undifferentiated connective tissue disease

Undifferentiated Connective Tissue Disease Possesses the clinical manifestations of certain connective tissue disorders, but does not meet the diagnostic criteria for any one particular disorder. It may belong to the early stages or tonic type of a particular diffuse connective tissue disease, and in some patients may Health Search be a separate disease. UCTD has not been found to have characteristic clinical manifestations and specific laboratory indicators.The diagnosis of UCTD is mainly based on clinical manifestations, and further academic research is still needed to determine whether this disease is another connective tissue disease.The concept of UCTD was first proposed by LeRoy et al. in 1980. However, it was not until after 1989 that the number of related clinical studies gradually increased. Some people call it cryptogenic lupus, incomplete or tonic lupus, early undifferentiated connective tissue disease and undifferentiated connective tissue syndrome, etc. The diagnostic criteria recommended by each researcher are not completely consistent. More studies have been conducted abroad on the clinical features laboratory abnormalities of this disease and its regression. Epidemiology There is no epidemiological survey data on undifferentiated connective tissue disease outside China Preliminary clinical studies have shown that UCTD is not uncommon and should be given full attention by clinicians, especially rheumatologists. Published clinical studies have shown that UCTD onset occurs between the ages of 18 and 67 years, and is more common in women of childbearing age. Gender is related to the onset of the disease, with a male to female ratio of 1:4 to 1:6. The disease can be seen in all races. Some foreign studies have suggested that the incidence may be higher in the white race. The prevalence of UCTD reported in the literature varies considerably due to the selection of different study subjects.In 1991, Alarcon et al. reported that 52% (213 cases) of 410 patients with connective tissue disease with a disease duration of less than 1 year of health searching were compatible with Early Undifferentiated Connective Tissue Disease (EUCD).Mosca et al. counted 91 connective tissue disease patients who were diagnosed in the period of 1979-1998, and about 20% of them were found to be with EUCD. Mosca et al. counted 91 cases of connective tissue disease seen between 1979 and 1998, of which about 20% were found to have UCTD, a significantly lower prevalence than in the former study, probably due to differences in the diagnostic criteria and disease duration of the candidates in these two studies. In the study by Alarcon et al, the duration of the disease was less than 12 months, and most of the patients had no specific clinical manifestations, some of them had negative ANA, and 10% of the patients with predominant polyarthritis had complete remission within 1 year. Therefore, some patients initially diagnosed with UCTD may have some type of arthritis. Etiology unknown The etiology of UCTD is unknown and few studies have been done but some researchers believe that some UCTD may be an early stage of systemic lupus erythematosus (SLE) or systemic sclerosis (SSc) and therefore the etiology of the disease should be the same as these two diseases. Research suggests that the development of this disease may be the result of some environmental factors such as long-term exposure to chemical agents acting on susceptible individuals. Both environmental and genetic factors play an important role in the pathogenesis of the disease. In 1998, Lacey et al. conducted a study on 205 UCTD patients and 2095 normal controls. It was found that 25% (52/205) of the 205 female Undifferentiated Connective Tissue Disease UCTD patients had a definite history of exposure to chemical solvents, whereas only 17% (364/2095) of the 2095 controls matched for racial composition, education, marital and economic status, and smoking and alcohol habits had a definite history of exposure to chemical solvents. A detailed study of the history revealed that chemicals, cosmetic products, drugs, rubber products, paints and pigments were significantly associated with the development of UCTD. Exposure to paints, detergents and turpentine may also be associated with the development of UCTD.The study by Lacey et al. further suggests that medical implants such as catheters, artificial joints and metal fixation brackets for orthopedic surgeries may also increase the risk of developing the disease. All these studies suggest that environmental factors play an important role in the development of UCTD. Pathogenesis As with most connective tissue diseases, there is a genetic basis for the development of UCTD. Some studies have shown that some patients have a family history of autoimmune disease. 