Raynaud's also affects the ears and sometimes internal organs. Without treatment, Raynaud's phenomenon can damage or sometimes destroy the affected part of the body. When blood does not flow to the tissues, ulcers may develop. If blood flow is completely stopped for a long period of time, tissue may die and turn black. This condition can occur at any age, but usually begins between ages 20 and 40. It is more common in women than men.
A joint is where two bones meet to allow movement of body parts. Arthritis means joint inflammation. The joint inflammation of rheumatoid arthritis causes swelling, pain, stiffness, and redness in the joints. The inflammation of rheumatoid disease can also occur in tissues around the joints, such as the tendons, ligaments, and muscles. In some patients with rheumatoid arthritis, chronic inflammation leads to the destruction and deformity of the joints.
Rheumatoid arthritis is a common rheumatic disease, affecting more than two million people in the United States. The disease is three times more common in women as in men. It afflicts people of all races equally. The disease can begin at any age, but most often starts after age forty and before sixty. In some families, multiple members can be affected, suggesting a genetic basis for the disorder. Infectious agents such as viruses, bacteria, and fungi have long been suspected as the cause.
The symptoms of rheumatoid arthritis come and go, depending on the degree of tissue inflammation. When body tissues are inflamed, the disease is active. When tissue inflammation subsides, the disease is inactive (in remission). Remissions can occur spontaneously or with treatment, and can last weeks, months, or years. During remissions, symptoms of the disease disappear, and patients generally feel well. When the disease becomes active again, symptoms return. The return of disease activity and symptoms is called a flare. The course of rheumatoid arthritis varies from patient to patient, and periods of flares and remissions are typical.
When the disease is active, symptoms can include fatigue, lack of appetite, low grade fever, muscle and joint aches, and stiffness. Muscle and joint stiffness are most notable in the morning and after long periods of inactivity. Arthritis is common during disease flares. During flares, joints become red, swollen, painful, and tender. This occurs because the lining tissue of the joint (synovium) becomes inflamed, resulting in the production of excessive joint fluid (synovial fluid). The synovium also thickens with inflammation (synovitis).
In rheumatoid arthritis, multiple joints are usually inflamed in a symmetrical pattern (both sides of the body affected). The small joints of both the hands and wrists are frequently involved. Simple tasks of daily living, such as turning door knobs and opening jars can become difficult during flares. The small joints of the feet are also commonly involved. In occasional patients, only one joint is inflamed. When only one joint is involved, the arthritis can mimic the arthritis caused by gout or infection. Chronic inflammation can cause damage to body tissues, cartilage and bone. This leads to a loss of cartilage and erosion and weakness of the bones as well as the muscles, resulting in joint deformity and destruction. Inflammation can affect other organs and areas of the body. Inflammation of the glands of the eyes and mouth can cause dryness of these areas and is referred to as Sjogren's Syndrome. Rheumatoid inflammation of the lung lining (pleuritis) causes chest pain with deep breathing or coughing. Inflammation around the heart (pericarditis) causes chest pain which changes when lying down or leaning forward. The disease can reduce the number of red blood cells (anemia), and white blood cells. Decreased white cells can increase the risk of infections. Firm lumps under the skin (rheumatoid nodules) can occur around the elbows and fingers where there is frequent pressure. Even though these nodules usually do not cause symptoms, occasionally they can become infected. A rare, serious complication, usually with long-standing rheumatoid disease, is blood vessel inflammation (vasculitis). Vasculitis can impair blood supply to tissues and lead to tissue death. This is most often initially visible as tiny black areas around the nail beds or as leg ulcers.
Scleroderma can be classified in terms of the degree and location of the skin involvement. Accordingly, scleroderma has been categorized into two major groups, diffuse and limited.
The diffuse or systemic form of scleroderma involves symmetric thickening of skin of the extremities, face, trunk (chest, back, abdomen, or flanks) which can rapidly progress to hardening after an early inflammatory phase. Organ disease can occur early on and be serious. Organs affected include the esophagus, bowels, lungs with scarring (fibrosis), heart, and kidneys. High blood pressure can be a troublesome side effect.
