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20-September-2008 10:21:22 - tissue disease Connective tissue disease Classification and external resources MeSH D003240 According to Dr. William C. Shiel, a connective tissue disease is any disease that has the connective tissues of the body as a target of pathology. Connective tissue is any type of biological tissue with an extensive extracellular matrix that supports, binds together, and protects organs. These tissues form a framework, or matrix, for the body, and are composed of two major structural protein molecules, collagen and elastin. There are many different types of collagen protein in each of the body's tissues. Elastin has the capability of stretching and returning to its original length - like a spring or rubber band. Elastin is the major component of ligaments tissues that attach bone to bone and skin. In patients with connective tissue disease, it is common for collagen and elastin to become injured by inflammation. Many connective tissue diseases feature abnormal immune system activity with inflammation in tissues as a result of an immune system that is directed against one's own body tissues autoimmunity.1 Diseases in which inflammation or weakness of collagen tends to occur are also referred to as collagen diseases. Collagen vascular disease is a somewhat antiquated term used to describe diseases that can be but are not necessarily associated with collagen and blood vessel abnormalities and that are autoimmune in nature.2 Connective tissue diseases can have strong or weak inheritance risks, and can also be caused by environmental factors. Contents 1 Heritable Connective Tissue Disorders 2 Autoimmune Connective Tissue Disorders 3 Other connective tissue disorders 4 External links Heritable Connective Tissue Disorders Marfan syndrome - a genetic disease causing abnormal fibrillin. Ehlers-Danlos syndrome - causes progressive deterioration of collagens, with different EDS types affecting different sites in the body, such as joints, heart valves, organ walls, arterial walls Osteogenesis imperfecta brittle bone disease - caused by insufficient production of good quality collagen to produce healthy, strong bones. Stickler syndrome - affects collagen, and may result in a distinctive facial appearance, eye abnormalities, hearing loss, and joint problems. Autoimmune Connective Tissue Disorders Main article: Autoimmunity These are also referred to as systemic autoimmune diseases. The autoimmune CTDs may have both genetic and environmental causes. Genetic factors may create a predisposition towards developing these autoimmune diseases. They are characterized as a group by the presence of spontaneous overactivity of the immune system that results in the production of extra antibodies into the circulation. The classic collagen vascular diseases have a classic presentation with typical findings that doctors can recognize during an examination. Each also has classic blood test abnormalities and abnormal antibody patterns. However, each of these diseases can evolve slowly or rapidly from very subtle abnormalities before demonstrating the classic features that help in the diagnosis. The classic collagen vascular diseases include: Systemic Lupus Erythematosus SLE - An inflammation of the connective tissues, SLE can afflict every organ system. It is up to nine times more common in women than men and strikes black women three times as often as white women. The condition is aggravated by sunlight. Rheumatoid Arthritis - Rheumatoid arthritis is a systemic disorder in which immune cells attack and inflame the membrane around joints. It also can affect the heart, lungs, and eyes. Of the estimated 2.1 million Americans with rheumatoid arthritis, approximately 1.5 million 71 percent are women. Scleroderma - an activation of immune cells that produces scar tissue in the skin, internal organs, and small blood vessels. It affects women three times more often than men overall, but increases to a rate 15 times greater for women during childbearing years, and appears to be more common among black women. Sjögren's syndrome - also called Sjögren's disease, is a chronic, slowly progressing inability to secrete saliva and tears. It can occur alone or with