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Sarcoidosis is an inflammatory disease characterized by the presence of noncaseating granulomas (collections of inflammatory cells that do not issue pus) that form small lumps in multiple organs. Once thought rare, this condition is now known to be common. In fact, it is among the most common chronic scarring lung disorders.

Sarcoidosis can be either chronic (long-term) or acute (short-term). It nearly always affects more than one organ and can affect any organ in the body. The lungs and lymph nodes, especially those in the chest, are affected most often, with a lingering cough or shortness of breath as the most common symptoms. However, in some cases symptoms are not present.

The disease also frequently affects the skin, eyes and liver. Less commonly it affects the joints, bones, tear glands, salivary glands, spleen, heart, or the nerves or brain. When the disease affects the central nervous system, it is known as neurosarcoidosis, a serious complication. More rarely, sarcoidosis can affect the thyroid gland, kidneys, breasts or reproductive organs.

According to the National Institutes of Health, about 75 percent of sarcoidosis patients have the acute form of the disease and the remaining 25 percent have the chronic form. In about half of those with the acute form, the disease leaves no significant problems. Scar tissue is the most common long-lasting problem, particularly in chronic cases.

Although physicians and researchers do not know what causes sarcoidosis or even precisely what is going on in the bodies of these patients, it is believed that it may be caused by overreaction of the immune system in genetically susceptible individuals to an unidentified toxin, drug or pathogen. Some believe that this unknown factor may be a mycobacterium, a bacterium from the family that is linked to tuberculosis. This theory has not been substantiated with clinical research.

In normal circumstances, the immune system forms granulomas around particles, germs or other foreign substances to isolate and eliminate them. In patients with sarcoidosis, granulomas apparently form without a cause. There is no visible particle or germ enclosed by them. Many granulomas may clump together, forming small to large lumps. These lumps show up on x-rays as shadows and may affect organ function and cause symptoms.

Sarcoidosis affects men and women of all ages, races and ethnic groups worldwide. It typically develops between the ages of 20 and 40 years. It is rare in children and uncommon in adults over 50 years. People of African, Asian, German, Irish, Puerto Rican or Scandinavian descent are most commonly affected.

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Sarcoidosis is a disease characterized by inflammatory lumps (granulomas) that form in multiple organs. It can be either short- or long-term and can affect any organ, most often the lungs and lymph nodes. The granulomas seem to form as an overreaction to an unknown threat to the body.

The cause of sarcoidosis is not known, but it appears that some patients have genetic factors that make them susceptible. It may also affect different races, ethnic groups and populations in varying ways. For example, Japanese people are more likely to be affected in the heart and eyes, whereas in Northern Europeans the skin is affected along with another organ, such as the lungs. The disease may be related to infections similar to tuberculosis. Environmental factors, such as exposure to potentially toxic substances, may increase the risk of developing it.

Sarcoidosis usually has minor symptoms or none at all. Symptoms depend greatly on which organs are affected and to what extent. General symptoms include fever, fatigue and an overall unwell feeling. Symptoms of sarcoidosis in the lungs include shortness of breath, cough and chest pain.

Sarcoidosis is often first detected by x-ray. A series of other tests, including a biopsy of affected tissue, confirm diagnosis. The criteria for diagnosis are detection of the characteristic symptoms and abnormalities, particularly on a chest x-ray, ruling out infections and finding sarcoidosis granulomas in biopsy.

Many cases of sarcoidosis do not need treatment, but the disease can be serious and sometimes even fatal when it affects vital organs. When treatment is required, corticosteroids are normally used. Relapse is much more common when the condition is serious enough that medical treatment is required. Tests (e.g., breathing tests) are performed for a few years to monitor the disease whether or not treatment is required.