The limited form of scleroderma tends to be confined to the skin of the fingers and face. The skin changes and other features of disease tend to occur more slowly than in the diffuse form. Because a characteristic clinical pattern can occur in patients with the limited form of scleroderma, this form has taken another name which is composed of the first initials of the common components. Thus, this form is also called the CREST variant of scleroderma. This name represents the following features:
Factors which activate the disturbances that cause lupus are not known. Genetics, viruses, ultraviolet light, and drugs may all play some role. Genetic factors increase the tendency of developing lupus. Diseases such as lupus, rheumatoid arthritis, and thyroid disorders are more common among relatives of patients with lupus than the general population. Some scientists postulate that the immune system in lupus patients is more easily activated by external factors like viruses or ultraviolet light. Sometimes, symptoms of lupus can be precipitated or aggravated by only a brief period of sun exposure. It is also known that women patients with systemic lupus not infrequently experience worsening of symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of those with the disease, suggest that female hormones play an integral role in the expression of systemic lupus.
In discoid lupus, only the skin is involved. Over time, 5 to 10% of patients with discoid lupus may develop systemic lupus. The skin involvement in discoid lupus is often found on the face and scalp. It is usually red, may have raised borders, and can scar. Discoid lupus skin rashes are usually painless and do not itch, but scarring of discoid skin lesions can cause permanent hair loss.
Patients with systemic lupus can develop different combinations of symptoms and organ involvement. Common complaints and symptoms include fatigue, low-grade fever, loss of appetite, muscle aches, arthritis, ulcers of the mouth and nose, facial rash ("butterfly rash"), unusual sensitivity to sunlight (photosensitivity), inflammation of the lining of the lung (pleuritis) and the heart (pericarditis), and poor circulation to the fingers and toes with cold exposure (Raynaud's phenomenon). More serious organ damage involves inflammation of the brain tissue, hepatitis, and kidney damage and failure. Certain blood cells and blood clotting factors can also be affected in systemic lupus, thereby increasing the risk of infection and bleeding.
Over half of the patients with systemic lupus develop a characteristic red, flat facial rash over the bridge of the nose. Because of its shape, it is frequently referred to as the "butterfly rash" of lupus. The rash is painless and does not itch. The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight, a condition called photosensitivity. This photosensitivity can be accompanied by a worsening of inflammation throughout the body, called a flare of disease.
Most systemic lupus patients will develop arthritis some time during the course of their illness. Arthritis in systemic lupus commonly involves swelling, pain, stiffness, and even deformity of the small joints of the hands, wrists, and feet. Sometimes, arthritis of systemic lupus can mimic that of rheumatoid arthritis.
Inflammation of the lining of the lungs (pleuritis) and of the heart (pericarditis) can cause sharp chest pain. The chest pain is aggravated by coughing, deep breathing, and certain changes in body posture. Kidney inflammation in systemic lupus can cause leakage of protein in the urine, fluid retention, high blood pressure, and even kidney failure. With kidney failure, machines are needed to cleanse the blood of accumulated poisons in a process called dialysis. Brain tissue involvement can cause personality changes, thought disorders (psychosis), seizures, and even coma. Damage to nerves can cause numbness, tingling, and weakness of the involved body parts or extremities. Many patients with systemic lupus experience hair loss (alopecia). Often, this occurs simultaneously with an increase in the activity of their disease. Some patients with systemic lupus have Raynaud's phenomenon. In these patients, blood supply to the fingers and toes becomes interrupted upon cold exposure, causing blanching, bluish discoloration, and pain in the exposed fingers and toes.
The onset of psoriatic arthritis generally occurs in the fourth and fifth decades of life. Males and females are affected equally. The skin disease (psoriasis) and the joint disease (arthritis) often appear separately. In fact, the skin disease precedes the arthritis in nearly 80% of patients. The arthritis may precede the psoriasis in up to 15% of patients. In some patients, the diagnosis of psoriatic arthritis can be difficult if the arthritis precedes psoriasis by many years. In fact, some patients have had arthritis for over twenty years before psoriasis eventually appears. Conversely, patients can have psoriasis for over 20 years prior to development of arthritis, leading to the ultimate diagnosis of psoriatic arthritis.
