Ms. Wang, 50 years old, complained of recurrent dry mouth and eyes for more than 10 years, accompanied by recurrent rashes on both lower limbs, and had been treated by stomatology, gastroenterology or dermatology, and also visited ophthalmology, but all had no obvious effect and was very painful. His children found out on the Internet that the disease could be treated at the Department of Rheumatology and Immunology, and they did relevant examinations in the hospital and were finally diagnosed with dry syndrome. Li Songwei, Department of Rheumatology, The First Affiliated Hospital of Henan College of Traditional Chinese Medicine
So what is dry syndrome? I will give you a brief introduction today.
Dry syndrome is a chronic inflammatory autoimmune disease that mainly involves the exocrine glands of the whole body, and it is also a common disease and multi-morbidity in the rheumatology department, mainly involving salivary glands and lacrimal glands, mainly manifesting as dry mouth and eyes. However, the respiratory system, digestive system, skin, vagina and other exocrine glands are often involved in the corresponding performance, and some of them also have blood system, urinary system, nervous system damage, etc..
Causes: Firstly, genetic factors, research shows that there are certain susceptibility genes for dry syndrome, and the risk of the immediate family members of dry syndrome patients to suffer from the disease is often higher than normal population; secondly, environmental factors, research proves that viral infection may induce the disease, such as EBV; and sex hormones, the disease mostly occurs in women, suggesting that the high level of estrogen may participate in the development of the disease.
Pathogenesis: The disease is an autoimmune disease, in which genetic factors, environmental factors and sex hormones are involved, resulting in abnormal cellular and humoral immune responses, which further cause tissue damage through various cytokines and inflammatory mediators.
Clinical manifestations.
I. Exocrine gland lesions.
(i) Superficial exocrine gland lesions.
1.Dry mouth: It is often the first symptom, seen in 80% of the patients, in serious cases, dry food must be sent down with water. 40% have salivary gland enlargement, parotid gland enlargement is more common, repeated parotid gland enlargement is mostly seen in young primary dry syndrome patients. 50% of the patients, due to saliva reduction, teeth lose nutrition, small flakes or powder-like loss, called “rampant tooth”. “This is one of the typical manifestations of dry syndrome.
2.Dry keratitis: manifested as dry eyes, less tears or even no tears, itching and pain, photophobia, burning sensation, foreign body sensation, etc. In serious cases, the cornea is cloudy, ulcerated or perforated.
3.Dry and itchy skin.
4.Dry nasal cavity and pharynx, decreased sense of smell.
5.Dry vulva and vagina, leading to painful sex and easy to secondary mycobacterial infection.
(II), visceral exocrine gland lesions.
1, respiratory system: 17% of patients may have dry cough performance, 30%-50% of patients have interstitial lung lesions, and severe cases have pulmonary alveoli, which is the main cause of death from the disease.
2. Digestive system: Gastrointestinal symptoms are common, mainly manifested as difficulty in swallowing. The liver is enlarged, recurrent liver enzymes are elevated, and the pathology is autoimmune hepatitis, which is easily misdiagnosed or missed in the clinic.
3.Urological system: about 30% of patients with dry syndrome have renal lesions, 90% of which are damage of distal renal tubules. Urine concentration disorder will show polyuria and irritability; acidification dysfunction will show hypokalemic paralysis, metabolic bone disease (osteoporosis, fracture, etc.), and it is reported that 30%-40% of hypokalemia is related to desiccation syndrome.
(c), lymphoma: 5%-10% of patients have enlargement of lymph nodes, and at least 50% have infiltration of a large number of lymphocytes in the internal organs during the course of the disease. Be highly alert to the formation of lymphoma within 5 years of the appearance of dry syndrome. Macroglobulinemia and hypergammaglobulinemia often precede the development of lymphoma, and after the development of lymphoma globulin levels decline or return to normal and autoantibodies disappear. When persistent enlargement of spleen, parotid gland and lymph nodes with cough, lung nodules and Raynaud’s phenomenon occurs, be highly alert to the appearance of lymphoma.
Second, lesions other than exocrine glands.
1. Skin lesions: purpura-like rash is the most common, mainly on the lower limbs, heavily on the buttocks, and rarely on the upper limbs, with pigmentation after fading.
2, joint muscle lesions: 70% of patients will have joint pain, but rarely deformation of joints.
3. Neurological lesions: 10% of patients will develop neurological damage, including central nervous system and peripheral nervous system lesions. The manifestation is cranial neuritis, stroke, epilepsy, multiple sclerosis, mental consciousness disorder, etc.
4, Raynaud’s phenomenon: seen in 13% of patients.
5.Low thyroid function: seen in 15-20% of patients
6.Hematological system lesions: 25% of the patients showed anemia and 15% of the patients showed significant decrease in platelets, especially the latter is one of the main causes of death in patients with dry syndrome.
Laboratory tests.
1.Hyperglobulinemia: It is one of the characteristics of this disease. Especially, IgG is the most obvious, and its elevated degree is often positively correlated with clinical manifestations.
2. Autoantibodies: 75% of patients are positive for antinuclear antibodies, with the highest rate of anti-SSA and anti-SSB positivity, 75% and 52%, respectively, and higher specificity for anti-SSB. Positive antithyroglobulin antibodies suggest damage to the thyroid gland, and positive antimitochondrial antibodies often suggest liver lesions. About 3/4 of patients have positive RF.
3, salivary gland examination: salivary flow monitoring; parotid angiography.
4, lacrimal gland examination: filter paper test, corneal staining test.
Treatment.
There is no ideal treatment for this disease in Western medicine, mainly symptomatic treatment to relieve symptoms. Hormones and immunosuppressants are given to those with significant combined internal organ damage.
Chinese medicine has obvious advantages in treating the disease, especially in improving the damage caused by the patient’s exocrine gland lesions. According to Chinese medicine, this disease belongs to the category of “dryness paralysis”, and the general pathogenesis is “dryness is dry when it is victorious”, mainly because dryness damages the yin of the stomach, lung, liver and kidney, resulting in the imbalance of yin and yang of the five organs and yin deficiency and yang hyperactivity. The dry weather in autumn can easily trigger or aggravate the problem. In terms of treatment, Chinese medicine believes that “dryness is moistened”, the treatment should be to nourish Yin and clear heat, depending on the different internal organs involved, or nourish blood and soften the liver, or nourish the kidneys and nourish Yin, or nourish the stomach and produce fluid, or moisten the lungs and nourish Yin, or the lungs, stomach, kidneys and liver simultaneously. At the same time, it is often necessary to remove blood stasis and open the ligaments throughout. As long as the identification is accurate, we must keep the formula and observe the law, and more than serve the party can be effective.
In addition, the diet should be light, avoid spicy and fatty, sweet and thick-tasting products; maintain an optimistic, positive attitude; appropriate exercise. These aspects are also beneficial to the improvement and prevention of the disease.
This article is authorized by Dr. Songwei Li.