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Mixed connective tissue diseaseMixed connective tissue disease (MCTD) 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.Etiology, pathogenesis and prevalenceUnknown etiology and prevalence. In some patients will eventually evolve into a typical scleroderma and SLE. A lot of performance to support MCTD as a separate disease: overlapping clinical manifestations suggestive of several connective tissue diseases; high titer anti-RNP antibodies, and other anti-nuclear antibody (ANA) level is not high; with systemic lupus erythematosus compared to most patients with this disease reticuloendothelial system the ability to clear immune complexes normal; the immune T cells in patients during the development of abnormalities, and other rheumatic diseases are different; common pulmonary hypertension, accompanied by mild fibrosis proliferative disease.Pathology, symptoms and signsTypical clinical syndrome is characterized by: Raynaud's phenomenon, multi-joint pain or arthritis, swollen hands, proximal muscle inflammatory disease of the lower esophageal motor function and lung disease. Raynaud's phenomenon in another few years before the onset of clinical manifestations appeared to start early clinical findings often suggest systemic lupus erythematosus, scleroderma, polymyositis / dermatomyositis,


or rheumatoid arthritis. regardless of how the early performance, there is a tendency for the lesions from the more limited development of a wide range of lesions, clinical types over time is also changing.Performance is the most common skin swollen hands, fingers, sausage-like appearance. In common with diffuse scleroderma-like changes in scleroderma with finger or fingertip necrosis in fewer ulcers occurred in the MCTD. Other manifestations include lupus like rash, knuckle skin erythema, eyelid was violet, diffuse non-scarring alopecia, hands and face of the square telangiectasia.Almost all patients have multiple joint pain, 75% had significant arthritis. Joint often is not deformed, but also the erosion of RA similar changes and deformities. Common proximal muscle weakness, with or without tenderness, EMG revealed typical inflammatory myopathy, muscle biopsy showed muscle fiber degeneration and interstitial and perivascular infiltration of lymphocytes and plasma cells.Esophageal sphincter pressure abnormalities, including reduced under the distal 2 / 3 of the peristaltic wave amplitude decrease, 80% of patients reduce the pressure on the sphincter, including 70% of asymptomatic patients were .80% of the lung lesions can occur, before clinical symptoms may appears diffuse function were significantly abnormal. chest X ray may show pleural effusion, or diffuse interstitial infiltrates. part of the patient's lung disease to become as prominent clinical problem, resulting in post-respiratory distress or pulmonary hypertension. pulmonary hypertension and proliferative vascular injuries usually were hidden Progress become serious complications in some patients. kidney disease only occurs in 10% of patients, usually mild, but even can become a major clinical problem, patients can die from progressive renal failure. Renal biopsy usually shows Ball mesangial cell proliferation, focal glomerulonephritis and membranous glomerulonephritis, and membranoproliferative glomerulonephritis and proliferative vascular lesions rarely seen. trigeminal nerve sensory branch neuropathy in this disease than in other rheumatic diseases were more common.LaboratoryAlmost all patients of this disease have high titer (o 1:1000) fluorescent anti-nuclear antibody, was spotted type. Agglutination test with the blood cells could be detected in extractable nuclear antigen (ENA) antibody titer is high (> 1 : 100000), because the ENA is a component of ribonuclease (RNase)-sensitive nuclear sugar nuclear protein, it can be removed by RNase digestion ENA and aggregation of blood cells, immune diffusion method can be sure there is anti-RNP antibodies , and anti-ENA, Sm-resistant component of RNase antibodies generally do not exist. usually anti-RNP antibodies for years, but in the long-term remission patients, the antibody levels can be significantly decreased or negative.The disease is not common anti-DNA antibody and lupus cells. Agglutination did aforetime were positive for rheumatoid titer often increased erythrocyte sedimentation rate often faster, 75% of patients with diffuse hypergammaglobulinemia high-C, up to 2 "5g/dl. Only about 25% serum complement of patients with mild to moderate decrease. myositis are active when the serum creatine kinase and aldolase are usually higher.30% of the disease "40% of the patients had moderate anemia and leukopenia. Clinically significant Coombs test positive hemolytic anemia and thrombocytopenia are not uncommon. There glomerulonephritis can be found when the urine hematuria, pipe type and protein.Diagnosis and treatmentSystemic lupus erythematosus, scleroderma, polymyositis, rheumatoid arthritis, juvenile rheumatoid arthritis, Sj ogren syndrome, vasculitis, idiopathic thrombocytopenic purpura, lymphoma, or viral pericarditis inflammation of the patients, there should be considered an additional overlapping symptoms of the disease.Causes of death include pulmonary hypertension associated with proliferative vascular disease, renal failure, myocardial infarction, colon perforation, infection sp, and cerebral hemorrhage. Some patients in a small (or no) maintenance therapy corticosteroids case, also sustained remission years.General treatment measures and drug therapy similar to systemic lupus erythematosus. Most of the patients treated with corticosteroids good 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.



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