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Preparing questions in advance can help patients have more meaningful discussions with their physicians regarding their conditions. Patients may wish to ask their doctor the following questions about polymyalgia rheumatica (PMR):

1. Could my symptoms be due to polymyalgia rheumatica?
2. What diagnostic tests might I undergo, and what do they involve?
3. What do my test results show?
4. How can PMR affect other conditions I may have?
5. How likely is it that I also have temporal arteritis?
6. How frequently should I come in for checkups?
7. How is PMR typically treated, and which treatments do you recommend for me?
8. If corticosteroids are recommended for me, what is the dosage and the risks and benefits? How long will I likely need to take these drugs?
9. Should I alter my diet?
10. Which exercises should I consider?
11. Are there any exercises or activities I should avoid?

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There is no known way to prevent polymyalgia rheumatica (PMR). However, symptoms respond very well to treatment, and patients can usually return to their previous level of function.

Even without treatment, symptoms typically disappear after several years. With treatment, symptoms are often under control in just in few days, but they may recur if treatment is stopped too soon. If the symptoms do not disappear after a week of treatment, physicians will typically explore other possible diagnoses. Even after symptoms disappear, a minimum dose (maintenance dose) of medication is required for an extended period of time to suppress the disease.

Treatment focuses on reducing or eliminating the symptoms of PMR with a minimum of drug-induced side effects. Physicians may use blood tests during treatment to monitor the signs of PMR and adjust medication.

The most commonly prescribed medications for PMR are corticosteroids. In fact, some physicians consider the swift response of symptoms to corticosteroids among the criteria for the diagnosis of PMR. These are used to treat both PMR and temporal arteritis (a condition that causes arteries, particularly those in the head, to swell), but are used in higher doses for temporal arteritis.

Low doses of corticosteroids are used, especially if PMR is manifesting without the accompaniment of temporal arteritis. Often, patients report improvement after the first of these low-dose corticosteroids. If symptoms remain, doses may be increased for up to a week. As symptoms disappear, the dose is gradually reduced to the lowest effective dose.

Dosage must be reduced gradually because these drugs alter the body’s natural production of certain hormones. Stopping the medication suddenly can make a person very sick. Most people can stop taking corticosteroids in six months to two years, but treatment is occasionally prolonged beyond this. Corticosteroids may have negative long-term effects, including an increased risk of developing diabetes and a loss of bone density that may result in osteoporosis and fractures.

Regular follow-up appointments are important during and after treatment to catch any signs of relapse. If relapse occurs after corticosteroid therapy has ended, treatment will usually be restarted. As many as half of all PMR patients may experience a relapse. It is more common if the reduction of the corticosteroids occurs too quickly. It may be less common if other agents, such as those detailed below, are used along with the corticosteroids. However, treatment with other medications and corticosteroids remains a controversial point among physicians.

Even with low doses, corticosteroid treatment may result in numerous side effects (e.g., Osteoporosis involves the bones becoming thin, brittle and more prone to fracture, causing pain.osteoporosis, increased blood pressure). These are typically mild in the low doses used in the treatment of PMR but do remain a concern. All side effects should be reported to a physician. It is recommended that all patients on corticosteroids take calcium and vitamin D supplements to reduce the risk of osteoporosis.

Other medications that may be used in the treatment of PMR include:

* Antimetabolites. Slow the growth of certain cells. The addition of these drugs may control symptoms among patients at high risk of corticosteroid-induced side effects while the steroids are being reduced. This has also been suggested to reduce the rate of relapse. However, most PMR patients who are not also suffering from temporal arteritis, a condition often associated with PMR, do not need antimetabolite therapy.

* Nonsteroidal anti-inflammatory drugs (NSAIDs). Used alone, NSAIDs may provide a lesser degree of relief of PMR symptoms. They must be taken daily, although long-term use may damage the stomach or kidneys or have other side effects. In most cases, NSAIDs alone are not enough, but they may be added to corticosteroid therapy.

