Not smoking.

Avoiding substances like dusts and chemicals that can harm the lungs.

Trying to follow a healthy eating plan.

Being as active as possible without straining.

Joining a patient support group may help adjust to living with sarcoidosis. Talking to others who have the same symptoms can help a person to see possible similarities, as well as alternatives regarding treatment and health care management. There are times when a shared experience can be very useful when coping with the challenges of sarcoidosis.

A patient’s regular doctor may be able to diagnose and treat the sarcoidosis, but diagnosis and treatment by a doctor who specializes in sarcoidosis is recommended. If a person prefers to use their regular doctor, they should see a doctor who specializes in the organs that are affected by their sarcoidosis at least once. For example, an ophthalmologist should be seen if the eyes are affected or a pulmonologist if the sarcoidosis is in the lungs. These specialists are often found at major medical centers. They will work with a person’s regular doctor to help make a diagnosis, develop a treatment plan, and schedule periodic exams and lab tests.

Pregnancy

Many women give birth to healthy babies while being treated for sarcoidosis. Pregnancy usually doesn’t affect the course of sarcoidosis, and person can continue corticosteroid treatment through their pregnancy. None of the other drugs are recommended for use during pregnancy.

Sometimes sarcoidosis may get worse after the baby is delivered.

Women with severe sarcoidosis, especially if they are older, may have trouble becoming pregnant.

It’s important for a person to discuss this issue with their doctor. A patient who becomes pregnant should be sure to get both good prenatal care and regular sarcoidosis checkups during and after pregnancy.

Followup care

Regular followup care is important, even if a person isn’t taking medication for their sarcoidosis. New symptoms can occur at any time, and the condition can get worse slowly, without the person noticing.

Followup exams usually include:

A review of the symptoms

A physical exam

A chest X-ray and CT scan

Breathing tests

An eye exam

Blood tests

An electrocardiogram (EKG).

The frequency of examinations and tests depends on:

How severe the symptoms are

Which organs were affected at diagnosis

What treatment is being used

Any complications that may develop during treatment.

A person will probably need routine followup care for several years. Whether they see their regular doctor or a sarcoidosis specialist for this depends on the symptoms during the first year of followup.

Here are some examples of how followup care can be managed. They are based on either the person’s condition at the time of diagnosis with sarcoidosis or the treatment used.

Followup after initial diagnosis

If at diagnosis, there are no symptoms, a normal breathing test, and an abnormal chest X-ray:

A person should plan on having a followup exam every 6 to 12 months until their condition is stable or improving.

A breathing test may need to be repeated. The need to repeat it depends on the symptoms and ability to be active.

If at the first followup visit, there are no new symptoms and the chest X-ray is normal, a person can go to their regular doctor for future followup care.

If at diagnosis, there are some symptoms, an abnormal chest X-ray, but a person doesn’t need treatment:

They should plan on having a followup exam in 3 to 6 months.

If at the followup exam, a person’s condition has become worse (i.e., there are now more symptoms, an abnormal X-ray, or abnormal lab tests) they may need treatment.

If treatment is started, followup tests may be needed more often.

Followup based on the drug treatment

If treatment is begun with prednisone:

Checks should be carried out for the side effects of high blood pressure, too much weight gain, diabetes, loss of calcium from the bones, and pain in one or both hips.

If treatment is begun with hydroxychloroquine:

An eye exam should be performed every 6 months while taking this drug.

If treatment is begun with methotrexate:

Blood tests should be done every month or every other month to see if there is anemia, low white blood cell or platelet levels, or liver inflammation.

Other followup tests

Depending on how serious the condition is and what organs are affected, certain tests may need to be done regularly.

Eye tests

Everyone who is diagnosed with sarcoidosis, even if they don’t have eye symptoms, should see an ophthalmologist (eye doctor) for eye tests. This is important because there may be eye damage even if there are no symptoms.

These tests may include:

A slit lamp examination. An instrument with a high-intensity light source is used to look at the front of the eyes.

A visual fields examination. The patient will be asked to look at a light through an instrument.

Inspection of the retina and optic nerve.

If eye symptoms develop, a doctor will have you repeat the tests.

