03:53,14,Oct,2011 | (291/0/0) | Original

emedicine mixed connective tissue

Kasukawa diagnostic criteria (Japan):
1. Common symptoms (1) Raynaud's phenomenon (2) swelling of fingers or hands
2. Anti-snRNP antibodies
3. ⑴ SLE-like symptoms show mixed polyarthritis ① ② ③ facial erythema lymphadenopathy pericarditis or pleurisy ④ ⑤ white or thrombocytopenia ⑵ SSc finger-like hardening of the performance ① ② pulmonary fibrosis, restrictive ventilatory disorders or diffuse esophageal peristaltic function to reduce or minimize ③ expansion ⑶ PM esophageal muscle weakness, like the performance ① ② The serum levels of muscle enzymes (CPK) ③ EMG showed myogenic damage
Diagnostic criteria: at least two common symptoms of a positive, anti-snRNP antibodies and the mixed performance of the three, any two of each with more than one symptom.
kahn diagnostic criteria (France): 1. serological criteria: the presence of high titer antibodies against u1RNP, the corresponding dot-type ANA titer
emedicine mixed connective tissue

The names of diseases (English) mixed connective tissue disease Pinyin HUNHEXINGJIEDIZUZHIBING code alias Western connective tissue disease classification code of TCM Classification of Diseases definition of Western disease name mixed connective tissue disease (mixed connective tissue, called MCTD) is a special connective tissue disease syndrome, It is characterized by systemic lupus erythematosus (SLE), progressive system sclerosis (PSS), polymyositis or dermatomyositis (DM) on the combined clinical phenomenon, and with abnormally high titer RNP antibodies. Release Name Western medicine-based disease causes immune dysfunction main cause. A recent study of endothelial cells in serum factors that may cause proliferation of cytotoxic vasculopathy. Cause of internal medicine is the yin and yang qi imbalance. Seasonal population with age from 5 years to 80 years, mean 37 years, 80% of patients were female. The intensity and incidence of disease transmission seems more common than myositis, and more SLE rare. Pathogenesis of immune disorders mainly based disease pathogenesis: ① the following characteristics for several years based on ultra high titer RNP antibodies, indicating that persistent antigen stimulation or defects in the regulatory mechanisms of normal cells. ② significantly higher agammaglobulinemia. ③ Some patients have mild to moderate low complement the blood. ④ The disease activity of blood immune complexes. ⑤ joints in the epidermis and dermis and along the glomerular basement membrane, blood vessel wall muscle fibers in complement and IgG, IgM specific deposition. ⑥ skin speckled IgG formed the nucleus of focal fluorescence. ⑦ RNP antibodies were clean through the FC receptor into the body of mononuclear cells. ⑧ various tissues such as muscle, lung,
salivary gland, liver, heart and other lymphocytes, plasma cells, chronic inflammatory infiltration. System or parts of many diseases no clinical signs or symptoms, suggesting that the disease is slowly evolving, and that RNP antibodies and disease are closely related. Such as swelling of the hands, abnormal esophageal movement, pulmonary diffusion impairment and inflammatory myositis, suggesting that in some tissues of the body of focal lesions play an important role. RNP antibodies are considered a protective effect, may inhibit the DNA and between DNA antibody immune response, ie anti-RNP antibodies interfere with DNA of a DNA complex formation. TCM blood runs sluggish, blood stasis, blocking the meridians. Toxic heat evil (for viruses, bacteria, drugs or wind cold dampness evil heat of) invasion and more dirty.
Yin and yang, qi and blood disorders based, toxic heat, cold, wind evil as the standard. The disease pathology is the pathology of proliferative endometrium or middle vascular disease which caused the vessel lumen stenosis, including aortic, coronary, pulmonary and renal arteries. Pulmonary disease caused by pulmonary hypertension and pulmonary function abnormalities. Renal vascular disease causing renal failure is associated with (or without) high blood pressure.
