Dinner Fork Deformity Rheumatic Chorea

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Rheumatic fever is caused by an immunological reaction to an infection with a group A beta-hemolytic streptococcus. In some people this organism is part of the bacterial population normally living in the nose and throat, but it can be invasive and is the cause of strep throat. Streptococcal throat infection itself is often self-limiting, even without treatment. However, one cannot predict which infections are likely to precipitate heart disease, and therefore all are treated with antibiotics.

Scientists do not know why the streptococcus sometimes lives happily as part of the usual bacterial population and at other times causes streptococ-cal throat infection that can lead to rheumatic fever. Only about 1 percent of streptococcal infections lead to rheumatic fever. The genetic makeup of both the patient and the streptococcus seem to be important determinants of the type of infection and who goes on to develop rheumatic fever. Rheumatic fever is caused by antibodies against the streptococcus reacting against the patient's own tissues.


Rheumatic fever occurs two to five weeks after a streptococcal throat infection. The throat infection may have been minor and by the time rheumatic fever occurs has usually resolved. Fever and arthritis are common symptoms. The fever can be very high and responds to treatment with aspirin. Arthritis tends to affect large joints such as the knee, hip, ankle, wrist, and elbow. Arthritis tends to be flitting, moving from one joint to another, or additive, adding a new joint to the one that is already affected. Arthritis in rheumatic fever causes severe pain that is out of proportion to the amount of swelling or obvious inflammation. The pain can be so severe that the child is unable to walk.

other less common findings are a rash called erythema marginatum, subcutaneous nodules, a movement disorder called Sydenham's chorea, and carditis. Erythema marginatum is usually a flat, pinkish rash with a clear margin, sometimes with clearing in the center. it can be very subtle and evanescent. Subcutaneous nodules are found in rheumatic fever but are rare. These nodules are usually smaller than a pea and are found over tendons. Sydenham's chorea is a movement disorder that causes abnormal movements of the hands and face. The patient often disguises these involuntary movements by turning the movement into a semi-purposeful action. The result is that sometimes children with chorea are thought to be very fidgety or unable to sit still. Sydenham's chorea can recur years later during pregnancy without any symptoms of another attack of rheumatic fever. Carditis is inflammation of the heart. During acute rheumatic fever myocarditis, pericarditis, and endocarditis can occur. Myocarditis, inflammation of the heart muscle, often causes an increased heart rate and can cause heart failure. Pericarditis, inflammation of the outside lining of the heart, can cause chest pain and a pericardial effusion (fluid around the heart). Endocarditis, inflammation of the heart valves, causes a murmur, a noise caused by blood flowing across an abnormal heart valve.

Repeated attacks of rheumatic fever can cause chronic, permanent damage to heart valves. This can cause heart rhythm problems, enlargement of the heart, and heart failure.


The diagnosis of acute rheumatic fever is clinical. The Jones criteria are used for classification, but some patients with rheumatic fever do not fulfill the criteria. The Jones criteria require laboratory evidence of a streptococcal infection and the presence of two major criteria or one minor and one major criterion. The major criteria are carditis (myocarditis, pericarditis, or endocarditis), erythema marginatum, subcutaneous nodules, chorea, and arthritis. The minor criteria include fever, an elevated erythrocyte sedimentation rate (ESR), arthralgia, and a history of a previous attack of rheumatic fever.

A throat swab may still be positive for streptococcus when rheumatic fever develops, or if it is negative, an elevated antistreptolysin antibody level will suggest a recent streptococcal infection. The ESR is usually elevated, but other blood tests are not helpful. An electrocardiogram can show an increased PR interval, a widening of the distance between the P and R waves. An echocardiogram may show pericarditis or abnormalities of the heart valves.

Treatment and Outcome Preventing rheumatic fever is possible by treating streptococcal throat infections, particularly in children. If rheumatic fever develops, an NSAID, most often high doses of aspirin, relieves fever and arthritis. Penicillin is used to eliminate any remaining streptococci, and occasionally if there is carditis, a course of corticosteroids is prescribed. The acute attack subsides within a few months, However, if a patient has had one attack of rheumatic fever preventing further attacks is important. The risk of permanent damage to the heart valves is much higher after repeated attacks of rheumatic fever. A long-term antibiotic, most often a monthly injection of long-acting penicillin, is prescribed to prevent this. if there is permanent damage to heart valves, surgery to repair or replace these may be required. Heart valves that have been damaged by rheumatic fever have a greater risk of becoming infected (infective endocarditis).

rheumatoid arthritis (RA) A chronic inflammatory autoimmune disease that fluctuates in severity and affects the joints, causing swelling, pain, loss of function, and eventually deformity. It can also affect other organs. Purists maintain it should therefore be called rheumatoid disease rather then rheumatoid arthritis. RA has a major socioeconomic impact, not only by causing disability and decreasing the earning capacity of affected people but also by increasing use of medical resources such as doctor visits, hospital admissions, and surgery. In the last 20 years improvements in the drugs available to treat RA and recognition that early control of the disease is important have improved outcomes.