Although the symptoms of PMR respond very well to proper medication, patients may wish to take certain other factors into consideration:

* Exercise. Important to maintain joint flexibility and muscle strength and function. Exercise may be particularly useful in dealing with possible drug-induced side effects. Low-impact exercises (e.g., swimming, walking, tai chi) and stretching are usually emphasized. It is important to start slowly and work up gradually, particularly if the patient is not used to regular exercise.

* Nutrition. May help prevent potential problems associated with use of corticosteroids, such as thinning bones, high blood pressure and diabetes. Proper nutrition can also support the immune system. Fresh fruits and vegetables, whole grains and lean meats and fish are emphasized, as well as foods with plenty of calcium and vitamin D. Salt, sugar and alcohol are limited.

* Pacing. Physicians recommend that strenuous and/or repetitive tasks be alternated with easier ones to prevent muscle strain and fatigue. Plenty of rest is also important. This includes an adequate amount of sleep every night and rest time during the day. Occupational therapy may be prescribed to assist patients with energy conservation, task simplification and posture and ergonomics.

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As PMR is a diagnosis of exclusion, patients may have to through extensive workup to rule out other conditions before receiving the diagnosis. If a patient reports symptoms characteristic of PMR, a physician will review the medical history and perform a physical examination. Among the signs that the physician looks for is a decrease in the active range of motion in the neck, shoulders and hips. The physician may also check for muscle tenderness, but this is not a prominent feature of PMR. When it does occur, the tenderness is usually due to inflammation. The diagnostic procedure for PMR may be lengthier in patients who do not exhibit characteristic symptoms or presentations (e.g., patients younger than 50).

Laboratory and clinical tests follow the physical examination. Although no single test can be used to positively identify PMR, findings of multiple tests help to rule out other conditions that may be causing the signs and symptoms displayed. Tests that may be performed include:

* Blood tests. A number of tests may be used to detect abnormal levels of blood chemicals. In PMR:
- Sed rates are elevated.
- A C-reactive protein test reveals high levels of C-reactive protein (produced by the liver in response to injury or infection).
- An enzyme test reveals high levels of liver enzymes and normal levels of creatine kinase (a muscle enzyme).
- A complete blood count reveals high levels of platelets due to inflammation and low levels of red blood cells indicating mild anemia.
- Serologic tests (such as an ANA test for antinuclear antibodies) are negative.
- Rheumatoid factor (RF) tests are negative.

* Biopsies. Small tissue samples are removed for laboratory examination. Results are usually normal in PMR, but biopsies may be used to detect the presence of other conditions. Muscle biopsies reveal no damage that may cause the characteristic pain and stiffness. Temporal artery biopsy may be used to detect the presence of temporal arteritis, a condition that causes arteries, particularly those in the head, to swell.

Imaging tests may also be used to assist in ruling out other conditions. However, most physicians employ imaging tests only if the diagnosis remains uncertain.

Tests that may be performed include:

* MRI is an imaging test used in pain diagnosis, to guide treatment and to monitor for relapse.MRI (magnetic resonance imaging). Uses powerful magnets to produce images of internal structures. An MRI may confirm inflammation in the more distant joints (such as hands or feet) that is not restricted to the synovium.

* Ultrasound. Uses sound waves to create images of internal tissues. Ultrasound may reveal effusions (accumulation of fluid) in the shoulder. There is good correlation of results between ultrasound and MRI, so in most cases only one is used. Cost considerations typically favor ultrasound, but many physicians prefer MRI.

* X-ray. Images are produced using low doses of radiation. X-rays may be routine tests on inflamed joints. These rarely reveal any abnormalities in PMR.

* Electromyograms. Electrodiagnostics assess muscle function (e.g., electromyography [EMG], nerve conduction study).Graphic records of electrical activity in the muscle. These show no abnormalities.