Pathological examination revealed myocardial necrosis, perivascular inflammatory interstitial infiltration, interstitial fibrosis and intimal thickening and small blood vessels narrow. Traditional Chinese Medicine diagnostic criteria for pathological diagnosis of the physiological 1. Thermal injury of blood contact, blood heat to leak out, stagnate in the skin, then see the skin erythema.
2. Toxicity stagnate, blocked meridians are joint swelling and pain.
3. Toxin attack committed in hot dirty, the internal organs are affected.
4. Toxin hearts and minds will see the heart palpitations, irritability, or even trance.
5. Toxic hot liver injury, Cheuk Yin consumption of blood, liver and spleen disharmony, then see the poor appetite, Eat, chest tightness, abdominal distension, fatigue.
6. Heat consumption of yin, yin true loss, then see the fever, zygomatic red, five upset hot, sweating, eyes and ears of hearing loss. In severe cases, spleen and kidney yang, kidney decline, moisture spread, then see the whole body edema, oliguria.
7. Wind cold when the heating is not evil, you can enter into the meridian into the skin, dirty, injured veins is contracture, joint negative; injured spleen is satisfied that the delay is not of; harm to the kidneys stop the moisture within. Yun in the skin.
8. Xure endogenous inflammation of yin fire is high fever subside.
9. Alpine invasion and muscle, skin, joints, causing pain there, numbness, sorrow and grief, weight forward, rigid deformities.
Euphemistically not pass the blood due to run smooth resistance. But evil in the cold, wet and wind of the performance of different side wins. There is line of Bi Sheng wind, cold win for the Tongbi, wet win as a weakness, there is as hot as hot weakness, skin hard and thick, compared with coagulation weakness, skin paralysis; sub-alpine invasion of the blood (similar to vasculitis, arterial inflammation) is the blood of paralysis. Western medicine diagnosis is based on Western diagnostic criteria for control of the disease the following characteristics: â‘  Raynaud's phenomenon; â‘¡ sausage-like fingers, or fingers phenomenon of focal sclerosis; â‘¢ nephropathy light; â‘£ RNP antibodies strongly positive, with multiple joint pain, arthritis , cheek rash, pleurisy, myocarditis, endocarditis, serositis, and myositis, with other tests such as DNA antibody negative, LE cells and other rare and the normal complement of the disease can be diagnosed. Disease history of the disease is inherited. Clinical symptoms and signs are: 81% of Raynaud's phenomenon, multiple joint pain or arthritis 90% 74% 73% hand swelling, similar to the state of said sausage sausage fingers, esophageal motility disorders 58%, 50% of inflammatory myopathy. See also pulmonary fibrosis. Other immune abnormalities have ANA95% positive, high C hypergammaglobulinemia, positive rheumatoid lectin, hardened sign toe phenomenon, lymphadenopathy, rash, generalized scleroderma manifestations, fever, serositis, hepatosplenomegaly. Nervous system abnormalities and a rare kidney disease.
1. Early clinical manifestations
Beginning can be expressed as SLE, PSS myositis typical phenomenon, the three can occur simultaneously. Vigilance against the disease, such as doctors and RNP antibody test can be found more easily available when the early cases. Early symptoms are mild, such as Raynaud's phenomenon, muscle pain, joint pain, fatigue, inspection of high hypergammaglobulinemia, positive antinuclear antibody suspicious after several months or years after the diagnosis of progression to typical performance. Raynaud's phenomenon seen in 85% of cases of this disease can appear in other performance a few months or years before the show, hands and fingers, skin edema and thickening of the PSS phenomenon also for the early complaint. Muscle weakness (with or without pain or tenderness) can be diagnosed as myositis. Polyarthritis as the main performance can be diagnosed as early stage RA. Pleurisy, pericarditis can be the early signs, such as merger fever, joint pain, erythema, rash, can be diagnosed as SLE. Some diagnosed as having viral pericarditis, until the beginning of another phenomenon MCTD diagnosis based disease. Children can be diagnosed as early juvenile RA. Kidney, heart involvement and thrombocytopenia early in the disease is more common than in adults. Early respiratory difficulties may also be the performance of lung involvement and lymphadenopathy were diagnosed as lymphoma.
