren's syndrome has circulating immune complexes, which are formed by a large number of autoantibodies and antigens combined and due to reticular endothelial system clearance dysfunction, serum cryoglobulin appears, often representing There is damage outside the gland.
(4) Cytokines: The cytokines (IL-1, IL-6, TNF-, IFN-) secreted by blood lymphocytes around Sjogren's syndrome and the serum cytokine levels were not abnormal. The levels of IL-2 and TNF- in serum decreased, and mR-NA expression of IL-1, IL-6, TNF- and IFN- was expressed in labial epithelial cells and mononuclear cells, suggesting that cytokines are involved. Sjogren's syndrome local, the pathogenesis of labia.
(5) Abnormal immune function, T, B lymphocytes in peripheral blood have obvious differentiation, maturation, and functional abnormalities. The animal model of Sjogren's syndrome has premature thymic decline and decreased T lymphocyte function. B lymphocyte hyperfunction appears in a variety of autoantibodies that produce immune damage, and human Sjogren's syndrome may have a similar pathogenesis.
3. Pathology
The salivary glands, lacrimal glands, and any organs in the body can be affected, and there are two major pathological changes.
(1) Exocrine gland inflammation: There is a large amount of lymphocytes, including plasma cells and monocytes, infiltrating between the exocrine glands composed of columnar epithelial cells. This aggregated lymphocyte infiltrative lesion is a characteristic pathological change of this disease. Appears in the salivary glands (including the small parotid glands of the lips, ankles), lacrimal glands (including the small lacrimal glands of the conjunctiva), renal interstitial, interstitial lung, digestive tract mucosa, hepatic portal, bile ducts and lymph nodes, eventually leading to local The epithelial cells of the ducts and glands proliferate, followed by degeneration, atrophy, destruction, and the loss of their proper function by replacing the fibrous tissue. Some people in the salivary gland and the lacrimal gland have a large amount of lymphocyte infiltration called pseudo-lymph. Tumor (Figure 2, 3).
(2) vasculitis: caused by cryoglobulinemia, hyperglobulinemia or immune complex deposition, is the pathological basis of this disease complicated by glomerulonephritis, peripheral and central nervous system lesions, rash, and Raynaud's phenomenon.
4. The etiology and pathogenesis of TCM is higher than that of the normal population. Therefore, when the lymphocyte infiltration is more prominent and the cell morphology is more primitive, attention should be paid to the possibility of malignant lesions. If the local lymphocyte structure is damaged, it means It has been converted to a malignant lymphoma.
The cause is dryness and internal dryness. The disease is caused by internal dryness. The "Nei Jing" has a cloud: "The dryness is dry", and the later Liu Hehe pointed out in the "Su Wen Xuanji original disease": "Zhu Yu Dryness, dryness and sputum are all dry. The most common cause of dryness is the seasonality. After the autumn equinox, the main reason for the dry gold is that the people are steamed by Xiayueyan, the liquid is sweaty, the viscera is dry, and the water is exhausted. Feeling dry, or the old luck is dry Jinsitian, also easy to feel evil, this is dry; the body's lack of Yin liquid, or chronic illness and injury, postoperative, postpartum, yin essence damage plus high body weakness Or the treatment of mistreatment and other treatments can lead to dryness and stagnation of the sputum, but the sputum is lost, but the sputum is dry, the yin deficiency is deficient, the blood is insufficient, the phlegm is lost in phlegm, and the disease is long-lasting. , involving the skin mucosa, muscle joints, deep to the viscera and cost.
(1) congenital deficiency: the disease is often lack of congenital endowment, loss of yin essence; or yin deficiency, less fluid loss; or long-term illness can lead to yin and yang deficiency, clear sputum dystrophy, and the disease Women's body yin and use yang, 40-year-old women's scorpion is exhausted, blood deficiency, lack of Yin liquid, and more due to yin deficiency heat and injury to Tianjin, causing eyelid clearing and dystrophy, menstrual blood stasis and multiple The disease.