RA affects about 1 percent of the population in most countries. It is rare in some populations such as rural West Africans and more frequent in some, such as Pima Indians. The cause of RA is not known. Several ideas have been proposed—most often that it is either an infectious or genetic disease.

Infection An infectious cause has been suspected and hunted for decades but has never been convincingly or reproducibly proven. Patients with RA make antibodies to a wide range of antigens which has sometimes led researchers down misleading trails.

one of the first organisms suggested as a cause of RA was the Epstein-Barr virus (EBV), the organism that causes infections mononucleosis (mono). In the 1970s, it was found that many patients with RA had antibodies against the virus, and speculation mounted that the virus played a role in the cause of RA. This was an attractive theory because it was known that EBV activated B lymphocytes and was associated with rare types of cancer, suggesting it could interact with the immune system. Most adults have been infected with EBV at some time. Many experts think RA stimulates the B lymphocytes to produce many antibodies, one of which is the antibody against EBV, and that the virus does not cause RA.

Several viral illnesses such as rubella, parvovirus, HEPATITIS, and HUMAN IMMUNODEFICIENCY virus (HIV) (see viral arthritis) can cause arthritis, but efforts to isolate a specific virus from patients with RA have not been successful. Similarly, infection with Mycoplasma, Chlamydia, and Proteus have been considered as causes of RA, but the evidence is not convincing.

Genetic causes Undoubtedly genetic factors play a part in determining who gets RA, because it tends to run in families. If someone has a parent or sibling with RA, that person's risk of getting the disease is doubled. However, genetic factors are not the whole answer. If RA were entirely genetic, one would expect that identical twins would have 100 percent concordance. If one identical twin gets RA, though, there is about a 20 percent chance that the other twin, who has exactly the same DNA, will develop RA.

The genetic factors that increase the risk of RA are not clear (see hla and genetic factors). Rheumatoid arthritis has been known to be associated with HLA tissue types since the 1970s, with the risk of RA increased fourfold in individuals with the HLA-DR4 tissue type (new nomenclature HLA-DRB1 0401, 0402, and so on, for the different variants of the 04 tissue type). The strength of the association varies in different populations and is weaker in African-American than in Caucasian populations. Several research groups are performing genome-wide scans where the whole DNA sequence is examined in families with RA for markers that track with RA to try to localize the search to smaller areas of the genome. So far, other than confirming the association with the HLA region, the results have not been reproducible.

Other possible risk factors Women develop RA three times as often as men and RA often seems to improve in pregnancy, suggesting that hormones may influence the immune system in ways that alter the risk of developing RA.

Cigarette smoking is associated with about a 50 percent higher risk of developing RA, and RA is more severe in smokers. The mechanism is not clear, but smoking stimulates many liver enzymes that metabolize drugs and chemicals. These enzymes possibly convert an unidentified chemical into a by-product that affects the risk of RA.

RA is much less common in West Africa than it is in the United States, although critics argue that scientists are not certain about this because so few studies have been done in Africa. Observational studies from

Africa also suggest that as people move into urban areas, where the risk of infections such as malaria is decreased, the risk of RA increases. It has been proposed that chronic infection with an organism such as malaria may stimulate the immune system and protect against RA and that the Western-type lifestyle with excellent hygiene may increase risk. An argument against this is that recent evidence suggests that the incidence of RA is decreasing in developed countries such as the united States.

How RA Causes Disease

Although scientists do not know what causes RA, they do have an excellent understanding of how RA causes disease. Inflammation of the synovium is central to the pathogenesis of RA. The synovium that is normally thin becomes thickened, hypertro-phied, and filled with fibroblasts and inflammatory cells such as lymphocytes and macrophages. Small new blood vessels form (angiogenesis). The proliferating synovium, called a pannus, replaces normal synovium and is locally invasive, damaging the underlying bone and cartilage.

