Introduction to collagen-vascular diseases

Definition: Rheumatologic (or Rheumatic) Disease: diseases characterized by pain and inflammation in joints and connective tissues, often referred to as “collagen-vascular diseases”. In collagen vascular diseases, this immune system malfunctions. Rather than responding to foreign antigens, the body produces antibodies (autoantibodies) against its own antigens and normal proteins. Researchers do not understand what gets this autoimmune process started, but they have a fairly good idea of how it proceeds once it begins.

The Normal Joint

What causes connective tissue disease? The specific causes of most connective tissue diseases are not known. However, there are genetic patterns that are considered to increase the risk for developing connective tissue diseases. It is likely that a combination of genetic risks and environmental factors are necessary for the development of connective tissue disease. Fatigue, stress, and higher levels of certain antibodies also may lead to these diseases. Even ultraviolet rays of sunlight have been suggested as possible contributing causes. Collagen vascular diseases are not contagious; people cannot catch these diseases from one another.ultraviolet

Multiple Factors Contribute to the Development of Arthritis

The Normal Synovium Function of Normal Synovium: maintenance of intact non-adherent tissue surface lubrication of cartilage control of synovial fluid volume and composition (plasma and hyaluronan) nutrition of chondrocytes within joints

Choy, E. H.S. et al. N Engl J Med 2001;344: Pathogenesis of Rheumatoid Arthritis Inflammed synovial tissue (synovitis) Villous hyperplasia Intimal cell proliferation Inflammatory cell infiltration T cells, B cells, macrophages and plasma cells Production of cytokines and proteases Increased vascularity Self-amplifying process

Collagen vascular diseases include : Ankylosing spondylitis Dermatomyositis Polyarteritis nodosa Psoriatic arthritis Rheumatoid arthritis Scleroderma Systemic lupus erythematosus Sjögren syndrome

Rheumatoid Arthritis is Systemic Inflammatory Disease

SLE is a Systemic Inflammatory Disease

Symptoms: Symptoms differ depending on the illness, but they often include joint pain, fever, rash, recurrent infections, fatigue, mouth ulcers, dry mouth and dry eyes, hair loss, difficulty swallowing, swollen glands, or fingers and toes that get overly cold when exposed to cooler temperatures. In addition to systemic lupus erythematosus and rheumatoid arthritis, collagen vascular diseases include:

Diagnosis: A complete medical history and a physical examination are the basis for the diagnosis of autoimmune disease. A number of laboratory tests can be used to help diagnose collagen vascular diseases. Blood tests can check levels of autoantibodies. Other tests include rheumatoid factor tests, urinalysis, blood counts, liver and kidney tests, and a sedimentation rate, which will give a nonspecific indicator of inflammation. A chest x-ray and other tests of specific lung function also may be done, since collagen vascular disorders occasionally produce breathing difficulties.

Treatment: At present, there are no cures for autoimmune diseases, although some may go into remission as symptoms disappear for periods of time. Treatment depends on the extent of the disease. Doctors may prescribe steroid creams or anti- inflammatory medications to ease discomfort. In advanced cases, immunosuppressant drugs may help lessen the immune system's over-reaction.anti- inflammatory

Living with Collagen Vascular Diseases: These serious diseases often require adjustments in activities of daily living. People with rheumatoid arthritis often have early morning stiffness that lasts for about an hour, after which they can go on about their day. Avoiding certain foods, and reducing physical and emotional stresses, also seem to reduce symptoms for some people.

Importance and Impact of Rheumatologic Disease Prevalence (per 100,000) MaleFemale Rheumatoid Arthritis440 1,100 Ankylosing Spondylitis Gout SLE 7 32 Scleroderma 1 5 Osteoarthritis 3,470 5,870 All Musculoskeletal conditions 15,510 20,720

CDC: Census Bureau 2004

Progressive Chronic Inflammation Can Lead to Joint Destruction Early Arthritis - soft tissue swelling, especially around the PIP joints Chronic inflammation in the joint leads to bone destruction evident as erosions Prolonged severe chronic arthritis leads to deformity and disability.

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