(2) acquired factors: or hurt for emotional, excessive fatigue; or for long-term illness and dying, blood in the body; or for the high-income people will be exhausted; or for mistreatment, treatment, such as misuse of sweat , spit, the next method; or over the agent of Xin Wensheng powder; or the loss of blood, etc., can lead to insufficient Yin fluid, loss of righteousness, and the disease.
(3) Six kinky and evil: six kinky, wind, summer, dry, fire four evils called Yangxie, Yang heat Sheng, then eliminate the body fluid; and cold and cold people can heat, wind and heat can hurt people Squeeze dry, heat is liquid, dry is hurt, the beginning of the disease is in the table, the collaterals are blocked, the joint muscles are painful, the fluid is dry and the mouth is dry, and the disease will damage the internal organs.
Pathogenesis: dry from the upper, lung gold first, so many lungs see the disease, see dry cough, chest full, gas reversal or traction chest pain can not turn side, asthma and vomiting, dry nose and dry lips, dry tongue Shaojin, Sore throat, skin splitting, cold and body pain, etc.; lungs are the main body of the body, feeling evil qi stagnation is unfavorable, muscles and joints are painful, a painful body; lung dryness can not be transported with water essence, Zhonggong water is difficult to cloth It can be spilled into joint swelling; dry and dry gold murderous gas, Jinhuo Tonggong, all things withered, so the ancients have "fire on dry", the gas is dry and hot, the blood is forced to see the skin.
The basic pathogenesis of this disease is the weakness of the body, Yin and Jin deficiency, its disease in the mouth, eyes, nose, pharynx and other clear sputum can affect the whole body, closely related to the lungs, spleen, liver, kidney, and even the heart , stomach, as well as skin mucosa, muscle joints; nature is the virtual standard, lung, spleen, liver, kidney yin deficiency, hot and dry gas as the standard.
Prevention
Sjogren's syndrome prevention
The etiology of Sjogren's syndrome is not fully understood, but it is related to Epstein-Barr virus infection and is related to genetic factors. Active prevention and treatment of EB virus infection should have positive effects in preventing the occurrence of Sjogren's syndrome.
Disability analysis
(1) About half of the patients with rheumatoid arthritis, mainly invading the knee, elbow, and proximal metacarpophalangeal joints and metacarpophalangeal joints, showing pain and weakness, affecting joint function.
(2) 20% to 30% of patients with systemic vasculitis, neurological vasculitis may have neuropsychiatric symptoms, epileptic seizures, hemiplegia, hemianopia, ataxia.
(3) The incidence of malignant lymphoma is significantly increased, which can be fatal.
2. Personal prevention
(1) Primary prevention
Avoid factors that may cause disease, such as wind and heat, dry fire, etc.; life rules, work and rest; strengthen physical exercise, improve immunity; pay attention to health, prevent infection.
(2) Secondary prevention
1 early diagnosis: Sjogren's syndrome is a systemic disease, which can affect various organ systems, and the clinical manifestations are diversified, and the onset is concealed, slowly progressing, and it is difficult to diagnose early. Middle-aged women have dental caries, repeated parotid gland enlargement, and repeated eyelids. Suppurative infection, purulent discharge of eyelids, non-infectious organ damage, unexplained renal tubular acidosis, chronic pancreatitis, hypergammaglobulinemia, should be highly suspected of this disease, autoantibody examination, eye and mouth The relevant tests help early diagnosis.
2 early treatment: mainly to replace and symptomatic treatment, to prevent secondary lesions caused by dry mouth.
Complication
Sjogren's syndrome complications Complications chronic bronchitis atrophic gastritis epilepsy lymphoma
Parotid or submandibular gland enlargement, keratitis, decreased vision or even blindness; chronic bronchitis and interstitial pneumonia, atrophic gastritis; hepatosplenomegaly, chronic active hepatitis, distal renal tubular acidosis and hypokalemia Paralysis, renal diabetes, diabetes insipidus, kidney disease, glomerulonephritis, etc.; purpura, Hashimoto's thyroiditis, Raynaud's phenomenon; central nervous system damage caused by epilepsy, mental disorders; may also be complicated by lymphoma, arthritis, myositis Wait.
