Overview
Sjogrens syndrome (SS) is an autoimmune disease with high lymphocytic infiltration of the exocrine glands, especially the lacrimal and salivary glands. In addition to the exocrine glands, other organs may also be involved, with corresponding dysfunction. The disease is also known as autoimmune exocrine gland epithelial cell infection or autoimmune exocrinopathy because the immune inflammatory response is mainly manifested in the epithelial cells of the exocrine glands. In addition to dry mouth and eyes due to decreased function of salivary glands and lacrimal glands, other exocrine glands and other organs outside the glands are also involved, resulting in multisystem damage. The disease is characterized by a variety of autoantibodies and hyperimmunoglobulinemia in the serum. The disease is divided into primary and secondary, secondary to rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, etc. is called secondary dry syndrome (2°SS), and those who do not combine other autoimmune diseases are called primary dry syndrome (1°SS). This section mainly describes primary desiccation syndrome.
Primary desiccation syndrome is a global disease, and the prevalence in our country is 0.3%~0.7%, and the prevalence in the elderly group is 3%~4%. The disease is more common in women, and the ratio of men to women is 1:9~20. The age of onset is mostly from 40 to 50 years old. It is also seen in children.
Dry syndrome has no similar name in Chinese medical literature, but in 1989, the National Committee of Chinese Medicine Paralysis Specialists named it “dry paralysis”. It is usually caused by internal heat and fluid injury, or internal deficiency of essence and blood after prolonged illness, or excessive blood loss, or fluid loss after sweating, vomiting, or downward injury, resulting in deficiency of kidney essence and deficiency of the five organs.
Clinical manifestations
1, dry mouth due to reduced saliva secretion. Patients often drink water frequently, and in severe cases have difficulty eating. In 40% of patients, the salivary glands are symmetrically enlarged and recurrent, with smooth surface and not hard texture. If the gland is hard and nodular, it should be alerted to malignant change.
2, dry eyes due to low lacrimal gland secretion function. Dry eyes, foreign body rubbing sensation, with increased secretion, serious cases may lead to corneal ulceration, or even perforation, blindness.
3. Skin mucosa Dry, itchy skin, dry nasal and vaginal cavity. There may be urticaria-like rash, erythema nodosum and purpura-like rash due to hyperglobulinemia. In addition to dryness, mucous membrane damage may also appear as ulcers.
4, joint muscle 70% to 80% of patients have arthralgia, only 10% of arthritis, joint destruction is rare. There may be muscle weakness, elevated muscle enzyme profile and electromyography changes.
5.Respiratory system 50% of patients have alveolar inflammation, a few patients have interstitial lung fibrosis, lung function decreases, with the progress of the disease, chest tightness, wheezing and other symptoms.
6.Digestive system Difficulty in swallowing can occur due to sore throat, esophageal dryness and esophageal motility disorders. The decrease in glandular secretion is easily complicated by chronic atrophic gastritis, and in a few cases, acute and chronic pancreatitis. 25% of patients have liver function impairment, increased transaminases, and even jaundice, and hepatosplenomegaly is common. Some patients develop intractable diarrhea.
Renal damage About 50% of patients have renal damage, 90% of which are distal tubular involvement, causing type I renal tubular acidosis, which is characterized by periodic hypokalemic paralysis, nephrogenic chondropathy and nephrogenic uremia, with a daily urine output of more than 3000ml. Proximal tubular involvement is rare, and a few patients have glomerulonephritis, with a poor prognosis.
8. Neurological system Vasculitis in different parts of the body can cause lesions in the central nervous system and peripheral nervous system. The incidence of central nervous system is low, mostly temporary dysfunction, but can also form nerve damage; peripheral neuropathy is more common, mainly involving the trigeminal nerve and other sensory fibers, but can also involve motor nerves, resulting in sensory hypersensitivity, sensory loss or motor impairment.
9, lymphatic tissue hyperplasia Most lymphatic tissue hyperplasia is benign. It should also be alerted to the possibility of malignancy, because the incidence of lymphoma is 44 times higher than that of normal people.