A person should also have regular eye exams if they are being treated with:

Chloroquine or hydroxycholoroquine (Plaquenil)

Corticosteroids.

Breathing tests

These tests are used to check the course of sarcoidosis in the lungs. The results are compared over time.

Blood tests

A blood test for calcium should be done. If the calcium level is high, treatment is probably needed. A person also should not take vitamin and mineral supplements containing calcium or vitamin D, and should avoid too much exposure to the sun.

Electrocardiogram

This test is needed to make sure that the heart is still not affected by sarcoidosis. The heart can be affected at any time if the sarcoidosis is active.

Sarcoidosis is known worldwide, and affects people of all races and ages.

It occurs mostly in:

Adults usually between the ages of 20 and 40

In the United States, sarcoidosis affects African Americans somewhat more often and more severely than Caucasians.

African American women, are the most highly affected group

Scandinavian, German, Irish, Puerto Rican, and people of Asian, origin, are also affected.

Studies have shown that sarcoidosis is more likely to affect certain organs in certain populations. For example,

* Sarcoidosis of the heart and eye appears to be more common in Japan.

* Painful skin lumps on the legs occur more often in people from Northern Europe.

People who are more likely to get sarcoidosis include:

* Health care workers(possible environmental exposure)

* Nonsmokers(possible chemical exposure)

* Elementary and secondary school teachers (possible environmental exposure)

* People exposed to agricultural dust, insecticides, pesticides, or mold(possible chemical, environmental exposure)

* Firefighters. (possible chemical, environmental exposure)

Brothers and sisters, parents, and children of people who have sarcoidosis are more likely than others to have sarcoidosis, although this is speculative, even by both medical and homeopathic communities and is supported by no conclusive scientific data. The notion that sarcoidosis is hereditary is not a proven fact, however certain data supports the notion as a possibility.

Many people who have sarcoidosis have no symptoms. Often, the condition is discovered by accident only because a person has a chest X-ray for another reason, such as a pre-employment X-ray.

Some people have very few symptoms, but others have many.

Symptoms usually depend on which organs the disease affects.

Lung symptoms:

Shortness of breath

A dry cough that doesn’t bring up phlegm, or mucus

Wheezing

Pain in the middle of your chest that gets worse when you breathe deeply or cough (rare).

Lymph node symptoms:

Enlarged and sometimes tender lymph nodes – most often those in the neck and chest but sometimes those under the chin, in the arm pits, or in the groin.

Skin symptoms:

Various types of bumps, ulcers, or, rarely, flat areas of discolored skin, that appear mostly near the nose, eyes, back, arms, legs, and scalp. They usually itch but aren’t painful. They usually last a long time.

Painful bumps that usually appear on the ankles and shins and can be warm, tender, red or purple-to-red in color, and slightly raised. This is called erythema- nodosum. A person may have fever and swollen ankles and joint pain along with the bumps. The bumps often are an early sign of sarcoidosis, but they occur in other diseases too. The bumps usually go away in weeks to months, even without treatment.

Disfiguring skin sores that may affect your nose, nasal passages, cheeks, ears, eyelids, and fingers. This is called lupus pernio. The sores tend to be ongoing and can return after treatment is over.

Eye symptoms:

Burning, itching, tearing, pain

Red eye

Sensitivity to light

Dryness

Floaters (i.e., seeing black spots)

Blurred vision

Reduced color vision

Reduced visual clearness

Blindness (in rare cases).

Heart symptoms:

Shortness of breath

Swelling in the legs

Wheezing

Coughing

Irregular heartbeat, including palpitations (a fluttering feeling of rapid heartbeats) and skipped beats

Sudden loss of consciousness

Sudden death.

Joint and muscle symptoms:

Joint stiffness or swelling – usually in the ankles, feet, and hands.

Joint pain.

Muscle aches (myalgias).

Muscle pain, a mass in a muscle, or muscle weakness.

Painful arthritis in the ankles that results from erythema nodosum. It may need treatment but usually clears up in several weeks.

Painless arthritis that can last for months or even years. It should be treated.

Bone symptoms:

Painless holes in the bones.

Painless swelling, most often in the fingers.

Anemia that results from granulomas affecting the bone marrow. This usually should be treated.