Symptom
Symptoms of Sjogren's Syndrome Common Symptoms Dry skin re-infection, facial peeling, often thirst, joint pain, weakness, dry skin, congestion, weakness, dry cough
The incidence of women is more than that of men. The ratio of male to female is 1:9. The majority of the onset age is 40 to 60 years old, but adolescents can also develop the disease.
1. Dryness angle, conjunctivitis: patients often have foreign body sensation in the eyes, burning sensation, itchy eyes, dry eyes, excessive tears often appear in the early stage, as the disease progresses, the vision is gradually blurred, red eyes, eye pain, morning It is difficult to blink at the beginning, and tears cannot be produced in the future when foreign body is stimulated or emotionally excited.
Ophthalmologic examination can be seen around the cornea, sometimes visible lacrimal gland enlargement, advanced corneal formation in the late stage, accompanied by cloud sputum, followed by ulceration, sometimes due to perforation or iridocyclitis, full ocular inflammation, empyema in the eye And blindness.
2. Oral dryness: patients with early stage of the disease often feel insufficient saliva, dry mouth or sticky in the mouth, followed by lack of saliva during eating, loss of taste, pain in tongue and mouth cracking, difficulty in chewing and swallowing, etc. Awakening, due to the dry population of drinking water, can be similar to diabetes insipidus, food adheres to the dry oral mucosa, oral mucosal peeling and oral bleeding can occur, sometimes Candida albicans infection, due to reduced saliva, easy Dental caries occur, oral examination often found that the salivary glands around the tongue ligament are absent, and salivary salivary glands also do not secrete saliva.
3. Parotid gland enlargement: less than 1/3 of patients may have swollen parotid gland, most patients feel local mild discomfort, parotid gland texture is hard, no tenderness, but in the secondary infection may be tender, parotid gland enlargement Simple mouth, mostly dry eyes, parotid angiography shows that there are almost different degrees of parotid gland ductal expansion or stenosis, parotid swelling may be due to calcification or secondary infection, easily misdiagnosed as mumps, parotid gland Different episodes of fever can occur in each episode, which may be due to an acute attack of the disease itself or due to secondary infection.
4. Otolaryngeal performance: As the disease progresses, the change of dryness often affects the mucous membrane of the mucus, which is caused by the ear, nose and throat. It causes nasal discharge, dry nasal scarring, mucosal atrophy, olfactory insufficiency, dry throat pain and discomfort. The hoarse voice, a small number of patients can have a perforation of the nasal septum, easily misdiagnosed as Wegener granuloma.
5. Joint performance: Most patients may have joint symptoms, which are characterized by joint pain, swelling, a few joint fluids, and sometimes muscle pain and muscle atrophy around the joints. Most of the joint swelling and pain occur several months before the symptoms of dryness. In a few years, the mouth can be dry first, and joint symptoms appear after many years.
6. Respiratory system performance: laryngeal, trachea, bronchus can occur lymphocytes and plasma cells infiltration, gland atrophy, which can lead to respiratory mucosal atrophy, and finally lead to severe dry cough or sticky cough is not easy to cough up, as the disease progresses, often Causes repeated infection of the lungs, bronchiectasis, diffuse pulmonary fibrosis, and sometimes pleural effusion, even if the oral dry type is often accompanied by fibrous alveolitis, pulmonary hypertension may occur in the later stages of the disease leading to pulmonary heart disease.