Ancillary tests
1.Anti-nuclear antibody (ANA) The positive rate is 50%~80%, and a variety of anti-nuclear antibodies can be detected in the serum of SS patients, such as anti-SSA antibody, anti-SSB antibody, anti-RNP antibody, etc. Among them, anti-SSA antibody and anti-SSB antibody have the highest positive rate and are the more specific antibodies for the diagnosis of SS.
2, rheumatoid factor (RF) 70%-90% of patients are positive for RF.
3, hyperglobulinemia is another feature of the disease, most patients have significant hyperglobulinemia, polyclonal. When the immunoglobulin increase from polyclonal to monoclonal, be alert to malignant lymphoproliferation.
4, urinary pH is increased, when SS combined with renal tubular acidosis, urinary pH is increased, the general urinary examination pH is greater than 6.5.
Diagnostic criteria
1.Diagnostic criteria of dry mouth sign Any person with abnormalities in 2 of the following 4 tests can be diagnosed as dry mouth sign.
(1) Salivary flow rate: unstimulated salivary flow rate <0.06ml/min, which is different in each age group, and lower in the elderly.
(2) Parotid gland imaging: There are signs of destruction of the ducts and small glands in parotid lesions.
(3) Labial gland biopsy: ≥1 focal lymphocyte infiltration in the tissue of the lower lip biopsy is considered abnormal. 4mm2′ aggregation of ≥50 lymphocytes is considered 1 focal point.
(4) Radionuclide imaging: when salivary gland function is low, its uptake and secretion are lower than normal.
2, dry keratitis diagnostic criteria Anyone with 2 abnormalities in the following 4 tests can be diagnosed as dry keratitis.
(1) filter paper test (schirmer test): 5 minutes filter paper wetting length ≥ 15mm is normal, 5 minutes wetting length ≤ 10mm is abnormal.
(2) tear film breakup time (BUT): any shorter than 10 seconds is abnormal.
(3) Corneal staining: corneal staining point more than 10 under slit lamp is abnormal.
(4) Conjunctival biopsy: focal lymphocyte infiltration in the conjunctival tissue is abnormal.
3.At present, there is no unified diagnostic standard for SS internationally, and the following are the international classification (diagnosis) standards in 2002 (Table 1 and Table 2):
Table 1 Classification standard items of dry syndrome
Oral symptoms: 1 or more of 3 items
1. Daily dry mouth for more than 3 months
2.Recurrent or persistent enlargement of parotid glands in adulthood
3.Swallowing dry food with the help of water
II. Eye symptoms: 1 or more of the 3 items
1. Daily unbearable dry eyes for more than 3 months
2.Repeated sand in the eye or abrasive sensation
3. Need to use artificial tears every day
III. Ocular signs: Positive for any one or more of the following tests
1.Schimer I test(+)(≤5mm/5min)
2, corneal staining (+) (≥4van Bijsterveld scoring method)
Ⅳ. Histological examination: pathology of the lower lacrimal gland showed lymphocytic foci ≥ 1 (refers to 4mm2 tissue with at least 50 lymphocytes aggregated in the interstitial lacrimal gland as a foci)
V. Salivary gland damage: positive for any 1 or more of the following tests
1. Salivary flow rate (+) (1.5 ml/15 min)
2.Parotid gland imaging(+)
3.Salivary gland radionuclide test(+)
VI. Autoantibodies: anti-SSA antibody or anti-SSB antibody (+) (double diffusion method)
Table 2 Specific classification of items in Table 1
1.Primary dry syndrome Without any underlying disease, the diagnosis can be made if the following 2 items are present:
4 or more items in Table 1, but must contain entry IV (histological examination) and/or entry VI (autoantibodies), and positive for any 3 of the 4 entries III, IV, V, VI
2.Secondary dry syndrome Patients with underlying disease (such as any connective tissue disease), while meeting any 1 of entries I and II of Table 1, and any 2 of entries III, IV and V
3. Must exclude History of cervical head and facial radiation therapy, hepatitis C virus infection, AIDs, lymphoma, nodular disease, GVH disease, application of anti-acetylcholine drugs (such as atropine, scopolamine, bromoproterenol, belladonna, etc.)