Liver symptoms:

Fever

Fatigue

Itching

Pain in the upper right part of the abdomen, under the right ribs

Enlarged liver.

Parotid and other salivary gland symptoms:

Swelling, which makes the cheeks look puffy

Excessive dryness in the mouth and throat.

Blood, urinary tract, and kidney symptoms:

Increased calcium in the blood or urine, which can lead to painful kidney stones

Confusion

Increased urination.

Nervous system symptoms:

Headaches.

Vision problems.

Weakness or numbness of an arm or leg.

Coma (rare).

Drooping of one side of the face that results from sarcoidosis affecting a facial nerve. This can be confused with Bell’s palsy, a disorder that may be caused by a virus.

Paralysis of your arms or legs that results from sarcoidosis affecting the spinal cord.

Weakness, pain, or a “stinging needles” sensation in areas where many nerves are affected by sarcoidosis.

Pituitary gland symptoms (rare):

Headaches

Vision problems

Weakness or numbness of an arm or leg

Coma (rare).

Other symptoms:

Nasal obstruction or frequent bouts of sinusitis.

Enlarged spleen, which leads to a decrease in platelets in the blood and pain in the upper left abdomen. Platelets are needed to help the blood clot.

Sarcoidosis may also cause more general symptoms, including:

Uneasiness, feeling sick (malaise), an overall feeling of ill health

Tiredness, fatigue, weakness

Loss of appetite or weight

Fever

Night sweats

Sleep problems

These general symptoms are often caused by other conditions. If a person has these general symptoms but doesn’t have symptoms from affected organs, he or she probably does not have sarcoidosis.

Sarcoidosis is diagnosed by taking a detailed medical history and conducting a physical exam and several diagnostic tests. The purpose is to:

Identify the presence of granulomas in any of the organs.

Rule out other causes of the symptoms.

Determine the amount of damage to any of the affected organs.

Determine whether a person needs treatment.

Medical history

A doctor will ask for a detailed medical history. He or she will want to know about any family history of sarcoidosis and what jobs a person has had that may have increased their chances of getting sarcoidosis.

The doctor may also ask whether a person has ever been exposed to inhaled beryllium metal, which is used in aircraft and weapons manufacture, or organic dust from birds or hay. These things can produce granulomas in the lungs that look like the granulomas that are caused by sarcoidosis but are actually signs of other conditions.

Physical exam

A doctor will look for symptoms of sarcoidosis, such as red bumps on the skin; swollen lymph nodes; an enlarged liver, spleen, or salivary gland(s); or redness in the eyes. He or she will also listen for abnormal lung sounds or heart rhythm, and will check for other likely causes of the symptoms.

Diagnostic tests

There is no one specific test for diagnosing sarcoidosis. It is harder to diagnose sarcoidosis in some organs (e.g., heart, nervous system) than in others. A doctor will probably conduct a variety of tests and procedures to help in the diagnosis.

These include:

Chest X-ray. A chest X-ray takes a picture of the heart and lungs. It may show granulomas or enlarged lymph nodes in the chest. About 95 out of every 100 people who have sarcoidosis have an abnormal chest X-ray.

Doctors usually use a staging system for chest X-rays taken to detect sarcoidosis:

Stage 0: Normal chest X-ray

Stage 1: Chest X-ray showing enlarged lymph nodes but otherwise clear lungs

Stage 2: Chest X-ray showing enlarged lymph nodes and shadows in the lungs

Stage 3: Chest X-ray showing shadows in the lungs, but the lymph nodes are not enlarged

Stage 4: Chest X-ray showing scars in the lung tissue.

In general, the higher the stage of the X-ray, the worse the symptoms and lung function are. But there are a lot of differences among people. If the X-ray results show Stages 0, 1, 2, or 3, the patient may not have symptoms or need treatment, and may get better and have normal chest X-rays again over time.

Blood tests

These tests can show the number and type of cells in the blood. They also will show whether there are increases in the calcium levels or changes in the liver, kidney, and bone marrow that can occur with sarcoidosis.

Lung function tests

One test uses a spirometer, a device that measures how much and how fast a person can blow air out of their lungs after taking a deep breath. If there is a lot of inflammation and/or scarring in the lungs, a person will not be able to move normal amounts of air in and out.