Psoriatic arthritis is a systemic rheumatic disease that can also cause inflammation in the eyes, heart, lungs, and kidneys. In psoriatic arthritis patients with arthritis of the spine, a genetic marker HLA-B27 is frequently found. Blood tests are now available to test for the HLA-B27 gene. Several other genes have also been found to be more common in patients with psoriatic arthritis. The importance of infectious agents and other environmental factors in the cause of psoriatic arthritis is being investigated.
In most patients, the psoriasis precedes the arthritis by months to years. The arthritis frequently involve the knees, ankles, and joints in the feet. Usually, only a few joints are inflamed at a time. The inflamed joints become painful, swollen, hot, and red. Sometimes, joint inflammation in the fingers or toes can cause swelling of the entire digit, appearing like sausages.
Joint stiffness is common, and is worse early in the morning. In a few patients, psoriatic arthritis may involve many joints of the body in a symmetrical fashion, mimicking the pattern seen in rheumatoid arthritis. Psoriatic arthritis can also cause inflammation of the spine (spondylitis) and the sacrum, causing pain and stiffness in the low back, buttocks, neck and upper back. In rare instances, psoriatic arthritis involves the small joints at the ends of the fingers. A very destructive form of arthritis, called "mutilans," can cause rapid damage to the joints. Fortunately, this form of arthritis is rare in patients with psoriatic arthritis.
Patients with psoriatic arthritis can also develop inflammation of the tendons (tendonitis) and around cartilage. Inflammation of the tendon behind the heel causes Achilles tendonitis, leading to pain with walking and climbing stairs. Inflammation of the chest wall and of the cartilage around the breastbone can cause chest pain, as seen in (costochondritis).
Aside from arthritis and spondylitis, psoriatic arthritis can cause inflammation in other organs. Inflammation around the eyes causes iritis, a painful condition that can be aggravated by bright light. Inflammation in and around the lungs (pleuritis) causes chest pain and shortness of breath. Inflammation of the aorta (aortitis) can cause leakage of the aortic valve valves, leading to heart failure and shortness of breath.
Acne and nail changes are commonly seen in psoriatic arthritis. Pitting and ridges are seen in finger and toe nails of 80% of patients with psoriatic arthritis. Interestingly, these characteristic nail changes are observed in only 20% of psoriasis patients who do not have arthritis. Acne has been noted to occur in higher frequency in patients with psoriatic arthritis. A new syndrome has been described, characterized by inflammation of the joint lining (synovitis), acne and pustules on the feet or palms, thickened and inflamed bone (hyperostosis), and bone inflammation (osteitis). This syndrome is therefore given the eponym SAPHO syndrome.
Reiter's syndrome most frequently occurs in patients in their thirties or forties, but it can occur at any age. The form of Reiter's syndrome which occurs after genital infection occurs more frequently in males. The form which develops after bowel infection occurs in equal frequency in males and females.
Reiter's syndrome is considered a systemic rheumatic disease. This means it can affect other organs than the joints, such as the eyes, mouth, skin, kidneys, heart, and lungs. Reiter's syndrome shares many features with several other arthritic conditions, such as psoriatic arthritis, ankylosing spondylitis, and arthritis associated with Crohn's disease and ulcerative colitis. Each of these arthritic conditions can cause similar disease and inflammation in the spine and other joints, eyes, skin, mouth, and various organs. In view of their similarities and tendency to inflame the spine, these conditions are collectively referred to as "spondyloarthropathies."
As mentioned, Reiter's syndrome is felt in part to be genetic. There are certain genetic markers that are far more frequent in patients with Reiter's syndrome than in the normal population. For example, the HLA-B27 gene is commonly seen in patients with Reiter's syndrome. Even in patients who have the genetic background that predisposes them to developing Reiter's syndrome, exposure to certain infections seem to be required to trigger the onset of the disease.