Differential diagnosis
1, systemic lupus erythematosus Dry syndrome is mostly seen in middle-aged and elderly women, fever, especially high fever is uncommon, no zygomatic rash, dry mouth and eyes are obvious, renal tubular acidosis is its common and main renal damage, hyperglobulin is obvious, hypocomplementemia is rare, good prognosis.
2, rheumatoid arthritis dry syndrome arthritis symptoms are far less obvious and serious than rheumatoid arthritis, rarely have joint bone destruction, deformity and functional limitation. Rheumatoid arthritis rarely appears anti-SSA antibodies and anti-SSB antibodies.
3, non-autoimmune diseases of dry mouth, such as aging exocrine gland function decline, diabetic or drug-related dry mouth depends on the medical history and the characteristics of each disease to distinguish.
Treatment】
I. General treatment
1.Health education Educate the patients to understand the disease correctly, make them understand the principles of treatment and the usage of drugs and adverse reactions, actively cooperate with the treatment, and ask them to avoid wind, cold and dampness, take good care of the skin, eyes, mouth and respiratory tract, and eat food rich in potassium ions.
2.Sports medical treatment Patients are advised to carry out regular sports medical treatment to prevent the occurrence of osteoporosis and to maintain the flexibility of joints.
II. Drug treatment
(A) Chinese medicine treatment
1.Evidence-based treatment
(1) Dryness injures lung yin and paralysis of lung qi
Symptoms: dry cough with itchy throat, chest tightness and shortness of breath, thick and sticky phlegm, or blood in the phlegm, small amount and dark color; or hoarse voice, dry nose and little runny nose, or afternoon redness of the cheek, hot flashes and night sweats, heat in the heart of the hands and feet, fatigue and pain in the dorsal region, gradually losing weight, dry skin and fur, or local skin numbness. The tongue is red with little coating and lack of fluid, or the tongue is light and flaky, and the pulse is fine or sunken.
Treatment: Promote fluid and moisten dryness, lightly clear and promote lung.
Remedy: Clear the dryness and save the lung with addition and subtraction.
(2) Paralysis of the heart vessels due to dryness and heart yin.
Symptoms: palpitations and palpitations, restlessness, agitation, dreaminess and wakefulness, dull pain in the chest, or burning pain, or pain leading to the back of the shoulder and the inner arm, with intermittent onset and cessation. Dryness of the mouth and tongue, heat in the hands and feet, night sweating. Tongue red and less fluid, or with petechiae, no moss or less bitter, or tongue light and flaking, pulse fine or fine and astringent with knots and generation.
Treatment: Benefit qi and nourish yin, generate fluid and moisten dryness.
(3) Dryness injures stomach yin, spleen deficiency and muscle paralysis
Symptoms: hunger without appetite for food, or food does not dissolve; noisy stomach and epigastric, or vague pain, or erratic dry vomiting, dry mouth and throat, distraction, or dry stools, emaciation, or even muscle atrophy, weakness of the limbs, unhealthy gait. The tongue is dark red and less fluid, or the tongue is flaking, with thin yellow moss or no moss, and the pulse is fine or thin and astringent.
Treatment: Nourish the spleen and benefit the stomach, promote the production of fluid and moisten dryness.
Remedy: Nourishing the spleen and moistening the stomach with addition and subtraction.
(4) Dryness injures the yin of kidney and liver and paralysis of tendons and veins.
Symptoms: headache and dizziness, facial heat, dry eyes, dry mouth and throat, red lips and cheekbones; tendons and flesh, joint pain, unfavorable flexion and extension; irritability, pain in both sides of the body, five heartburn, hot flashes and night sweats, insomnia and dreaminess, constipation and urination, thinning of the body, low menstruation or amenorrhea in women. The tongue is red and less fluid, or the tongue is dark red or stasis purple, with little or no moss or flaking moss, and the pulse is fine or sunken.
Treatment: Nourish Yin and moisten dryness, glorify tendons and open ligaments.
Remedy: Zhi Bai Di Huang Wan plus or minus.
2.Treatment of proprietary Chinese medicine
(1) For those with cold symptoms: Chuanbei Loquat Ointment, compound fresh bamboo liquid and Qiju Dihuang Wan can be used to reduce dry cough and dry eyes.