Another test measures how much air the lungs can hold. Sarcoidosis can cause the lungs to shrink, and they will not be able to hold as much air as healthy lungs.

Electrocardiogram (EKG)

This test will help show if the heart is affected by sarcoidosis.

Pulse oximetry

A small clip attached to the finger tip can show how well the heart and lungs are moving oxygen into the blood.

Arterial blood gas test.

This test is more accurate than pulse oximetry for checking the level of oxygen in the bloodstream. Blood is taken from an artery (usually in the wrist). It is then analyzed for its oxygen and carbon dioxide levels.

Fiberoptic bronchoscopy

In this procedure, a doctor inserts a long, narrow, flexible tube with a light on the end through the nose or mouth into the lungs to look at your airways. This tube is called a bronchoscope. A person most likely would have this procedure as an outpatient in a hospital under local anesthesia.

Bronchoalveolar lavage (BAL)

During bronchoscopy, a doctor may inject a small amount of salt water (saline) through the bronchoscope into the lungs. This fluid washes the lungs and helps bring up cells and other material from the air sacs deep in the lungs where the inflammation usually starts to develop. The cells and fluid are then examined for signs of inflammation.

Biopsy

A doctor may take a small sample of tissue from one of the affected organs. For example, when breathing tests or chest X-rays show signs of sarcoidosis in the lungs, a doctor may do a fiberoptic bronchoscopy biopsy. This will help confirm the diagnosis. Tiny forceps as inserted through the bronchoscope to collect tissue that will be examined. Because the granulomas may be spread out in the lungs, the bronchoscope may miss some of them.

Biopsies of the skin and liver are sometimes done to detect granulomas in these organs.

A person may have sarcoidosis in other organs as well and multiple biopsies may be necessary. However, every organ involved does not need to be biopsied for a diagnosis to be made.

Computed tomography (CT) scan

This test provides a computer-generated image of the organs that has more detail than a regular chest X-ray. It can provide more information about how sarcoidosis has affected an organ.

A doctor may do a CT scan to:

Obtain more information about how much of the lung is affected by sarcoidosis.

Detect sarcoidosis in the liver. A CT scan of the abdomen will show if the liver is enlarged and if there is a pattern suggesting granulomas.

Magnetic resonance (MR) scan

This test is also called nuclear magnetic resonance (NMR) scanning or magnetic resonance imaging (MRI). This scan uses powerful magnets and radio waves to make images of some of the organs that a doctor doesn’t want to risk doing a biopsy on. For example, an MR scan can be used to diagnose sarcoidosis in the brain, spinal cord, nerves, or heart.

Thallium and gallium scans

These scans are often done to see if sarcoidosis is affecting the heart. Thallium and gallium are radioactive elements. A doctor injects a small amount of one of them into the patient’s vein in the arm. The elements collect at places in the body where there is inflammation. After a while, the body is scanned for radioactivity. Increased radioactivity at any place may be a sign of inflammation.

This test gives information on the tissue in the body that has been affected by sarcoidosis and the amount of damage to it. But since this test shows all inflammation in the body, even inflammation caused by conditions other than sarcoidosis, it does not give a definite diagnosis of sarcoidosis.

Positron emission tomography (PET) scan

This test also uses radioactive injections. It may be more sensitive than gallium in detecting areas of inflammation. Some doctors are using it instead of gallium scans.

Your doctor may not need to find every one of the organs affected by sarcoidosis, only those that cause symptoms. Often the organs affected by the condition continue to function well and don’t need to be treated.

How is sarcoidosis treated?

The goals of treatment are to:

Improve how the organs affected by sarcoidosis work

Relieve symptoms

Shrink the granulomas.

Treatment may shrink the granulomas and even cause them to disappear, but this may take many months. If scars have formed, treatment may not help, and a person may have ongoing symptoms.

A person’s treatment depends on:

What symptoms he or she has

How severe the symptoms are

Whether any of the vital organs (e.g., the lungs, eyes, heart, or brain) are affected

How the organ is affected.