Many conditions mimic the symptoms of PMR and may explain the findings. The presence of another disease excludes a diagnosis of PMR. For an accurate diagnosis of PMR, these conditions are usually excluded:

* Rheumatoid arthritis. A type of chronic inflammation that may disfigure joints. There may be some overlap between PMR and rheumatoid arthritis, particularly due to the age of the patients involved. However, rheumatoid arthritis is only partially responsive to low doses of corticosteroids. Fewer joints are swollen in PMR, and the swelling subsides completely with low doses of corticosteroids. Also, RF tests are positive in rheumatoid arthritis and negative in PMR. In the instances where patients have both rheumatoid arthritis and PMR, the two conditions will be treated separately.

* Hypothyroidism. Insufficient thyroid function. Some instances of this condition may result in swelling suggestive of PMR. Certain characteristics of hypothyroidism are not seen in PMR, such as the slow relaxation of deep tendon reflexes, low concentrations of an amino acid called tyrosine (T4) and high serum concentrations of a hormone called thyrotropin (also called thyroid-stimulating hormone, TSH).

* Infective endocarditis. Inflammation of the membrane surrounding the heart due to infection. A persistent fever accompanying the criteria for PMR may suggest infective endocarditis. Physicians may check for heart murmur, positive blood cultures and growth of bacteria on a heart valve (using echocardiogram).

* Fibromyalgia. A chronic condition involving widespread musculoskeletal pain. Many instances of fibromyalgia pain are similar to the pain associated with PMR. Most fibromyalgia patients are under age 50. Physical examination reveals no obvious abnormalities. Sed rate is normal.

* Cancer. There is no association between PMR and cancer, but malignancy can mimic the diffuse (scattered) muscle and joint pains of PMR. Cancer pain will not resolve with the use of corticosteroids.

* Polymyositis. Muscle inflammation and weakness. Proximal muscle weakness (in and around the torso) is associated with polymyositis, but prominent shoulder and/or hip pain is not. Diagnosis of this condition may be established by elevated muscle enzymes (e.g., creatine kinase), abnormal electromyogram and evidence of myositis (muscle swelling) on muscle biopsy.

* Bursitis or tendinitis. Inflammation of the bursae (fluid-filled cavities between tendons and bones) or tendons. PMR symptoms in the shoulders may be similar to those of bursitis or tendinitis. However, tenderness is minimal in most cases of PMR. Bursitis and tendinitis do not tend to affect both shoulders and there is no elevated sed rate. Some researchers, however, believe that a form of bursitis may be involved in the stiffness of the shoulders in PMR.

Certain disorders may coexist with PMR. A physician will usually test for:

* Temporal arteritis (also known as giant cell arteritis). A chronic disorder that results in the swelling of the arteries of the head, neck and arms. The temporal arteries, located on the temples on each side of the head, are most commonly affected. The exact nature of the relationship between temporal arteritis and PMR is not understood, but some evidence suggests that the conditions may represent a single disease spectrum. Both conditions affect the same demographic (white, over age 50) more often than others.

Symptoms of temporal arteritis that do not occur with PMR alone include headache, jaw pain, loss of vision and tenderness of the temporal arteries. Because of potentially serious complications of temporal arteritis, which can include blindness and stroke, physicians may actively monitor PMR patients for signs of the disease. According to the National Institutes of Health, about 15 percent of PMR patients also develop temporal arteritis, either at onset or after PMR symptoms disappear. If temporal arteritis is suspected, testing should include an eye exam. Treatment of temporal arteritis should be started earlier to prevent serious complications.

Corticosteroids are very effective in the treatment of both of these conditions, but temporal arteritis requires a higher dose. To determine the presence of temporal arteritis, a biopsy on a blood vessel in the scalp is usually performed. A small piece of the artery is removed and examined under a microscope.

* Remitting seronegative symmetrical synovitis with pitting edema syndrome (RS3PE). Typically marked by a sudden onset of arthritis in patients who are frequently over age 50 and do not possess factors of rheumatoid arthritis. The signs and symptoms of RS3PE are frequently mistaken for those of PMR. RS3PE generally responds well to corticosteroids, the predominant drugs used in the treatment of PMR. Some physicians consider this a variant of PMR with prominent edema.