2. System performance
(1) Skin manifestations: The most common is swelling of hands, especially fingers, sausage-like cone-shaped head and sharp, taut skin, thickening and accompanied by significant edema, which is due to the increase of skin collagen. Telangiectasia may be combined levy. Including acute lupus rash rash cheeks; diffuse, non-scab resistance, erythema STD loss; and (or) chronic discoid skin lesions. Others include dermatomyositis knuckle similar atrophic erythema around the eyelids purple rash, disseminated, non-scarring alopecia and pigment abnormalities, facial and hand scaling telangiectasia and telangiectasia around the nail.
(2) the performance of esophageal movement disorders: 80% of the patients had esophageal dysfunction, including 70% of the patients asymptomatic. Different upper and lower esophageal sphincter pressure usually presents lower and the distal esophagus 2 / 3 degree of creep reduced the severity of esophageal dysfunction and often directly proportional to the duration of the disease.
(3) pulmonary manifestations: Common lung involvement, about 2 / 3 dysfunction in patients with pulmonary diffusion, 1 / 2 patients with restrictive ventilatory dysfunction. Clinical manifestations of lung function impairment is not necessarily so difficult to find unless the earlier series of pulmonary function tests done. 70% of asymptomatic patients had abnormal pulmonary function and (or) X-ray abnormalities. There are also reduced lung capacity. A small number of breathing difficulties and (or) pulmonary hypertension.
(4) cardiac manifestations: The disease rate in children than adults were more common in cardiac involvement, including pericarditis, myocarditis, heart failure and aortic insufficiency. Pericarditis may be the main phenomenon based disease can be misdiagnosed as viral pericarditis.
(5) kidney performance: the performance of the last report of this rare kidney disease, kidney damage, but later discovered to be gradually increased. Kidney damage manifested as proteinuria and hematuria, and renal failure due to the death. Hematuria and proteinuria.
(6) joint and muscle: how joint pain almost all patients, 3 / 4 of patients have significant arthritis and Raynaud's phenomenon diagnosed with RA and myopathy, joint general no deformity, a small number of the relevant section of erosion erosion, deformity. But the more restrictions in the hand, wrist or foot, may be associated with subcutaneous nodules. Symptoms of proximal muscle weakness with or without muscle fatigue, tenderness,
(7) the nervous system: 10% of the patients had severe neurological abnormalities, trigeminal neuropathy is a common, followed by physical mental syndrome, such as paranoid psychosis, progressive stupor, orientation discrimination power loss, vascular headache, no bacterial meningitis, grand mal epilepsy, the majority of peripheral neuropathy, cerebral hemorrhage or infarction, cerebellar ataxia and so on.
(8) blood system: a moderate degree of anemia, leukopenia, Coombe positive hemolytic anemia and thrombocytopenia were rare, severe thrombocytopenia are required splenectomy may be due to intracranial hemorrhage died.
(9) other kinds of manifestations: fever, lymphadenopathy, hepatosplenomegaly, but severe liver dysfunction is rare. Bowel dysfunction caused by cramps, bloating, alternating constipation and diarrhea and malabsorption. May also have a wide range of gastrointestinal diseases. In addition, the disease can be combined Sjogren's syndrome, thyroiditis, and diseases such as persistent hoarseness. Medical diagnostic EMG power icon inflammatory myopathy. Diagnostic Imaging chest showed diffuse interstitial infiltrates, pulmonary or pleural disease, the loss of more than gas. Laboratory diagnosis of muscle creatine kinase, aldolase, aspartate aminotransferase, lactate dehydrogenase increased. Cerebrospinal fluid stool blood in urine 1 other diagnostic immunology. In addition to the performance of general inspection of Hematology visible outside CPK (creatine phosphokinase), aldolase (aldolase), AST high, high hypergammaglobulinemia, antinuclear antibody titer. High dilution can produce nuclear spots, low spots or ring-shaped dilution produce nuclear type. Rheumatoid lectin can be positive, the blood, mild to moderate reduction of complement. ds DNA antibody positive LE cells are rare.