Several cell types play an important role. T lymphocytes were thought to be the key cell driving RA, and drug companies produced antibodies that effectively depleted T cells in patients with RA. Surprisingly, this had little effect on disease activity, suggesting that perhaps T cells were not critical to the development of RA. current opinion is that T cells do play a role in the pathogenesis of RA, but other cells such as macrophages, which produce cytokines, are also important. B lymphocytes produce antibodies, including rheumatoid factor, which is discussed later. Many mediators such as the cytokines tumor necrosis factor (TNF) and interleukin-1 and the chemokines amplify and sustain the inflammatory process. The strategy of developing drugs to block cytokines has led to effective new drugs for RA.


The key symptoms and laboratory findings in RA are shown in the Table.

General symptoms RA is an illness that affects much more than just the joints, and systemic symptoms are common. Symptoms often start gradually, over months. Patients often experience difficulty pinning down exactly when their illness started. The first symptoms are often vague and include fatigue, lack of energy, feeling depressed or washed out, and stiffness in the morning that lasts longer than 30 minutes. The stiffness in most noninflammatory causes of arthritis such as osteoarthritis lasts only a short time and improves once the patient is moving, whereas the stiffness in inflammatory diseases is much more severe and long lasting. It is not unusual for patients to say that their morning stiffness lasts until they take a hot bath or place their hands under running hot water. Loss of weight can occur with more severe RA and is part of the systemic illness. Lymph nodes can increase in size. This does not usually cause symptoms. However, if a physician notices the enlarged nodes, it may trigger a series of tests to make sure the patients does not have cancer, infection, or lymphoma.

Joint (articular) symptoms Typically RA affects many small joints in a symmetrical distribution at the same time. The metacarpophalangeal and proximal interphalangeal joints (knuckles) of






Morning stiffness


Rheumatoid nodules



Symmetrical swelling


High platelet count

Low-grade fever


Lung disease

High ESR

Lack of energy



Rheumatoid factor


Loss of function

Eye inflammation

Normal white cell count

Loss of weight


Felty's syndrome

Erosions on X ray

Lymph nodes


Sjogren's syndrome

Inflammation on imaging

both hands and wrists are most often affected. in some patients, though, the illness first causes symptoms in the small joints of the feet. Affected joints show signs of inflammation such as warmth, swelling, pain, and reduced movement. in a few patients joint swelling and other symptoms can occur in short, sharp attacks that resolve completely until they recur. This is called palindromic rheumatism. More frequently, however, RA causes persistent symptoms in joints that may fluctuate in severity.

RA has a predilection for the small joints of the hands and feet, but most joints can be affected. Listing those that are seldom or never affected is easier. RA seldom affects the distal interphalangeal joints (the last joint of the fingers) and does not affect the lower back. it can affect hips, knees, shoulders, wrists, ankles, cervical spine (neck), and temporomandibular joint (jaw). The joints of the small bones in the larynx (voice box) can become inflamed, causing hoarseness or narrowing of the airway. Symptoms vary in severity. Some patients are so severely affected that they are unable to function, and others are able to continue their day-to-day activities with some limitations.

Early in the illness swelling predominates, and this may lead to restricted movement of a joint and affect the patient's ability to make a fist or grip objects. Later in the illness permanent deformities can occur. one of the most common is called ulnar deviation. if a patient stretches out his or her fingers with the palms facing the ground, the fingers instead of being straight point outward, away from the rest of the hand. Early in the disease the patient can correct the deformity by using other muscles to pull the fingers straight, but later the deformity becomes fixed and permanent. other common deformities affect the fingers and wrist. A boutonniere (buttonhole) deformity affects the middle knuckle of the finger so that the joint is permanently flexed. it is called a boutonniere deformity because the joint sticks out between two slips of a tendon so that the anatomy looks like a button popping out of a buttonhole. A swan neck deformity is when the end joint of the finger is flexed and the middle joint is hyperextended, so the finger ends up resembling a swan's neck. if the wrist is chronically inflamed the whole hand can migrate downward off the wrist, resulting in a step-down or dinner fork deformity. These deformities can be corrected surgically, but such procedures do not necessarily improve function.

Late in the disease affected joints permanently lose their ability to move through a normal range of motion or even fuse and lose their ability to move at all. Fusion is a mixed blessing because while joints that cannot move do not fulfill their normal function, they are also frequently less painful. in fact, a common surgical treatment for chronic pain in a joint that does not respond to medical treatment and where movement is not vital is surgical fusion (arthrodesis).

Joints move, stabilize, absorb shock, and help with balance. The major symptom of arthritis, apart from pain, is loss of function. This may affect daily activities such as walking, eating, dressing, and bathing but can also affect a person's ability to hold a particular job. A concert pianist and a bricklayer may seem to require very different tasks from their joints, but both jobs demand hands that can function effortlessly. Frequently patients with RA have to modify their lifestyle, hobbies, and employment because of limitations imposed by their arthritis.