7. Digestive tract performance: In addition to oral symptoms, severe patients due to posterior esophageal stricture of the cartilage and esophageal mucosa dry, esophageal motility disorder, dysphagia may occur, individual patients may develop esophagitis, chronic atrophic gastritis, pernicious anemia or chronic pancreatitis Etc., about 10% of patients can develop autoimmune hepatitis, primary cholestasis hepatitis and occult cirrhosis.
8. Urinary system performance: abnormal renal function, mainly affecting renal tubular function, about 1/4 of patients with renal tubular acidosis, other renal tubular dysfunction including renal diabetes insipidus, multiple amino aciduria, renal tubular re Absorption of uric acid dysfunction, hyperuricemia, renal tubular dysfunction may be caused by interstitial nephritis, chronic pyelonephritis or hypergammaglobulinemia, a small number of patients may develop glomerulonephritis and renal arteritis, fluorescent immunity Examination revealed that the glomerular basement membrane had IgM and C3 precipitates.
9. Neuromuscular system manifestations: Individual patients may have isolated cranial nerve spasm, and sometimes multiple cranial nerves and peripheral neuropathy may occur. Nearly half of the patients have neurasthenia, and some patients may develop myositis and myasthenia gravis.
10. Skin mucosa performance: Due to partial or complete atrophy of sweat glands, more than half of patients may have dry skin, partial or complete sweatlessness, 1/3 of patients may have vulva and vaginal dryness, and severe patients may have vaginal burning or sexual intercourse difficulties. Gynecological examination shows dry mucous membranes, sometimes erythema, genital dryness often coincides with severe dry mouth, about 10% of patients may have non-thrombocytopenic purpura, which is caused by hypergammaglobulinemia, purpura Generally repeated in batches, round, pink, more common in the lower limbs.
11. Cardiovascular system changes: some patients may have pericarditis, myocarditis, congestive heart failure and so on.
12. Others: Some patients may be accompanied by systemic lupus erythematosus, scleroderma, dermatomyositis, polyarteritis, vasculitis, chronic lymphocytic thyroiditis, periodic fever and other clinical manifestations, in addition, the disease Like systemic lupus erythematosus, it is also prone to allergies such as penicillin.
In the advanced stage, lymphoma may occur. Lymphoma is divided into salivary gland lymphoma and salivary gland lymphoma. Salivary gland lymphoma does not involve lacrimal gland and salivary gland. When lymphoma is present, salivary gland is significantly enlarged, and lymph nodes in other parts are also significantly increased. In severe cases, non-thrombocytopenic purpura can occur, liver and spleen increase significantly, neuropathy or Raynaud phenomenon is also aggravated, in addition, Walström macroglobulinemia, thymoma, acute In granulocyte leukemia, if IgM is found by immunoelectrophoresis, it is highly suspected of malignant transformation even if there is no lymphoma in clinical practice, and IgM may decrease after malignant transformation or malignant transformation.
Examine
Sjogren's syndrome check
Laboratory inspection
1. Blood routine : Half of patients may have mild positive cell-positive anemia, and mild leukopenia may occur in individual patients.
2. Biochemical examination: half of the patients may have decreased plasma albumin, increased globulin, and increased globulin to multiple peaks, mainly in the gamma globulin fraction, or increased in alpha or beta globulin, and globulin can be as high as 40 ~ 60dl, combined with polymyositis and systemic sclerosis is more obvious.
3. Immunological examination: IgM, IgA and secretory IgA are elevated. Individual patients can find macroglobulin and cold agglutinin. Because of the presence of IgG and its complexes in the blood, blood viscosity sometimes increases.
50% to 60% of patients are positive for anti-SSA antibody and/or anti-SSB antibody. More than half of the patients are positive for rheumatoid factor. About 33% of patients are positive for anti-thyroid and anti-smooth muscle antibodies. All patients with positive anti-thyroid antibody are There are anti-stomach cell antibody positive, about half of the patients have anti-saliva gland antibody, about 10% of patients with lupus cells positive, in recent years, there are also precipitated antibodies to lymphoid cell exudate soluble nucleic acid antigen, tuberculin and Dinitrochlorobenzene skin test, lymphocyte transformation test, all suggest that cellular immune function is low.