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Symptoms of polymyalgia rheumatica (PMR) may develop suddenly – even overnight, in some cases – or they may develop gradually over several weeks. Both sides of the body are typically affected equally. However, pain and stiffness may be more prominent on one side than the other, particularly at the earliest stages of development.

The characteristic symptoms of PMR are moderate to severe pain and stiffness in and around the neck, shoulders and hips. This pain and stiffness is typically worse in the morning and after periods of inactivity (e.g., long car rides, watching movies). Pain generally improves over the course of the day but lasts at least 30 minutes.

Pain and stiffness may decrease the ability to actively move the neck, shoulders and hips and may be severe enough to rouse a patient from sleep. PMR patients may have trouble with the following and similar actions because of pain and stiffness:

* Getting out of bed
* Getting dressed
* Rising from a chair
* Getting into or out of automobiles
* Lifting arms overhead

The symptoms of PMR are often difficult to pinpoint, but the pain is usually said to come from the muscles around the joints. Most of the pain and stiffness occurs around the neck, shoulders and hips, but joints in other areas of the body may also ache. The affected areas may be tender to the touch, but this does not always occur.

Although muscles feel stiff and painful, muscle strength is usually normal. PMR does not damage muscle tissue. When muscle weakness is present, it is usually a problem due to muscle atrophy (deterioration) from disuse because of pain.

Pain and stiffness are not the only symptoms of PMR. Other symptoms may include:

* Arthritis (joint inflammation). The small joints of the hands and feet of PMR patients may develop arthritis, which is typically mild. In contrast to other forms, arthritis related to PMR may be brief and responds well to treatment.

* Swelling (edema). An increase in fluids in the tissues of the hands, wrists, ankles and/or tops Carpal tunnel syndrome is a compression of the median nerve in the wrist that causes wrist pain.of the feet may cause swelling. Knee joints may also swell. The swelling in the wrists may lead to symptoms of carpal tunnel syndrome, such as numbness and tingling in the fingers. Carpal tunnel symptoms occur in less than a quarter of PMR cases.

* Pitting edema. This occurs when a depression remains in swollen tissue after it is pressed. It usually accompanies other signs of PMR but may be the first sign noticed.

Other symptoms, including:

* Slight fever
* Malaise (general weakness and unwell feeling)
* Unexplained weight loss
* Loss of appetite
* Depression
* Night sweats
* Fatigue

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No causes of polymyalgia rheumatica (PMR) have yet been identified. The inflammation associated with PMR is known to be caused by the white blood cells attacking the synovium (lining of the joints), but why they do this is unknown.

There are certain factors that seem to increase the risk of developing PMR. These include:

* Genetics, race and sex. White women are affected more often than any other demographic. People of Northern European descent seem to be particularly at risk. This suggests that there may be a genetic susceptibility in certain individuals to PMR, but no research has been able to identify a particular genetic link.

* Age. Incidences of PMR in individuals under the age of 50 are extremely rare. Many researchers have suggested that PMR may be connected to the aging process. How it might be connected and to what degree remains controversial.

* Immune system abnormalities. The attack on the synovium by the white blood cells appears to be due to abnormalities in the immune system. The cause is not known.

* Climate. PMR is more prevalent in cooler climates. It is not known whether this is due to an environmental effect on PMR susceptibility or to the genetic background of the people who live in these environments.

* Viruses. Some research has indicated a possible link between PMR and certain viruses, such as adenovirus (any of a group of viruses that cause upper respiratory tract infections and conjunctivitis) and fifth disease (a viral disease that is usually mild and typically occurs in childhood).

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Polymyalgia rheumatica (PMR) is a rheumatic disease characterized by muscle pain and stiffness in and around the neck, shoulders and hips. Mild inflammation in the joints and surrounding tissues produces this pain, even though there is usually no sign of arthritis. The term “polymyalgia” means “pain in many muscles.”

