Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
A connective tissue disease is any disease that has the connective tissues of the body as a primary target of pathology. The connective tissues are the structural portions of our body that essentially hold the cells of the body together. These tissues form a framework, or matrix, for the body. The connective tissues are composed of two major structural protein molecules, collagen and elastin. There are many different types of collagen protein that vary in amount 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 diseases, 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).
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 of the connective tissues that typically
include diseases which can be (but are not necessarily)
associated with blood-vessel abnormalities.
Connective tissue diseases can have strong or weak inheritance risks
Connective tissue diseases that are strictly
due to genetic inheritance include Marfan
syndrome (can have tissue abnormalities in the heart,
aorta, lungs, eyes, and skeleton) and Ehlers-Danlos
syndrome (many types may have loose, fragile skin or loose
[hyperextensible] joints depending on type).
Other diseases of connective tissue cannot be regularly defined by gene abnormalities.
These connective tissue diseases occur for unknown reasons but may have weaker genetic factors that predispose to their development.
They are characterized as a group by the presence of
spontaneous overactivity of the immune system which results
in the production of extra antibodies into the circulation.
The connective tissue diseases include systemic lupus
erythematosus, rheumatoid arthritis, scleroderma,
polymyositis, and dermatomyositis. These are considered
classic collagen vascular diseases. Each of these diseases
has a "classic" presentation with typical findings that
doctors can recognize during an examination. Each also has various
typical blood test abnormalities and a variety of abnormal antibodies
that are commonly found in blood testing. However, each of these diseases can evolve slowly
or rapidly from very subtle abnormalities before
demonstrating the classic features which help in the
diagnosis.
Sometimes, in the early stages, doctors simply
refer to the "undifferentiated" condition as a collagen
vascular disease or undifferentiated connective tissue disease until
more defined symptoms appear. The change into a more definable
disease may occur over years or never happen. Furthermore, the
undifferentiated features may, themselves, disappear at which point
there is no disease at all.
Rheumatoid arthritis is an autoimmune disease that causes chronic inflammation of the joints, the tissue around the joints, as well as other organs in the body. Because it can affect multiple other organs of the body, rheumatoid arthritis is referred to as a systemic illness and is sometimes called rheumatoid disease.
Sjögren's syndrome is an autoimmune disease involving the abnormal production of extra antibodies that attack the glands and connective tissue. Sjögren's syndrome with gland inflammation (resulting dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjögren's syndrome. Sjögren's syndrome that is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjögren's syndrome. Though there is no cure for Sjögren's syndrome, the symptoms may be treated by using lubricating eye ointments, drinking plenty of water, humidifying the air, and using glycerin swabs. Medications are also available to treat dry eye and dry mouth.
Systemic lupus erythematosus is a condition characterized by chronic inflammation of body tissues caused by autoimmune disease. Lupus can cause disease of the skin, heart, lungs, kidneys, joints, and nervous
system. When only the skin is involved, the condition is called discoid lupus.
When internal organs are involved, the condition is called systemic lupus
erythematosus (SLE).
Psoriatic arthritis is a disease that causes skin and joint inflammation. Symptoms include painful, stiff, and swollen joints, tendinitis, and organ inflammation. Treatment involves antiinflammatory medications and exercise.
Scleroderma is an autoimmune disease of the connective tissue. It is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body, leading to thickness and firmness of involved areas. Scleroderma is also referred to as systemic sclerosis, and the cause is unknown. Treatment of scleroderma is directed toward the individual features that are most troubling to the patient.
Marfan syndrome is hereditary condition affecting connective tissue. A person with Marfan syndrome may exhibit the following symptoms and characteristics: dislocation of one or both lenses of the eye; a protruding or indented breastbone; scoliosis; flat feet; aortic dilatation; dural ectasia; stretch marks; hernia; and lung collapse. Though there is no cure for Marfan syndrome, there are treatments that can minimize and sometimes prevent some complications.
Henoch-Schonlein Purpura (HSP or anaphylactoid purpura), a type of blood vessel inflammation, results in rash, arthritis, and occasional abdominal cramping. HSP often resolves on its own. Joint pain may be treated with anti-inflammatory and cortisone medications.