rheumatoid arthritis, scleroderma, or systemic lupus erythematosus. Nine out of 10 cases occur in women, most often at or around mid-life. Mixed Connective Tissue Disease - Mixed connective-tissue disease MCTD is a disorder in which features of various connective-tissue diseases CTDs such as systemic lupus erythematosus SLE; systemic sclerosis SSc; dermatomyositis DM; polymyositis PM; and, occasionally, Sjögren syndrome can coexist and overlap. The course of the disease is chronic and usually milder than other CTDs. In most cases, MCTD is considered an intermediate stage of a disease that eventually becomes either SLE or Scleroderma. Other connective tissue disorders Scurvy - caused by a dietary deficiency in vitamin C, leading to abnormal collagen. External links Merck Manual: Musculoskeletal and connective tissue disorders Merck Manual: Inherited connective tissue disorders Arthritis Foundation The Myositis Foundation v d e Diseases of the musculoskeletal system and connective tissue M, 710-739 Arthropathies Arthritis Septic arthritis - Reactive arthritis - Rheumatoid arthritis - Psoriatic arthritis - Felty syndrome - Juvenile idiopathic arthritis - Still's disease - crystal Gout, Chondrocalcinosis - Osteoarthritis Heberden's node, Bouchard's nodes - Monoarthritis/Polyarthritis Specific joints shoulder Winged scapula - elbow Cubitus valgus, Cubitus varus - hand Wrist drop, Boutonniere deformity, Swan neck deformity hip Protrusio acetabuli, Coxa valga, Coxa vara - leg Foot drop, Flat feet, Club foot, Unequal leg length - patella Luxating patella, Chondromalacia patellae - foot Bunion/hallux valgus, Hallux varus, Hallux rigidus, Hammer toe general terms Valgus deformity, Varus deformity Other Hemarthrosis - Arthralgia - Osteophyte - Hypermobility Systemic CT disorders vasculitis: Arteritis Polyarteritis nodosa, Takayasu's arteritis, Temporal arteritis - arterioles/capillaries Wegener's granulomatosis, Kawasaki disease, Churg-Strauss syndrome, Microscopic polyangiitis hypersensitivity/autoimmune: Hypersensitivity vasculitis - Goodpasture's syndrome - Systemic lupus erythematosus Drug-induced - Dermatomyositis Juvenile dermatomyositis - Polymyositis - Scleroderma - Sjögren's syndrome - Behçet's disease - Polymyalgia rheumatica - Eosinophilic fasciitis Dorsopathies spinal curvature Kyphosis, Lordosis, Scoliosis - Scheuermann's disease - Spondylolysis - Torticollis - Spondylolisthesis Spondylopathies Ankylosing spondylitis, Spondylosis, Spinal stenosis - Schmorl's nodes - Degenerative disc disease - Coccydynia - Back pain Radiculopathy, Neck pain, Sciatica, Low back pain Soft tissue disorders Muscle Myositis Pyomyositis - Myositis ossificans Fibrodysplasia ossificans progressiva Synovium and tendon Synovitis/Tenosynovitis Calcific tendinitis, Stenosing tenosynovitis, Trigger finger, DeQuervain's syndrome - Irritable hip - Ganglion cyst Bursa Bursitis Olecranon, Prepatellar, Trochanteric - Baker's cyst Fascia/fibroblastic Fasciitis Plantar fasciitis, Nodular fasciitis, Necrotizing fasciitis - Dupuytren's contracture - Fibromatosis Shoulder lesions Adhesive capsulitis - Rotator cuff tear - Subacromial bursitis Enthesis Enthesopathies Iliotibial band syndrome, Achilles tendinitis, Patellar tendinitis, Golfer's elbow, Tennis elbow, Metatarsalgia, Bone spur, Tendinitis Other, NEC Muscle weakness - Rheumatism - Myalgia - Neuralgia - Neuritis - Panniculitis - Fibromyalgia Osteopathies disorders of bone density and structure: Osteoporosis - Osteomalacia - continuity of bone Pseudarthrosis, Stress fracture - Monostotic fibrous dysplasia - Skeletal fluorosis - Aneurysmal bone cyst - Hyperostosis - Osteosclerosis Osteomyelitis - Avascular necrosis - Paget's disease of bone - Algoneurodystrophy - Osteolysis - Infantile cortical hyperostosis Chondropathies Juvenile osteochondrosis Legg-Calvé-Perthes syndrome, Osgood-Schlatter disease, Köhler disease, Sever's disease - Osteochondritis - Tietze's syndrome - Relapsing polychondritis See also congenital Retrieved from http://en..org/wiki/Connective_tissue_disease Categories: Diseases Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Français Italiano Polski This page was last modified on 24 June 2008, at 09:22
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