4. Rose red or fluorescein staining test : 1% rose red or 2% fluorescein for corneal conjunctiva staining, can show the overlap of rheumatism with ulcers, can be found with partial slitting of corneal epithelial cord, corneal debris or table Shallow corneal stroma infiltration.
5. Filter paper test: It is the simplest method to check the tear secretion reduction. Take a 35mm × 5mm filter paper and fold it at a right angle of 5mm at one end. Place the end in the conjunctival sac of the eyelid. After 5min, measure the wet dipping of the filter paper. The length to the fold is 15mm or more for normal people.
6. Salivary gland detection : Determination of salivary secretion (sugar test with sucrose compressed into tablets, 800mg per piece, placed in the center of the tongue, recording the time required for complete dissolution, <30 normal; saliva flow rate measurement: connected by hollow catheter The small suction cup is adsorbed to the unilateral parotid duct opening with negative pressure, and the saliva secretion is collected, normal >0.5ml/min), parotid angiography, 40% lipiodol angiography, gland morphology, damage and atrophy, The contrast agent stayed in the parotid gland, the parotid duct was narrowed or dilated, and the parotid gland was scanned with 131 iodine or 99 m. The radioactivity distribution was observed, and whether the excretion and concentration were delayed or decreased to understand the secretory function, from the lips or nose. Mucosal biopsy to observe gland pathological changes, the above two positives are consistent with xerostomia.
Film degree exam
X-ray inspection
(1) Parotid gland angiography: Injecting 40% iodized oil from the parotid duct into 2-3 ml, which can show irregular expansion and stenosis of the main duct, irregular edges, branch ducts with different degrees of expansion, and 3 to 4 small ducts. The number is significantly reduced or disappeared. In severe cases, the parenchyma is destroyed, the lipiodol is retained, and the acinus is punctate, small spherical or cotton-like.
(2) chest radiography or CT examination: lung changes in a variety of, extensive network, nodular or patchy infiltrating lesions, with the bottom of the lungs, hilar lymph nodes, sometimes with pneumonia, pleurisy Or the lungs are not stretched.
(3) Skeletal radiography: The small joints of the limbs are changed to osteoporosis.
2. Parotid ECT examination: After intravenous injection of radionuclide 99m sputum, the parotid gland is scanned in a positive position to observe its shape and size. Since saliva can concentrate 99m , saliva specimens can be collected at the same time, and the radioactivity count can be measured to reflect salivary glands. The function of this disease is that the salivary gland is inferior.
Diagnosis
Diagnostic identification of Sjogren
diagnosis
The diagnosis of this syndrome should be based on the symptoms of the mouth, dry eyes, and the lacrimal gland, salivary gland secretion examination, parotid angiography and histological examination. The following diagnostic criteria are available for reference:
1. The diagnostic criteria for xerostomia are dry mouth and the following test abnormalities:
(1) Salivary flow rate When the salivary gland has lesions, the unstimulated natural salivary flow rate is reduced, and the normal values of each age group are different, with an average of >0.6 ml per minute, and the elderly are lower.
(2) The ductal and small glands are damaged when the parotid gland is glandular.
(3) lymphatic infiltration in the glandular tissue of the lower lip biopsy of the labia gland, where 50 or more cells are aggregated into a heap called a foci, and one or more focal cell infiltrates are abnormal. .
(4) Radionuclide When the salivary gland function is low, its intake and excretion are lower than normal.
The above should exclude the effects of the elderly and other diseases, and 2 of the 4 abnormalities are dry mouth signs.
2. Diagnostic criteria for dry keratitis
(1) Sehirmer test (Sehirmer test) 5 min filter paper wet length > 15 mm is normal, l0mm is abnormal.
(2) The tear film breakup time (BUT) is abnormal for less than 10 s.