1988 Ganczarczyk et al. compared the HLA subtypes of 22 patients with UCTD and 211 cases of SLE, and found that the positivity rate of HLA-B8 and HLA-DR3 subtypes in patients with UCTD was significantly higher than that in normal subjects. In contrast, the 7 patients who eventually progressed to SLE had significantly lower HLA-DR1 subtype positivity rates compared with normal subjects similar to those of SLE patients. This suggests that the HLA-DR1 subtype may be an anti-UCTD gene. A study by Mosca et al. found an increased rate of recurrence during pregnancy in patients with UCTD. Of the 22 study subjects, 6 (24%) had a recurrence or exacerbation of the disease during pregnancy, whereas only 7% of the control group had a recurrence of the disease. Therefore, changes in sex hormone levels or imbalances in the estrogen/progesterone ratio are likely to be involved in the development of this disease. Clinical manifestations In general, the disease often has an insidious onset, with an average time from onset of clinical symptoms to presentation of the disease of 2 to 3 years, or 38 months on average. The clinical manifestations of undifferentiated connective tissue disease are often mild, and non-specific symptoms such as malaise, low-grade fever, and enlarged lymph nodes are common. Some larger clinical investigations have found that arthralgias, Raynaud’s phenomenon, and skin and mucous membrane lesions are the most frequent symptoms, while involvement of vital organs such as the kidneys and central nervous system is rare. During follow-up, it was found that the clinical symptoms of the patients fluctuated with the course of the disease and treatment, mostly showing a trend of gradual remission, but in general the activity of the disease did not change much. All tissues and organs of the human body are composed of connective tissue, which shows how important connective tissue is to a human body! Many diseases that go into multifunctional failure affect the connective tissue, so the general term connective tissue disease is not appropriate. Undifferentiated connective tissue disease or undetermined connective tissue disease. There are one or two clinical manifestations of connective tissue disease, the most common is mild muscle pain in the joints of the limbs, morning stiffness is not obvious, or there is atypical Raynaud’s phenomenon, usually diagnosed as rheumatoid arthritis in general. positive ANA, or RF, or SS-A, or Rib, can not be diagnosed as which connective tissue disease. The diagnosis can only be made after the evolution of the disease over a period of time. This is an early and mild manifestation of connective tissue disease, which is the best time for TCM treatment. Controlling the disease with Chinese medicine can achieve complete relief and nip the disease in the bud. This disease is equivalent to the category of paralysis in Chinese medicine. The treatment is based on the method of lupus erythematosus and rheumatoid arthritis, which is based on nourishing yin and clearing heat, and combining with clinical manifestations to add flavor. For example, for joint pain, add Gang Nim Root, Qiang Wu, Wei Ling Xian, etc.; for mouth ulcers, add Tu Fu Ling, Chuan Lian, etc.; for Raynaud’s phenomenon, add Dang Pi, Chuan Xiong, Gui Mu Fei, etc.; for dry mouth, add Dendrobium, Fresh Maoguan, Fresh Lutong Root, etc. If you use western medicine, you can use immunosuppressive drugs such as azathioprine, and do not use corticosteroids too early. According to Yao medicine, the factors that cause the normal balance of surplus and deficit of the human body to be destroyed and the disease occurs are the causes of the disease. Yao medicine has a long history of more than two thousand years. Yao medicine’s understanding of the causes of disease can be traced back to the understanding of miasma in the Han Dynasty, and since then, with the continuous development of Yao medicine, Yao medicine’s understanding of the causes of disease has continued to progress and develop. There are two ways to find out the cause of disease in Yao medicine: one is to inquire in detail about the onset of the disease to understand the direct or indirect cause of the disease; the other is to analyze the symptoms and signs of various diseases and find out the cause of the disease based on the symptoms and signs of various diseases. Due to the particularity of the living environment of the Yao people, the understanding of the cause of disease in Yao medicine includes not only the causes of disease generally recognized by other medical science, that is, the general cause of disease, but also some causes of disease with regional characteristics, such as poison, gua sha, miasma, compulsion, etc. However, no matter how to categorize the cause of disease, Yao medicine has always believed that the cause of disease is nothing but the cause of disease from the outside, and from the inside to the two ends of the birth of the disease, and this understanding, although a little bit rough, in the aetiology of the disease, but it can be played a simple and simple role in managing the complexity of the cause of the disease. Yao medicine treatment of connective tissue diseases adhere to the elimination of because to be, that is, in the treatment of disease, must be for the name of the disease, looking for the root cause of the disease, and then the use of drugs or other means to get rid of disease-causing factors or disease-causing substances, so that the evil go to the positive security. The premise is to examine the cause of the disease, and the purpose is to get rid