Polymyositis is a disease of the muscle featuring inflammation of the muscle fibers. It results in weakness of the muscles which can be severe and when associated with skin rash, is referred to as dermatomyositis. Although the cause of this disease is unknown, diagnosis includes physical examination of muscle strength, blood tests for muscle enzymes, electrical tests of muscle and nerves, and conformation by a muscle biopsy. Treatment of Polymyositis and Dermatomyositis includes high doses of cortisone-related medications, immune suppression, and physical therapy.
Kawasaki disease is a rare children's disease characterized by a fever that lasts more than five days and at least four of the following five symptoms are present: rash, swollen neck lymph gland, red tongue, swelling or redness of the hands or feet, and conjunctivitis. High doses of aspirin are used to treat Kawasaki disease. Cortisone and antiinflammatory drugs may also be used during treatment.
Wegener's granulomatosis, a condition that usually affects young or middle-aged adults, is an inflammation of the arteries supplying blood to the sinuses, lungs, and kidneys. Symptoms of Wegener's granulomatosis include bloody sputum, fatigue, weight loss, joint pain, sinusitis, shortness of breath, and fever. Wegener's granulomatosis may be fatal within months without treatment. Treatment aims to stop inflammation with high doses of prednisone and cyclophosphamide.
Ehlers-Danlos syndromes are genetic disorders that include symptoms such as loose joints, tissue weakness, easy bruising, and skin that stretches easily. There are seven types of Ehlers-Danlos syndromes: Classical type, Hypermobility type, Vascular type, Kyphoscoliosis type, Arthrochalsia type, Dermatosparaxis type, and Tenascin-X Deficient type. Treatment for Ehlers-Danlos syndromes depends on which symptoms are present.
Still's disease (Systemic-Onset Juvenile Rheumatoid Arthritis) is a disorder characterized by inflammation with high fever spikes, fatigue, salmon-colored rash and/or arthritis. Though there have been several theories regarding the cause(s) of Still's disease, the cause is not yet known. Many symptoms of Still's disease are often treatable with anti-inflammatory drugs.
Connective tissue diseases are disorders featuring abnormalities involving the collagen and elastin.
Connective tissue diseases that are strictly inheritable include Marfan syndrome and Ehlers-Danlos syndrome. The classic immune-related connective tissue diseases include systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymyositis, and dermatomyositis. Treatment is often directed at suppressing the inflammation present in the tissues by using anti-inflammatory and immunosuppressive medications.
Reactive arthritis is a chronic, systemic rheumatic disease characterized by three conditions, including conjunctivitis, joint inflammation, and genital, urinary or gastrointestinal system inflammation. Inflammation leads to pain, swelling, warmth, redness, and stiffness of the affected joints. Non-joint areas may experience irritation and pain. Treatment for reactive arthritis depends on which area of the body is affected. Joint inflammation is treated with antiinflammatory medications.
Takayasu disease (also referred to as Takayasu arteritis) is a chronic inflammation of the aorta and it's branch arteries. Takayasu disease is most common of Women of Asian descent and usually begins between 10 and 30 years of age. Symptoms include: painful extremities, dizziness, headaches, chest and abdominal pain, and a low-grade fever. Treatment for Takayasu disease includes cortisone medication to suppress the inflammation.
Polyarteritis nodosa is a rare autoimmune disease characterized by spontaneous inflammation of the arteries of the body. The most common areas of involvement include the muscles, joints, intestines (bowels), nerves, kidneys, and skin. Poor function or pain in any of these organs can be a symptom. Polyarteritis nodosa is most common in middle age persons. Polyarteritis is a serious illness that can be fatal. Treatment is focused on decreasing the inflammation of the arteries by suppressing the immune system.
Takayasu disease is a chronic inflammation of the large blood vessels that distributes blood from the heart.
The cause of Takayasu disease is unknown.
Symptoms of Takayasu disease include painful, cool, or blanched extremities, dizziness, headaches, chest pain, and abdominal pain.
The diagnosis of Takayasu disease involves detecting abnormal narrowing of the characteristic blood vessels.
The treatment of Takayasu disease involves suppressing the inflammation in the blood vessels. Surgical procedures can be required.
What is Takayasu disease?
Takayasu disease is a chronic inflammation of the large blood vessels that distribute blood from the heart, including the aorta and its main branches. Inflammation of blood vessels is also called vasculitis. It is most common in women (90%) of Asian descent. It usually begins between 10 and 30 years of age.