2. Serological abnormalities characteristic of the disease with high titers of serum antibodies to thymus nuclear antigen, can be spotty fluorescent antinuclear antibody response. Extractable nuclear antigen in blood cells agglutination: ENA (to mention nuclear antigen); in MCTD patients have high titer ENA antibodies. ENA antibodies in half of patients with SLE may also be positive, but mostly low titer, and other connective tissue disease in its incidence is very low. ENA has two different antigen, a nuclear ribonucleoprotein (RNP), the equivalent of MO antigen, and the other is equivalent to Sm antigen. ANA dot type can be generated, and in the remission of the disease activity and the persistence of a small number of patients with acute low titer or disappear.
Serological characteristics typical of this disease are: ① High-titer point type FANA; ② ENA high titer antibody response; ③ Sm antibody negative hemagglutination reaction; ④ natural DNA antibodies and LE cells rare; ⑤ complement normal or high. Histological examination of lung biopsy in interstitial fibrosis, interstitial infiltration of mononuclear cells, vascular intimal hyperplasia and middle hypertrophy, severe vascular occlusion, chronic fibrosing pleurisy.
Tube of urine in patients with renal biopsy was diffuse membranous glomerulonephritis, focal glomerulonephritis and mesangial proliferation, renal vascular damage with the ball in the cell proliferation and focal basement membrane thickening and small blood vessels (arterial) was significantly intimal hyperplasia and occlusion. Glomerular basement membrane with IgG, C3C4 granular deposition. Mesangial area in the deep, and there are electron-dense deposits on the skin.
Muscle biopsy between the qualitative change of fibrosis and around the vessels and infiltration of organized lymphoid and plasma cells. Biopsy showed type I fiber, fascia around the atrophy. Or around the muscle membrane, muscle bundle within the membrane of connective tissue, muscle fibers, blood vessels are the deposition of IgG or IgM. Western medicine diagnosis of early diagnosis of this disease is difficult and other similar diseases such as SLE, systemic sclerosis, polymyositis, rheumatoid arthritis, Sjogren's syndrome, vasculitis, viral pericarditis, idiopathic thrombocytopenic purpura , lymphoma, chronic active hepatitis, vascular insufficiency syndrome and fever of unknown origin and other identification, to be followed up.
1, the identification of the disease and SLE may be funding the following reference points: ① the high incidence of Raynaud's phenomenon; ② swelling of the hands; ③ myositis; ④ esophageal movement disorders; ⑤ lung disease; ⑥ severe renal and central nervous Department of rare diseases; ⑦ ds DNA or SM antibody, LE cell, low complement a low incidence of hyperlipidemia.
2, the identification of disease and scleroderma: â‘  the high incidence of multiple arthritis, myositis, lymphadenopathy, leukopenia, high hypergammaglobulinemia; â‘¡ generalized sclerosis of this rare disease.
3, identification of the disease and polymyositis: Raynaud's phenomenon in this disease, arthritis, swelling of the hands, esophageal motility disorders, lung disease, lymphadenopathy, neutropenia was significantly higher than that of polymyositis. RNP antibody persistence of the disease is rarely found in polymyositis. PM-1 (nucleic acid of a protein antigen) antibodies found in half of patients with polymyositis, the disease is extremely rare.
4, identification of the disease and overlap syndrome: â‘  overlap syndrome (OLS) with the overlapping connective tissue disease diagnostic criteria for each part; â‘¡ OLS with the other nucleic acid protein antigens (other than RNP); â‘¢ poor prognosis general survival rate less than 5 years. Myositis overlap syndrome of scleroderma in recent non-RNP. The disease characteristics of a high titer of RNP, the RNP can be used as indicators of the disease diagnosed, especially when the clinical manifestations of early incomplete, in addition to high titer ANA dot type can also be prompted with clinical MCTD, and testing for the RNP such as high-titer, conduct a thorough examination can often abnormal. Identification card in Traditional Chinese Medicine evaluation standard prognosis of this disease better prognosis than SLE, but also easy to improper treatment of death. The average survival period of the disease 1 year, between 1 to 25 years. There are no hormones or trace hormones that alleviate the stability of the disease by several years. Causes of death include renal failure, pulmonary disease, myocardial infarction, colon perforation, brain hemorrhage, suicide. Western medicine treatment of complications of the disease systemic lupus erythematosus treated with identical, the majority of the corticosteroid treatment is effective, especially in the early non-steroidal anti-inflammatory mild can a small amount of drugs or corticosteroids to control the disease. When a serious system damage will need to use relatively large number of prednisone. After the application of corticosteroids can significantly improve symptoms and signs. Then you can gradually reduce the amount of hormones, as well as back to normal, hormone relapse after withdrawal of large doses can be reused.