of the cause of the disease. The occurrence and development of any disease is always shown through a number of symptoms and signs. To treat a disease, the patient’s symptoms and signs should be analyzed and summarized, according to the cause of the disease, taking into account the body’s constitution, determining the location of the disease, distinguishing different pathological characteristics, and then administering treatment. Symptoms and signs are the basic indexes and factors for Yao medicine to identify diseases. Yao medicine analyzes these specific symptoms and signs, finds out the root cause, determines the name of the disease, and treats the disease with special prescription. Yao medicine believes that symptoms are the symptom of disease, and the cause of disease is the root cause, and the two are cause and effect relationship. In the process of disease development, there will be neither symptom without cause nor cause without symptom, but it is either clear or obscure, so it is especially important to recognize it in detail. The relationship between the cause and symptoms is dialectical unity, they are interconnected, interdependent, and their manifestations are often very complex. A cause of disease in different conditions, can produce a variety of symptoms, and a disease is often caused by a variety of reasons. Therefore, in clinical practice, for some complex cases, the patient’s cause of disease, onset and treatment should be investigated in detail. In clinical practice, Yao medicine attaches great importance to the search for the cause of disease, and pays attention to understanding some possible causes of disease perceived by the patient, if these causes and symptoms can be clarified, the accuracy of diagnosis can be improved, so as to carry out the cause of the treatment and improve the therapeutic effect. The causes of disease recognized by Yao medicine include those that exist in nature and in the human body, such as gua sha, miasma, parasites, poisons, wind, consumption, stasis, cold, heat, etc. The purpose of searching for the causes of disease is to find out the possible causes of disease perceived by the patient. The purpose of searching for the cause of disease is to treat the disease, therefore, in the clinic, Yao doctors pay more attention to how to get rid of the disease and make the sick body recover. Yao doctors believe that diseases arise because evils accumulate in the body, and the only way to heal the disease is to get rid of the evils. Disease is not a substance that exists in the human body itself, there are two ways to attack people, one is from the outside, one is from the inside, so the human body to restore the balance of the state, must be accumulated in the body of the evil out of the outside. There are three ways for evil to come out of the body: through the sweat pore, through the nose, and through the lower orifices. It should be emphasized that there is a scientific connotation to the elimination of evil qi. The common methods to get rid of the cause of disease include sneezing, drug moxibustion, steaming, fumigation, ironing, acupuncture, bloodletting, gua sha, combing the breast, medicinal baths and so on. Skin lesions 1, skin and mucous membrane lesions. Skin lesions are quite common, and the performance of the rash type is diverse, some patients with rash as the first symptom. Discoid erythema is more common than in SLE patients, appearing in about 34% of patients. It is found in all races, with a higher incidence in black patients. It presents as a red papule above the surface of the skin on exposed areas of the body, most commonly on the head and neck Health Search. Rash size is not equal, the shape is not the same surface with scales after healing often leave scars localized skin atrophy. The incidence of zygomatic erythema is about 10% for the cheeks red macules, can be typical butterfly-like distribution, can also be irregular in shape health search after healing more than scarring. The incidence of photosensitivity is between 13% and 24%. About 18% of patients experience dry mouth and dry eyes Health Search. The incidence of mucosal ulcers is lower than that of SLE, ranging from 3% to 13%. Diffuse swelling of both hands and subcutaneous nodules have also been reported. Joint and muscle lesions 2, joint and muscle lesions. Joint and muscle lesions are more common. About 37% to 80% of patients may present with arthralgia or joints Undifferentiated connective tissue disease Inflammation manifestations, the average incidence of 55%, while the average incidence of arthritis is 42%. It is mostly a noninvasive polyarthritis, and joint destruction and deformity rarely occurs. It can involve all large and small joints of the body, including interphalangeal joints metatarsophalangeal joints mandibular joints, etc., but the large arthritis is more common. It can be accompanied by morning stiffness, but most of the time it is short. The synovial fluid is mostly inflammatory exudate with low cell count, yellow color when protein content is high, and negative bacterial culture. Muscle involvement is often seen as myalgia and muscle weakness in the proximal muscle groups of the extremities. Individual reports