Some organs must be treated, regardless of the symptoms. Others may not need to be treated. Usually, if a person doesn’t have symptoms, he or she doesn’t need treatment, and they probably will recover in time.

Drugs

The main treatment for sarcoidosis is prednisone. Prednisone is a corticosteroid, or anti-inflammatory drug. Sometimes it is used with other drugs. Sometimes other corticosteroids are used.

Prednisone almost always relieves symptoms of inflammation. If a symptom doesn’t improve with prednisone treatment within a couple of months, consult your physician.

Prednisone is usually given for many months, sometimes for a year or more.

Low doses of prednisone can often relieve symptoms without causing major side effects.

When used at high doses, prednisone can cause serious side effects.

Side effects can include:

Weight gain.

Diabetes.

High blood pressure.

Mood swings (depression).

Difficulty sleeping at night.

Heartburn.

Acne.

Thinning of the skin and bones (called osteoporosis).

Cataracts.

Glaucoma.

Adrenal gland insufficiency, which occurs when the adrenal glands don’t make enough of certain hormones. This requires treatment by an endocrinologist, a doctor who specializes in the diagnosis and treatment of the endocrine glands. The endocrine glands include the adrenal and pituitary glands.

Aseptic or avascular necrosis of the hip, the development of cysts and hardened and dead tissue in the hip. A doctor can usually help a person manage these side effects.

When it is time to stop taking prednisone, a person should cut back slowly, with a doctor’s help. This will help prevent flare-ups of sarcoidosis and allow the body to adjust to life without the drug.

A person may also want to see an endocrinologist to make sure that the endocrine glands are making enough hormones. The endocrinologist may prescribe certain hormones for the patient to take until their endocrine glands are working well again.

Other drugs used to treat sarcoidosis

Other drugs are sometimes used to treat sarcoidosis. A doctor may prescribe one of them if:

A person’s condition gets worse while they are taking prednisone

A person can’t stand the side effects of prednisone.

Most of these other drugs are immune system suppressants. This means that they prevent the immune system from fighting things like bacteria and viruses. As a result, a person may have a greater chance of getting infections.

Most of these drugs also can cause serious side effects. Some also could increase the chances of getting cancer, especially if they are taken at high doses.

A person and his or her doctor must weigh living with the symptoms of sarcoidosis against the side effects of the drugs.

Some drugs work better than others for different people.

A person may be given more than one drug.

Some drugs used to treat sarcoidosis are taken by mouth. Others are applied locally to an affected area.

Local therapy is the safest way to treat sarcoidosis. The drug is applied directly to the affected area. As a result, only small amounts of the drug reach other parts of the body.

Drugs used for local therapy include:

Eye drops

Inhaled drugs for the lungs

Skin creams.

Drugs can be used locally only if the affected area is easily reached. For instance, inhaled steroids can ease coughing and wheezing in the upper airways, but they don’t seem to relieve these symptoms when the affected lung tissue is deep within the chest.

A person should talk with their doctor about these treatments and the side effects that may occur.

The other drugs used to treat sarcoidosis include:

Hydroxychloroquine (Plaquenil). This drug can usually help people who have sarcoidosis in the skin or a high level of calcium in their blood. This drug can irritate the stomach. It also can cause eye problems. Before starting on this drug, a person should see an ophthalmologist, or eye doctor, for some baseline tests. Once a person starts taking it, they should have their eyes examined every 6 months.

Methotrexate. This drug is taken once a week by mouth or injection and usually takes up to 6 months to relieve symptoms. This drug may cause side effects, especially if taken in high doses. These include:

Nausea.

Mouth sores.

A decrease in infection-fighting white blood cells. A person then has a greater chance of getting an infection. If this drug is taken, a person should have regular blood tests to check the levels of their white blood cells.

An allergic reaction in the lungs that goes away when the person stops taking the drug. This is extremely rare.

Liver damage. This is the most serious side effect. If methotrexate is taken, a person should be monitored regularly by a physician.

If a person is pregnant, they should not take this drug. Taking folic acid can help reduce the chances of having bad side effects from methotrexate.

Azathioprine (Imuran). This drug may work in about half of the people who have sarcoidosis. It is usually taken for at least 6 months. Side effects include:

Nausea

Reduced white blood cell levels, which increases a person’s chances of getting an infection.