Extra-articular symptoms RA affects not only the joints but can also affect almost any organ. For this reason many experts prefer to call it rheumatoid disease rather than rheumatoid arthritis.

Rheumatoid nodules Nodules ranging in size from smaller than a quarter-inch to larger than an inch (rheumatoid nodules) are a frequent manifestation of extra-articular disease and are a marker of more severe and aggressive RA.

Pleurisy The main symptom of pleurisy is chest pain that gets worse on taking a deep breath or coughing. These symptoms can also occur in patients who have a broken rib or inflammation or infection of the lung. Many patients with RA have mild pleurisy without knowing about it. More severe disease causes symptoms of pleurisy and sometimes a collection of fluid in the space between the lung and its lining (pleura). This is called a pleural effusion, and because it can be caused by cancer, pneumonia, infection, and many other diseases, it often starts a train of tests to try and find the cause. unfortunately, there is no specific test that differentiates between a pleural effusion caused by RA and other diseases. The pleural fluid in RA

often has a very low glucose concentration, but this finding is not specific.

Lung disease In addition to causing pleurisy, RA can affect the lungs in several other ways. The most common is called interstitial fibrosis, which causes streaks of scarring throughout the lung tissue. Many patients with interstitial fibrosis do not have any symptoms early on. However, if a chest X ray is taken, it can show scarring even at this stage. Why RA causes interstitial fibrosis is not clear, but male sex, severity of RA, and smoking increase the risk. Severe interstitial fibrosis causes shortness of breath and cough, and it decreases exercise capacity. Tests of lung function usually show what is called a restrictive defect, in other words, the volume of the lungs is decreased. Very occasionally lung disease can occur before arthritis symptoms occur, resulting in confusion about the diagnosis.

RA can cause rounded nodules within the lung. This used to occur more commonly in people with pneumoconiosis, an occupational lung disease usually acquired from working in coal mines, but nodules can occur in any patient. When only one or a few nodules are seen it can be very difficult to differentiate this from cancer, and a biopsy is often required.

Pericarditis Just as fluid can accumulate between the lung and its lining layer, it can also accumulate between the heart and its lining layer, the pericardium. Again, many patients with RA have mild pericarditis that does not cause major symptoms or problems, and they are not aware of it. Mild asymptomatic pericarditis does not need any specific treatment. More severe disease that causes chest pain and a large pericardial effusion should be treated. The symptoms of a pericardial effusion are variable but can include chest pain or tightness, edema, and shortness of breath. Typically the chest pain from pericarditis is felt in the middle of the chest, varies with position, and can get worse with breathing. other heart problems are rare, but rheumatoid nodules have been found in the heart, where they may cause rhythm problems.

Eye inflammation Dry eyes, as a component of SJOGREN'S syndrome accompanying RA, are common but serious inflammation of the eye that threatens vision is uncommon. Scleritis, or inflammation of the fibrous layer of the eye, can cause pain, redness, and more seriously, thinning and weakness of the eyeball. if this happens, the white part of the eye looks blue or black because thinning of the lining allows the darker interior to show. inflammation of the sclera usually causes a red, painful eye. The pain is deeper, more severe, and more aching in character than the superficial scratchiness caused by conjunctivitis or episcleritis. Vision can be affected, and scleritis should be treated by an ophthalmologist. Treatment involves management of the underlying RA and may require corticosteroids and immunosuppressive drugs. In patients with severe RA, rheumatoid nodules can occur in the eye and cause the sclera to become thin, and if inflammation is not controlled, to weaken so that the inside of the eye ruptures through the wall and blindness results. (See also eye problems.)

Felty's syndrome This rare complication of RA is discussed separately (see felty's syndrome).

Sjogren's syndrome See SJOGREN'S syndrome.

Other symptoms RA rarely affects other organs such as the liver, kidneys, or peripheral nerves, but rheumatoid vasculitis can occur and affect these organs. if symptoms of kidney disease such as pro-teinuria develop, they are more likely to have been caused by drugs used to treat RA than the disease.

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Arthritis Relief Now

Arthritis Relief Now

When you hear the word arthritis, images of painful hands and joints comes into play. Few people fully understand arthritis and this guide is dedicated to anyone suffering with this chronic condition and wants relief now.

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  • Joseph
    How to treat dinner folk deformity in rhematoid arthritis?
    4 years ago
  • filiberta
    Can deformities still happen on ra medication?
    1 year ago

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