may even show mild to moderate elevation of muscle enzymes, but electromyography is not abnormal or mild myogenic damage, muscle biopsy has no obvious abnormality, not in line with the diagnostic criteria of myositis or other connective tissue diseases. Vasculitis Raynaud’s phenomenon 3, vasculitis Raynaud’s phenomenon is one of the most common clinical manifestations of UCTD health search seen in about 50% of patients, and may be the only clinical symptom lasting for many years manifested as episodes of limb pallor, bruising and redness, accompanied by localized pain or numbness before the onset of most of the triggers of the cold or emotional excitement, and gradually relieved in minutes or tens of minutes after the spasm of the small arteries is the basis of its pathology. Long-term frequent episodes of authors can appear local soft tissue atrophy and necrosis and other dystrophic manifestations, severe cases of bone resorption of the extremities. In addition, about 5% of patients can appear hypertension, imaging examination of local vascular wall hyperplasia or stenosis important organs vasculitis such as cardiac vasculitis, renal artery stenosis, arteriovenous embolism, etc.,. Lung and cardiac lesions 4. Plasmacytitis is the most common, but the incidence is slightly lower than SLE, about 11%, can be manifested as pleural effusion undifferentiated connective tissue disease pericardial effusion or both at the same time. The severity of the disease varies, with mild cases having no obvious clinical manifestations and severe cases even presenting with cardiac tamponade Plasmapheresis is often suggestive of fluid leakage, and antinuclear antibodies can be positive. Other pulmonary manifestations are interstitial fibrosis and interstitial pneumonia and other rare manifestations such as endogenous lipoid pneumonia. Interstitial fibrosis has an insidious onset, manifesting as progressive dyspnea, and X-ray examination shows thickening of the lung texture and disordered lung function suggesting reduced diffusion function. Exercise lung function and high-resolution CT of the chest are more sensitive and can assist in early diagnosis. Cardiac lesions can involve the whole heart, including pericarditis, myocarditis and endocarditis, etc. Clinical symptoms include chest tightness, palpitation and dyspnea, etc. Electrocardiogram can have various arrhythmias and ST-T changes, etc. Hematologic lesions 5, hematologic lesions. About 20% of the patients have this lesion can be manifested as white blood cells, thrombocytopenia and anemia to the white blood cells moderate reduction and non-hemolytic anemia is the most common health search. Thrombocytopenia occurs in about 7% of patients and can be quite severe. Individual cases have a marked tendency to bleed, even resulting in death. Hemolytic anemia is rare, mostly anemia of chronic disease and iron deficiency anemia. Decrease in whole blood cells can also be seen. Renal damage 6. The incidence of renal damage is around 11%, which is less common in patients with discoid erythema and negative ANA. Clinical manifestations may include edema, hypertensive proteinuria, hematuria, and elevated serum creatinine levels, but rarely result in severe renal insufficiency. Other neurological damage 7, other neurological damage is rare, can be manifested as migraine, convulsions behavioral abnormalities and hallucinations and other psychiatric symptoms can also appear organic neurological disease manifestations, such as peripheral neuritis, headache, hemianopsia, sensory and activity disorders. Complications 1, Raynaud’s phenomenon long-term frequent occurrence of authors can appear local soft tissue atrophy and necrosis and other dystrophic manifestations, severe cases of bone resorption of the extremities about 5% of the patients can appear high blood pressure, but also can appear in important organs vasculitis such as cardiac vasculitis, renal artery stenosis arterial and venous embolism and so on. 2, pleural effusion, pericardial effusion or both concurrently. 3.Individual cases have obvious bleeding tendency and even cause death. Diagnostic criteria There are no uniform diagnostic criteria for UCTD. Table 3 lists the names of UCTD and its diagnostic conditions for clinical and research reference. According to foreign reports and analysis of 70 patients with UCTD in the People’s Hospital of Peking University, the diagnosis of UCTD should have more than one typical rheumatic symptom or sign, accompanied by more than one high-titer autoantibody positivity, with a duration of the disease of more than two years, and with the exclusion of any other CTDs. The analysis of the limited number of cases in foreign countries and in our hospital suggests that those who conform to the clinical features of UCTD health search but have a shorter course of the disease may develop other CTDs within a The analysis of limited cases in foreign countries and in our hospital suggests that patients who meet the clinical characteristics of UCTD, but have a shorter course of the disease, may develop typical clinical manifestations of other CTDs or laboratory abnormalities within a certain period of time, such as Raynaud’s phenomenon with ANA