PMR may develop as rapidly as overnight or more gradually over a period of weeks. It may occur in individuals who are in otherwise excellent health. PMR is generally a benign (not life-threatening) condition that usually goes away without treatment in several months to years, but it can be resolved more quickly with treatment.

The inflammation that characterizes PMR is the result of white blood cells attacking the synovium (lining of the joints), although it is not known why this occurs. The precise location of the inflammation is disputed, but many researchers and physicians believe it may be in or near the large joints, such as the shoulders and hips. However, other joints, such as those of the hands and feet, are also affected in some patients. This inflammation is not as severe as that caused by inflammatory types of arthritis and is usually not noticeable upon physical examination. Detection is made more difficult because large muscles cover the joints of the shoulders and hips.

PMR does not directly cause any muscle weakness or damage, but the muscles may atrophy (deteriorate) from disuse due to pain. Further, PMR is not itself life–threatening, but dangerous complications may arise because of medications and coexisting conditions.

PMR occurs almost exclusively in individuals over the age of 50, with an average age at onset of 70 years. It is twice as common in women as in men.  Although it can occur in any race or ethnic group, the vast majority of cases occur in whites. PMR affects about 700 per 100,000 Americans (less than 1 percent) over age 50, according to the National Institutes of Health.

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Also called: Arthritic Rheumatoid Disease, PMR, Anarthritic Syndrome

Polymyalgia rheumatica (PMR) is an essentially benign (not life-threatening) inflammatory disease characterized by pain and stiffness in the muscles in and around the neck, shoulders and hips. It may develop rapidly but usually goes away over several years, even without treatment. With treatment, symptoms typically disappear in less than a week.

PMR may occur along with temporal arteritis, a condition that causes arteries, particularly those in the head, to swell. It is not known how or why PMR and temporal arteritis are connected, but temporal arteritis can potentially be life-threatening and is typically tested for along with PMR.

The cause of PMR has not been identified, but white women over the age of 50 are most commonly affected. Theories on the possible causes of PMR include links to genetics, aging, climate and viral infections.

Muscle pain and morning stiffness are the characteristic symptoms of PMR. Other symptoms include arthritis, joint swelling, edema (swollen tissue) and flu-like symptoms (e.g., general weakness, feeling of being unwell, loss of appetite).

PMR is a clinical diagnosis, mainly a diagnosis of exclusion, meaning other conditions should be ruled out. The only blood test found positive is the sedimentation rate (sed rate). Most physicians agree that the following criteria must be met for diagnosis of PMR:

* Patient over age 50.
* Aching and stiffness on both sides of the body that lasts at least 30 minutes and persists for at least one month.
* Sed rate over 40 millimeters per hour. This blood test detects inflammation by measuring the rate at which red blood cells fall in blood plasma.

Some physicians add another requirement – that symptoms respond quickly to corticosteroids. Also, other conditions that may cause the necessary criteria (e.g., rheumatoid arthritis, fibromyalgia, polymyositis) must be ruled out.

PMR is typically treated with low-dose corticosteroids, which eliminate symptoms quickly. Once symptoms disappear, the dose is reduced to the lowest effective dose and treatment with this drug is eventually ended. However, if the dosage is reduced too quickly or treatment is ended too soon, a relapse may occur.

PMR affects less than 1 percent of the U.S. population over the age of 50, according to the National Institutes of Health.

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Preparing questions in advance can help patients have more meaningful discussions with their physicians regarding their conditions. Patients may wish to ask their doctor the following questions about myositis:

1. What form of myositis do I have?
2. How may myositis affect my other medical conditions?
3. How severe is my myositis likely to become?
4. What treatment options are available to me?
5. Which do you recommend?
6. How well is my myositis likely to respond to treatment?
7. How long will I have to remain on treatment?
8. How can my medications affect pregnancy? What other side effects might I experience?
9. How should I consider restructuring my life to work with my myositis?
10. What signs of relapse should I be on the lookout for?

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