System damage in disease progression is up to the big dose of hormones, cytotoxic agents can be added to make use of clinical improvement. However, the result is not ideal and can cause serious toxicity and sometimes fatal complications. Less effective treatment of scleroderma-like phenomenon. Concurrent neuropsychiatric disorders including those of aseptic meningitis-like illness and recurrent seizures responded well to steroid therapy, trigeminal neuropathy and peripheral neuropathy, hormone therapy is not valid.
Other Treatment: The disease can be added with other immunosuppressant drugs, such as intravenous cyclophosphamide 200mg, 2 times per week, Nandrolone phenylpropionate 25mg intramuscular injection, 2 times per week or tumor Ning 2mg 3 times a day low molecular weight dextran 500ml / day intravenously (Note allergic reaction) the symptomatic treatment including the application of vasodilators, joint pain or arthritis can be applied to non-steroidal anti-inflammatory agents such as indomethacin, aspirin, increase the hormone of high fever dose. With Raynaud's phenomenon outside attention than vasodilator insulation, thermal insulation, such as handbags when they go out with.
A Chinese medicine treatment to Qi Yin, adjusting blood, promoting blood circulation to treat them the meridians, dispelling wind and dampness detoxification, allergy treat the symptoms.
1. Tripterygium preparations have blood type, heat, swelling effect on the edema and inflammation of the skin lesions, arthritis, joint pain had a better effect. However, the medicinal application of improper or longer or the patient has liver and kidney damage, can lead to disease in patients with drug-induced death, which should be vigilant. China in recent years reported increasing cases of drug poisoning, so the application should be very cautious.
2. Salvia have blood circulation, nourishing, Tongluo effect on limb circulation, skin and soft, to improve the function of menstruation, which are good.
3. Blood circulation in the TPG compound, Chuanxiong other skin sclerosis, joint damage has a therapeutic effect. Such as the inhibition of fibrin formation Chuanxiong, Dan anti-inflammatory, hemostatic, anti-thrombosis Motherwort, peach kernel of the anti-allergic, TPG regulation nerves. The role of these drugs are likely to play a different effect on the MCTD. MCTD is only one manifestation of blood stasis, so a single blood circulation can not be cured MCTD, should be combined with the etiology, pathogenesis, pathophysiology for Integrative Medicine, will it be possible to cure.
Second, the combined symptoms of traditional Chinese medicine treatment of dry and co
Department of Physical deficiency disease, dry Sheng endless, turns into poison, fry the dry burning more beneficial body fluid, a slow buildup. Disease by the guilt of the "virtual, weakness, blood stasis." Renal pedicle for two dirty, Yin and moistening treatment to the basic rule is, and the like can be used by liquid soup. Yin and dryness can be applied with other therapies. If Runzao nourishing blood, blood, soothing drink with other students; Runzao Qingying detoxification with three Purple Tang (Radix Salvia purple black bamboo root), Rehmannia Decoction rhino horn; Runzao raising project with Qijudihuang class. But detoxification Qingzao should always runs through, try soil Fuling, raw licorice, green beans, magnets, Lithospermum and so on.
Other Sheng Jin Yin method can also be combined or used, in particular the spleen and fluid, sour and sweet Yin, Xing Wei Sheng Jin, blood fluid and other laws. Drugs such as ebony, habitat, Pueraria, papaya, raw licorice, Clematis, hawthorn, Scrophulariaceae, etc., heat fluid of drugs such as gypsum, skullcap and the like.