positivity, and may develop typical manifestations of SLE or scleroderma within a year or two, or even a few years after the diagnosis of UCTD, which is not uncommon. Therefore, the disease should not be readily diagnosed in patients with a disease duration of less than two years. Even in cases with a longer course and a clear diagnosis, close follow-up is needed to monitor the possibility of developing other CTDs. For patients with diffuse CTD who have been treated with immunosuppressive drugs and glucocorticoids at an early stage, the diagnosis of UCTD may be fulfilled because the disease is partially controlled and does not show the clinical manifestations and laboratory abnormalities of other CTDs. This “setback” after treatment is itself a reflection of the effectiveness of treatment, but it is also easy to neglect the patient’s further examination and formal treatment health search. Therefore, clinically such patients should still carry out more systematic laboratory tests, such as muscle biopsy for those who suspect dermatomyositis, anti-nuclear antibody and anti-ds-DNA antibody test for those who suspect SLE, in order to make a correct diagnosis and give regular treatment in time. Clinically, attention should be paid to differentiate undifferentiated connective tissue disease from overlap syndrome and mixed connective tissue disease (MCTD). Overlap syndrome refers to the simultaneous or sequential occurrence of the clinical manifestations of two connective tissue diseases, and meets the respective diagnostic criteria. Mixed connective tissue disease has internationally recognized diagnostic criteria and may have clinical signs similar to, but not meeting the diagnostic criteria for, systemic lupus erythematosus, polymyositis, or progressive systemic sclerosis, and is characterized by Raynaud’s phenomenon, swollen hands with lung involvement, and high titers of nRNP antibodies. Laboratory Tests Patients with UCTD may present with a wide range of abnormal laboratory tests. However, for each individual, most patients with UCTD have only one or two laboratory abnormalities autoantibody profile is more homogeneous. Blood tests may show leukopenia, thrombocytopenia, or anemia. Those with hemolytic anemia may have a positive Coombs test Prolonged partial thromboplastin time in patients with autoimmune thrombocytopenia. Urine routine may show proteinuria and hematuria. Accelerated hemocyte sedimentation rate and elevated gamma-globulin can be seen in some patients with elevated transaminases, often suggesting autoimmune liver damage. ANA positivity is the most common serologic test, with a positive rate of 55% to 100%, and an average of about 58%. Fluorescent karyotypes are most common in the speckled type, homogeneous and perinuclear types are less common, and the titer is similar to that of SLE. A small percentage of patients may be positive for anti-RNP antibodies, anti-SSA or SSB antibodies to rheumatoid factors. The presence of anti-RNP antibodies is often associated with Raynaud’s phenomenon and arthritis, while anti-SSA antibody positivity is often accompanied by dry mouth Health Search anti-dsDNA antibody positivity, anti-Sm antibody positivity syphilis serologic test false positivity and complement reduction are rare. Other ancillary tests: In other ancillary tests, liver and spleen lymph node enlargement can be seen on ultrasound, and pericardial or pleural effusion may also be found on ultrasound and X-ray. Abnormal pulmonary function is rare. Treatment Patients with UCTD often have mild clinical manifestations and are generally treated symptomatically. The purpose of treatment is to reduce the clinical symptoms of the patient, so that the condition of long-term remission and prevention of adverse regression of the treatment plan and drug dosage should pay attention to the principle of individualization, and pay attention to the observation of adverse drug reactions. 1, symptomatic treatment of fatigue, fever, arthralgia or arthritis can choose non-steroidal anti-inflammatory drugs. Non-steroidal anti-inflammatory drug efficacy of individual differences in general, the more severe symptoms preferred diclofenac and other anti-inflammatory effect of the better; less severe symptoms or long-term use of the drug can be chosen to have a small adverse reaction, easy to take slow-release non-steroidal anti-inflammatory drugs, such as meloxicam and nabumetazone, etc.; upper gastrointestinal tract inflammation, ulcers and other history of the appropriate choice of rofecoxib and celecoxib and other selective COX-2 inhibitors. Patients with Raynaud’s phenomenon need to pay attention to warmth, and depending on the degree of the disease, give vasodilating drugs such as calcium channel antagonists and other treatments. Patients with severe symptoms or ulcers can be given intravenous prostaglandins and regidan and other drugs to improve circulation quietly, clinical observation in most patients have better results. Patients with photosensitivity should pay attention to avoid direct sunlight. 