(3) Corneal staining Under the slit lamp, the cornea staining point 10 points is abnormal.
(4) Conjunctival biopsy Focal lymphocyte infiltration in the conjunctival tissue is abnormal.
Two of the above four abnormalities were dry keratitis.
3. There is no unified international diagnostic standard for primary Sjogren's syndrome. The following criteria also apply to the incidence of disease in China:
(1) European Diagnostic Criteria (1992):
1 There are more than 3 months of dry eyes or sandy eyes, or more than 3 artificial tears per day, one of them is positive.
2 There are more than 3 months of dry mouth, or when you go to dry food, you need to send it with water, or there are recurring or persistent parotid gland enlargement, one of which is positive.
3 filter paper test 5mm / 5min or corneal staining index 4 is positive.
4 The cell infiltration of the lower lip mucosa biopsy was positive for 1/4 mm2.
5 parotid angiography, salivary gland radionuclide scan, one of the salivary flow rate, which is positive.
6 serum anti-SSA, anti-SSB antibody positive, ANA, RF any one positive.
At least 4 of the above 6 items, except for other collagen diseases, lymphoma, AIDS, sarcoidosis, graft-versus-host disease, can be diagnosed as primary Sjogren's syndrome, there is a certain connective tissue disease At the same time, there are 1 or 2 above, and 2 of the 3 to 5 are positive, which can be diagnosed as secondary Sjogren's syndrome.
(2) Copenhagen diagnostic criteria (1976-1977) confirmed cases of patients with xerostomia and dry keratitis, and excluded the existence of another classification of connective tissue disease.
There are at least two of the following test abnormalities in a dry mouth: unstimulated salivary flow rate decline; labial gland inflammation contains more than one focal lymphocytic infiltration; salivary gland isotope contrast abnormalities.
2 Dry keratitis There are at least two of the following test abnormalities: the filter paper test is lower than normal; the tear film rupture time is lower than normal; the cornea staining point is increased.
(3) Diagnostic criteria proposed by Dong Yi (1991):
1 dry keratitis with Copenhagen standards.
2 mouth dryness with Copenhagen standards.
3 anti-SSA antibody positive or anti-SSB antibody positive or ANA > 1:20 or RF > 1:20.
Those with the above three items and other connective tissue diseases, lymphoma, AIDS, GVH and other diseases are diagnosed; two are possible.
Differential diagnosis
Pay attention to the identification of diseases that cause swelling of the parotid gland.
1. Systemic lupus erythematosus: Although the disease and SS have in common that both are autoimmune rheumatism, anti-nuclear antibodies, anti-RNP antibodies, anti-SSA antibodies and anti-SSB antibodies are positive, but by examining anti-dsDNA antibodies Anti-Sm antibody and clinical manifestations are not difficult to identify, it should be noted that 60% of patients overlap.
2. Rheumatoid arthritis: The common feature of both diseases is that rheumatoid factor can be positive. The joint disease of rheumatoid arthritis is an aggressive arthritis. It is easier to distinguish from SS, but should pay attention to 60%~70. % rheumatoid arthritis patients overlap with SS.
3. renal tubular acidosis: for unexplained renal tubular acidosis, should be highly alert to the possibility of SS secondary.
4. Epidemic mumps: more common in children, is epidemic, contact with the source of infection after 2 to 3 weeks of incubation period, the disease is not repeated, the symptoms are relieved in about 1 week, sometimes accompanied by arthritis, arthritis Can be reduced in a few weeks.
5. Suppurative mumps: more common in adults and diabetic patients, when the body's resistance declines, most of them are one-sided, with fever, increased white blood cells and local obvious inflammation.
6. Parotid malignant tumor: unilateral slow increase, such as invasion of facial nerve, can cause facial nerve paralysis.
7. Chronic granuloma: Chronic granuloma of the parotid gland caused by tuberculosis, sarcoidosis, mold, identification is more difficult, sometimes rely on pathogens and pathological examination to identify.