(2) For those with heat symptoms: it can be combined with Bai Shao total glycoside capsule.
(3) Chinese herbal tea drink: If the patient complains of dry mouth and dry throat, clear heat and throat drink can be given: 6g of honeysuckle, 6g of maitake, 6g of jade butterfly, 2 pieces of Tibetan green fruit.
(4) Intravenous medication.
For those with blood stasis symptoms, intravenous drip can be used to activate blood circulation, resolve blood stasis and promote circulation.
3.External treatment method for identification of evidence
(1)For those with cold symptoms: Chinese herbal hot compress + ion guide, ultrasonic drug penetration, medicine jar therapy + electromagnetic therapy.
(2) For those who are partial to heat: Chinese medicine wet wrap, semiconductor laser treatment, ultrasonic drug penetration.
Western medicine treatment
(I) Local treatment
1.Treatment of dry mouth
2.Treatment of dry eyes
3.Treatment of muscle and joint pain .
4.Correction of hypokalemia
(II) Treatment of systemic damage
(C) biological agents
(IV) Other treatments
[Conditioning]
1, psychological conditioning The incidence of anxiety and depression in pSS patients is significantly higher than that in the normal population, and the relatively low function of the neuroendocrine axis (adrenal, gonadal and thyroid axis) can partially explain the mood disorders seen in pSS patients. pSS patients are most commonly characterized by melancholic traits, hysteria and depressive tendencies, indicating that pSS patients carry personality changes and are prone to depression, anxiety and other psychological disorders. It is suggested that pSS patients have been suffering from serious psychological distress for a long time and have obvious depression, anxiety and other emotional manifestations. pSS is a chronic disease with a long course and no cure at present, prone to recurrent attacks, and multi-system damage can occur, causing a serious decline in the quality of life of patients, bringing heavy psychological pressure on patients, and adversely affecting their cognition, mood and behavior, especially some patients who lack medical knowledge may even Especially some patients who lack medical knowledge may even have fear and suspicion, thus hindering the communication between doctors and patients.
For patients with dry mouth, avoid smoking and drinking alcohol, and avoid taking drugs that cause dry mouth to aggravate; pay attention to oral hygiene and good oral care, always use toothpicks to remove food residues after meals, and rinse your mouth regularly to reduce dental caries and oral secondary infection. For dry, flaky and itchy skin caused by the involvement of sweat glands, use less or no alkaline soap, and choose neutral soap. You can use compound glycerin anti-itch milk, vitamin E milk and commercially available moisturizer, etc. all have good skin protection effects. Change your clothes and bedding regularly to keep your skin clean. Keep the humidity in the room at 50% to 60% and the temperature at 18℃ to 21℃ to relieve the symptoms of dry cough caused by dry respiratory mucosa and to prevent infection. It is advisable to eat easily digestible food, pay attention to the reasonable allocation of nutritional structure, and eat a light diet. Avoid eating fatty, sweet, thick and spicy foods.
3.Treatment and care
(1) oral care: when oral ulcers occur, you can first use saline cotton balls to scrub the local, and then 5% metronidazole, avoid the use of nail violet, so as not to aggravate the symptoms of oral dryness. For oral secondary fungal infection, topical mycobacterial tablets 500,000 units dissolved in 500 ml of saline, 10-20 ml each time, 3-4 times daily gargle, or give 4% sodium bicarbonate solution 10-20 ml each time, 3-4 times daily gargle, serious cases can be given fluconazole 50 mg daily, for 7-14 days.
(2) Eye care: Avoid the application of agents that reduce tear secretion such as diuretics, antihypertensives, and antidepressants.
(3) Skin care: Those with skin lesions in primary dry syndrome should be cleared and changed according to the skin lesions, and antibiotics can be used appropriately in case of infection. If you have dry and itchy vagina and burning pain during sexual intercourse, you should pay attention to the hygiene of your pubic area, and you can use cleansing lotion or lubricant such as glycerin and castor oil.
(4) respiratory care: patients with sticky sputum that is difficult to cough up can do nebulized aspiration. If necessary, antibiotics and chymotrypsin can be added to control infection and promote sputum excretion.