2, adrenocorticotropic hormone facial rash can be local application of hormone ointment. Difficult to relieve the arthritis can also be given to the joint cavity local injection betamethasone (Depo-Prozon) acetate deinflammatory treatment. Organ involvement such as pericarditis, thrombocytopenia or hemolytic anemia can be applied systemic hormone therapy, but should not be used in large doses of hormone. In addition to special circumstances, general prednisone 0,5mg/(kg?d) can make the condition improve, at this time should be reduced as soon as possible to 10mg/d below the maintenance of small doses, in order to reduce the incidence of hormonal adverse reactions. European anti-rheumatic alliance survey found that 38% of patients in the initial diagnosis of oral prednisone therapy, but the amount of hormone are ≤ 10mg / d, 1 year and 2 years of follow-up the proportion of 43% and 27%, respectively. In 1989, Jonathan et al. summarized the clinical data of 38 patients and found that 47% (18/38) needed only non-steroidal anti-inflammatory drugs, 11% (4/38) needed topical hormone therapy, 32% (12/38) needed oral hormone therapy, 29% (11/38) needed hydroxychloroquine or chloroquine therapy, and only 1 patient needed immunosuppressant therapy, which accounted for 3%. Only one case (3%) required immunosuppressive therapy. Except for one case of autoimmune thrombocytopenia, the dose of oral hormone was mostly 20mg/d or less, and could be quickly reduced. 3, immunosuppressant for conventional treatment is ineffective patients can also try immunosuppressant, for its clinical experience in the treatment of the report less health search. Generally according to the different clinical symptoms with reference to other connective tissue disease treatment to give different programs. However, it is advisable to adopt a small dose and short course program. Commonly used immunosuppressants include methotrexate and azathioprine. In 1995, Wise et al. reported that methotrexate could be used in patients with polyarthritis and difficulty in hormone reduction, and the dosage was similar to that used in systemic lupus erythematosus. Fifty-three percent of the patients treated were effective, with polyarthritis and mucocutaneous lesions showing the most marked improvement. 60% of the patients experienced adverse effects, of which 33% had to discontinue treatment. One patient in the 25 mg/week dosage group developed an opportunistic pathogenic infection, cryptococcal encephalitis, in which the patient was concurrently taking prednisone 30 mg/d Health Search. Overall, the safety profile of methotrexate therapy is fair with attention to the use of lower doses and the ability to follow up closely during the course of treatment. In addition, in patients with refractory UCTD, the administration of appropriate amounts of leflunomide cyclosporine A and other immunosuppressive drugs may be effective but more clinical studies are needed to confirm. 4.Anti-malarial drugs can be tried in patients with fever, facial rash, arthritis, and can be combined with non-steroidal anti-inflammatory drugs. The common dose of hydroxychloroquine is 200-400mg/d. At this dose, damage to the fundus of the eye rarely occurs. However, as a precautionary measure, ophthalmologic examinations should be performed before and every 3 to 6 months after administration to note changes in the visual field and the occurrence of lesions such as the fundus. A survey conducted by the European League Against Rheumatism (ELAR) in 2000 found that about 17% of 112 UCTD patients were treated with antimalarials, but the percentage increased to about 32% after 2 years. This result suggests that patients are compliant with antimalarial therapy Health Search and that the low incidence of discontinuation due to adverse effects allows long-term use. Prognosis and prevention Studies have shown that the incidence of visceral involvement such as interstitial fibrosis of the lungs, renal damage and damage to the central nervous system in this disease is low, and the prognosis is relatively good Long-term follow-up more than half of the patients can be in complete remission. 1, remove the triggers of the disease, pay attention to hygiene, strengthen physical exercise to improve their own immunity, to prevent infection. 2, early diagnosis and early treatment, do not easily give up treatment when the disease is in remission. Systemic lupus erythematosus is an autoimmune disease with multiple organ damage, in which lupus encephalopathy (also known as central nervous system lupus) is a critical condition that requires timely and correct treatment. The clinical manifestations of lupus encephalopathy are various and the treatment is different. (1) Theological symptoms: If hormones can be ruled out as the cause, tranquilizers and moderate doses of hormones can be given. (2) Seizures: give anticonvulsant drugs and high-dose hormone (about 1mg/(kg, d)) treatment. (3) Progressive dementia: medium-dose hormone therapy and regular testing of intelligence level. (4) Diffuse lupus encephalitis: its pathological manifestation is vasculitis caused by immune complex deposition, and the condition is critical, methylprednisolone 1~2mg/(kg, d) can be given, and if necessary, methylprednisolone 500~1000mg/d shock can be given for 2~3 consecutive days. Combined with cyclophosphamide 800~1000mg, shock once a month.