What Is Connective Tissue Disease?

Connective tissue disease refers to diseases involving connective tissue, including lupus erythematosus, rheumatoid arthritis, scleroderma, dermatomyositis, nodular polyarteritis, Wegener's granulomatosis, giant cell arteritis, and Sjögren's syndrome Wait. In the rheumatism classification revised by the American Rheumatology Association in 1982, connective tissue disease may also include allergic vasculitis, Behcet syndrome, nodular non-purulent febrile panniculitis, and the like. Connective tissue disease has some common characteristics in clinical, pathological and immunological aspects, such as multi-system involvement (ie, skin, joints, muscles, heart, kidney, hematopoietic system, central nervous system, etc. can be affected at the same time), a long course and a complicated condition May be associated with fever, joint pain, vasculitis, increased erythrocyte sedimentation, increased gamma globulin, and so on.

Basic Information

English name

connective tissue disease, CTD

Visiting department

Division of Rheumatology

Common causes

It has a certain relationship with genetics, immune abnormalities, and viral infections.

Common symptoms

Multi-system involvement, recurrent attacks and remissions, each with characteristic performance

Etiology of connective tissue

The etiology of this disease is not very clear, and it is generally believed to be related to heredity, immune abnormalities, and viral infections, and it is a multi-cause disease. With the development of immunology, it is found that most connective tissue diseases are accompanied by immunological abnormalities, such as low suppressor T cell function and hyperhumoral immune function. Most connective tissue diseases have autoantibodies, so this group of diseases is also classified Autoimmune diseases.

Clinical manifestations of connective tissue disease

Connective tissue disease has certain clinical, pathological, and immunological features in common, such as multi-system involvement (ie, skin, joints, muscles, heart, kidney, hematopoietic system, central nervous system, etc. can be affected at the same time), a long course and a complex condition May be accompanied by fever, joint pain, vasculitis, increased erythrocyte sedimentation, increased globulin, etc .; but each has its own characteristic manifestations.

Connective tissue disease examination

Autoantibodies against autoantigens are characteristic of many diseases including systemic lupus erythematosus, scleroderma, Sjogren's syndrome, primary biliary cirrhosis, certain tumors and paraneoplastic syndromes. Some of these autoantibodies are disease-specific markers. Their antigens include nucleosomes, nucleoli, small nuclear ribonucleoproteins (snRNPs), centromere antigens, mitochondria, and heterogeneous ribonucleoproteins (hn-RNP). Intracellular macromolecular complexes or particles. B cells are overactive, T cells are dysfunctional, and HLA susceptibility can induce autoantibodies. Rheumatoid factor is often positive, titers often increase, and erythrocyte sedimentation often increases, and some patients have diffuse hyperglycemia. Some patients have mild to moderate reductions in serum complement. Serum creatine kinase and aldolase are usually elevated in patients with active myositis. Some patients have moderate anemia and decreased white blood cell counts. Clinically significant Coombs test-positive hemolytic anemia and thrombocytopenia are common in systemic lupus erythematosus. The urine of patients with glomerulonephritis can be found in hematuria, cast and protein.

Connective tissue disease diagnosis

Diagnosis of mixed connective tissue disease:

Commonly used Sharp standards in clinical practice:

Main criteria

Severe myositis; Pulmonary involvement (less than 70% of carbon dioxide diffusion, pulmonary hypertension, and proliferative vascular injury on lung biopsy); Reynolds / esophageal motility reduction; joint swelling, tenderness, or hardened fingers; Antibody positive, titer> 1: 320, and / or ENA antibody positive.

2. Minor criteria

hair loss; decreased white blood cell count; anemia; pleurisy; pericarditis; arthritis; trigeminal neuropathy; cheek erythema; thrombocytopenia; mild myositis.

3. Confirmation

4 main criteria; Serological antinuclear antibody is positive, titer> 1: 320, infectious or tumorous diseases need to be excluded.

4. Possible diagnosis

Clinically, there are 3 main standards or 2 main standards and 2 secondary standards; serological antinuclear antibodies are positive, and the titer is> 1: 320.

Connective tissue disease treatment

Generally symptomatic treatment and control of disease development. The treatment plan and drug dosage should pay attention to the principle of individualization, and pay attention to observe the adverse reactions of the drug.