Chinese Acupuncture and Integrative Medicine Massage syndrome combined TCM treatment of renal injury
Available Qi, Yin, Bao-chun, heat, detoxification, blood circulation to the Rule of the Law Governing specimens wen. An election Simiao side soup Biejiajian Pill, or the usual fried, Dabuyin Pill.
Although the hormone can only be less stressed, or combined with a small amount of immunosuppressive agents, may be appropriate Sjogren's syndrome complicated with hormone doses, or similar treatment of SLE-like, with methyl-prednisolone 40 ~ 80mg short term 3 to 5 days Shock therapy can improve renal function and systemic symptoms of dry.
Preventive care and rehabilitation of historical research
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ask100682402e send a message add as a friend I want to reward 0 Favorites answer complaints (4) questions :2010-5-3 21:01:23
Mixed connective tissue disease
Age: 30
Patients Gender: Female
All the symptoms: Hand, foot joint pain, facial swelling
, The phenomenon of thunder Ruo
Onset time and reason: November 2009 incidence
Treatment: The present strength of the three to eat, severe joint pain.
Want to get help: What should I pay attention in life? Now eat does not affect the fertility of this medicine?
Added an additional reward additional questions to create an anonymous medical records without a satisfactory answer to the question asked to delete the relevant tab: Let me answer: questions for users, detailed analysis of the disease, made reference to observations, answer a question, help a netizen! Enter
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Zhang Jinfeng
Credit: 530
Response to their questions more doctors, 2010-5-3 21:09:30 complaint
Condition analysis:
Hello, mixed connective tissue disease is a kind of SLE, scleroderma, dermatomyositis and polymyositis, or RA-like symptoms rheumatic syndrome characterized by overlapping, with high titers of circulating anti-nuclear antibodies, that is, Anti-nuclear ribonucleoprotein antibodies positive rate. Less renal involvement, a good response on the corticosteroids, the prognosis is good.
Therapeutic measures and drug therapy generally similar to systemic lupus erythematosus. The majority of patients with corticosteroid treatment effect, especially early in the course of the disease. Mild patients used salicylates, other nonsteroidal anti-inflammatory drugs, antimalarials, or small doses corticosteroids that can be controlled. major organs severely affected often require large doses of corticosteroids, such as the starting dose of prednisone was 1mg/kg.
I wish you health!
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Money into
Credit: 81
Response to their questions more doctors, 2010-5-3 21:11:24 complaint
Condition analysis:
Eating prednisone only temporary emergency rule, and can not rule this disease, and the great side effects.
You can try another medicine, Integrative Medicine now there have been numerous cases of the disease can reduce the side effects of hormones, the effect is not bad.
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The art of disposable waste
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Details of questions back to their doctors, 2010-5-3 21:35:14 complaint
Anger, from what you said, your face is not swollen, moon face is caused by hormones. You are 30 years old to live by hormones? Prednisone can really cure your disease? The more the disease will rule you more. Halo, or find an experienced Chinese medicine, with medicine conditioning about it. People should live a quality life to. Do you hang out with the hormone.
【You have a new perspective on the answer you in a timely manner to support the comments】 1 No answer to the reply comment!
The art of disposable waste
Credit: 18
Details of questions back
to their doctors, 2010-5-3 21:35:33 complaint
Anger, from what you said, your face is not swollen, moon face is caused by hormones. You are 30 years old to live by hormones? Prednisone can really cure your disease? The more the disease will rule you more. Halo, or find an experienced Chinese medicine, with medicine conditioning about it. People should live a quality life to. Do you hang out with the hormone.
【You have a new perspective on the answer you in a timely manner to support the comments】 1 No answer to the reply comment! View more related questions . I want to ask the doctor the right treatment on the disease concept is - 3 times to answer my mother often lower back pain, but also by the legs - 1 times where the best answer to governance can cure epilepsy - 1 time to answer dumb will do genetic - 2 times to answer how I did not smell of sweat stream? - 2 answers slowly eating gonorrhea people should pay attention to even the ordinary life - 2 times to answer
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