Reiter's syndrome can occur after genital infections. The most common bacteria that has been associated with this form of Reiter's syndrome is an organism called Chlamydia. Reiter's syndrome also occurs after infectious dysentery, with bacterial organisms in the bowel, such as Salmonella, Shigella, Yersinia, and Campylobacter. Typically, the arthritis develops one to three weeks after the onset of the bacterial infection.
The symptoms of Reiter's syndrome can be divided into those which affect the joints and those which affect the non-joint areas. The classic joints that become inflamed in Reiter's syndrome are the knees, ankles, feet, and wrists. The particular joints involved are usually asymmetric, that is, one side of the body or the other is affected, rather than both sides simultaneously. The inflammation leads to stiffness, pain, swelling, warmth, and redness of the joints involved. Patients may develop inflammation of entire fingers or toes which can give the appearance of a "sausage digit." This is also seen in patients with another type of arthritis associated with psoriasis, called psoriatic arthritis. The arthritis of Reiter's syndrome can be associated with inflammation of the spine, leading to stiffness and pain in the back or neck (characteristic of all of the spondyloarthropathies). Cartilage can also become inflamed, especially around the breastbone where the ribs meet in the front of the chest, this condition is called costochondritis. Muscles attach to the bones by tendons. In Reiter's syndrome, the tendon insertion points can become inflamed (tendonitis), tender, and painful when exercised.
Non-joint areas that become inflamed and cause symptoms in Reiter's syndrome include the eyes, genitals, urinary tract (urethra, bladder and prostate gland), mouth lining, large bowel, and the aorta. Inflammation of the whites of the eye (conjunctivitis) and the iris of the eye (iritis) is frequently seen early in Reiter's syndrome and may be intermittent. When the whites of the eye are inflamed causing conjunctivitis, there may be no pain. When the colored part of the eye (iris) is inflamed, causing iritis, it can be very painful and especially worse when looking into bright lights. Urinary tract inflammation commonly involves the urethra, the tube that drains urine from the bladder. This inflammation (urethritis) can be associated with burning on urination and/or pus drainage from the end of the penis. The skin around the penis can become inflamed and scale. The bladder and prostate gland can also become inflamed, leading to an urge to urinate. The mouth can develop open sores (ulcerations) on the hard and soft palate, and even on the tongue. These may go unnoticed by the patient, as they are often painless. Inflammation of the large bowel (colitis) can cause diarrhea, or pus or blood in the stool. Inflammation of the aorta (aortitis) can be seen in a small percentage of patients who have Reiter's syndrome. It can lead to failure of the aortic valve of the heart, which can cause heart failure. The electrical conducting pathway of the heart can also become scarred in Reiter's syndrome, leading to irregular heartbeats (arrhythmias) that may require placement of a pacemaker to regulate the heartbeat.
Ankylosing spondylitis is nine times more common in males than in females. In women, joints away from the spine are more frequently affected than in men. Ankylosing spondylitis affects all age groups, including children. The most common age of onset of symptoms is in the second and third decades of life. The tendency for developing ankylosing spondylitis is believed to be genetically inherited, and the majority (90%) of patients with ankylosing spondylitis are born with the HLA-B27 gene. Even among HLA-B27 positive individuals, the risk of developing ankylosing spondylitis appears to be further related to heredity. In HLA-B27 positive individuals who have relatives with the disease, their risk of developing ankylosing spondylitis is 12% (6 times greater than for those whose relatives do not have ankylosing spondylitis).
The symptoms of ankylosing spondylitis are related to inflammation of the spine, joints, and other organs. Inflammation of the spine causes pain and stiffness in the low back, upper buttock area, neck, and the remainder of the spine. The onset of pain and stiffness is usually gradual and progressively worsens over months. Occasionally, the onset is rapid and intense. The symptoms of pain and stiffness are often worse in the morning, or after prolonged periods of inactivity. The pain and stiffness are often eased by motion, heat and a warm shower in the morning. Because ankylosing spondylitis often affects patients in adolescence, the onset of low back pain is sometimes incorrectly attributed to athletic injuries in younger patients.
Patients who have chronic, severe inflammation of the spine can develop a complete bony fusion of the spine (ankylosis). Once fused, the pain in the spine disappears, but the patient has a complete loss of spine mobility. These fused spines are particularly brittle and vulnerable to breakage (fracture) when involved in trauma, such as motor vehicle accidents. A sudden onset of pain and mobility in the spinal area of these patients can indicate bone fracture. The lower neck (cervical spine) is the most common area for such fractures.
Chronic spondylitis and ankylosis cause forward curvature of the upper torso (thoracic spine), limiting breathing capacity. Spondylitis can also affect areas where ribs attach to the upper spine, further limiting lung capacity. Ankylosing spondylitis can cause inflammation and scarring of the lungs, causing coughing and shortness of breath, especially with exercise and infections. Therefore, breathing difficulty can be a serious complication of ankylosing spondylitis.
Patients with ankylosing spondylitis can also have arthritis in joints other than the spine. Patients may notice pain, stiffness, heat, swelling, warmth, and/or redness in joints such as the hips, knees, and ankles. Occasionally, the small joints of the toes can become inflamed, or sausage-shaped. Inflammation can occur in the cartilage around the breast bone (costochondritis) as well as in the tendons where the muscles attach to the bone (tendinitis). Some patients with this disease develop Achilles tendinitis, causing pain and stiffness in the back of the heel, especially when pushing off with the foot while walking up stairs.
Other areas of the body affected by ankylosing spondylitis include the eyes, heart, and kidneys. Patients with ankylosing spondylitis can develop inflammation of the iris, called "iritis." Iritis is characterized by redness and pain in the eye, especially when looking at bright lights. Iritis can be a serious complication of ankylosing spondylitis, and may require an eye specialist's (ophthalmologist) supervision.
A rare complication of ankylosing spondylitis involves scarring of the heart's electrical system, causing an abnormally slow heart rate. A heart pacemaker may be necessary in these patients to maintain adequate heart rate and output. The part of the aorta closest to the heart can become inflamed, resulting in leakage of the aortic valve. These patients can develop shortness of breath, dizziness, and heart failure.
Advanced spondylitis can lead to deposits of protein material called amyloid into the kidneys and result in kidney failure. Progressive kidney disease can lead to chronic fatigue and nausea and can require removal of accumulated blood poisons by a filtering machine (dialysis).
Polymyositis is slightly more common in females. It affects all age groups, although its onset is most common in middle childhood and in the twenties. It occurs throughout the world. It can be associated with skin rash and is then referred to as dermatomyositis. It also can affect other areas of the body and is, therefore, a systemic illness. Occasionally, it is associated with cancer, or with other diseases of connective tissue (see systemic lupus erythematosus, scleroderma and rheumatoid arthritis).
There are indicators of heredity (genetic) susceptibility that can be found in some patients. There is indirect evidence of infection by a virus that has yet to be identified in a form of polymyositis that is particularly resistant to treatment, called inclusion body myositis. This form of polymyositis is diagnosed by the pathologist, a physician specialist who interprets the microscope findings of muscle tissue. The muscle tissue in this form of polymyositis displays clear areas within the muscle cells (called vacuoles) when viewed under the magnification of a microscope.
Weakness of muscles is the most common symptom of polymyositis. The muscles involved usually are those that are closest to the trunk of the body. The onset can be gradual or rapid. This results in varying degrees of loss of muscle power. The loss of strength can be noticed as difficulty getting up from chairs, climbing stairs or lifting above the shoulders. Trouble with swallowing and weakness lifting the head from the pillow can occur. Occasionally the muscles ache and are tender to the touch (25%). Patients can also feel fatigue, a general feeling of discomfort and have weight loss and/or low-grade fever.
With skin involvement (dermatomyositis), the eyes can be surrounded by a violet discoloration with swelling. There can be scaly reddish discoloration over the knuckles, elbows and knees (Gottron's sign). There can also be reddish rash on the face, neck and upper chest. Hard lumps of calcium deposits can develop in the fatty layer of the skin, most commonly in childhood dermatomyositis. Heart and lung involvement can lead to irregular heart rhythm and shortness of breath.