This drug has caused cancer in some people, especially when they have taken it at high doses. It should not be taken by patients who are pregnant.

Cyclophosphamide (Cytoxan). This is a very toxic drug. It is rarely used to treat sarcoidosis. It is given only to people who have serious forms of sarcoidosis, such as sarcoidosis in their central nervous system (neurosarcoidosis). This drug is more likely to cause nausea and reduce the white blood cell levels than either methotrexate or azathioprine. A doctor should check the white blood cell levels often while a person is taking this drug to make sure they have a high enough level to fight infection. Cyclophosphamide can also irritate the bladder. Some people who have taken it for more than 2 years have developed bladder cancer. It should not be taken during pregnancy. Cyclophosphamide can be given intravenously (through one of the veins), which lessens some of its side effects, but this doesn’t reduce the risk of cancer.

Treatments for specific types of sarcoidosis

Eyes. Sarcoidosis in the eyes almost always responds well to treatment. Often, the only treatment needed is eye drops containing corticosteroids. A person should have yearly eye exams, even if they think their eyes are doing well.

Spleen. Sarcoidosis can cause the spleen to enlarge. This can lead to a decrease in the red or white blood cells or platelets and increase the chances of infection and blood clotting disorders. Treatment is usually given to increase the number of blood cells and ease pain. In rare cases, the spleen may need to be removed.

Liver. Sarcoidosis rarely causes permanent liver damage. As a result, the liver usually isn’t treated unless it’s causing major symptoms (e.g., fever). Drug treatment can usually reduce granulomas in the liver. Liver transplantation has been successful in those rare cases in which the condition has become worse. Followup care includes regular blood tests to find out how well your liver is working. A person should check with their doctor to find out how often they need these tests.

Nervous system. Sarcoidosis in the nervous system (neurosarcoidosis) usually needs treatment. Nerve tissue heals slowly, so treatment often takes a long time. A person may need to take several drugs at high doses.

Erythema nodosum. These painful bumps on the shins often go away in weeks to months without treatment. A doctor probably will not give medication unless a person is very uncomfortable. Aspirin or ibuprofen, an anti-inflammatory drug that can be bought without a prescription, will usually help.

Heart. Sarcoidosis in the heart is usually treated with steroids. Heart drugs may also be given to improve the heart’s pumping ability or to correct a disturbed heart rhythm. If a person has a severe heart rhythm disturbance, their doctor may prescribe one of these devices:

A cardiac pacemaker, a small battery-operated device, often put under the skin, that regulates the heartbeat

A defibrillator, an implanted device that shocks the heart into a normal heartbeat or, if it has stopped, into beating.

If the heart is severely affected and doesn’t respond to treatment, a transplant may be done. But this is rarely needed.

Lupus Pernio. This rash on the face, especially the cheeks and nose, can be distressing because it’s in a very visible area. It often occurs with loss of the sense of smell, nasal stuffiness, and sinus infections. Options for treatment include:

Local treatment with skin creams

Oral drugs (plaquenil or prednisone, for example)

Local injections of steroid preparations.

Lupus pernio is often treated by dermatologists, doctors who specialize in skin diseases, working with a sarcoidosis specialist. Because sarcoidosis varies so much among different people, a doctor may find it hard to tell whether the treatment is helping.

Other drugs being studied for possible use in treating sarcoidosis

Scientists also are studying drugs that are used for other conditions to see if they can help people who have sarcoidosis. These drugs include:

Etanercept (Enbrel). This drug is an immune system suppressant. It’s injected under the skin to reduce symptoms of rheumatoid arthritis. It may also be used to treat psoriasis or ankylosing spondylitis, a type of arthritis that affects the joints in the spine. Early studies suggest that it will not be useful in treating sarcoidosis, but research is ongoing.

Infliximab (Remicaide). This drug is an immune system suppressant. It’s injected into a vein in the arm. It’s used to treat Crohn’s Disease, rheumatoid arthritis, and ankylosing spondylitis. Some studies have shown it to help sarcoidosis patients who also have lupus pernio, eye disease, or neurosarcoidosis. This drug has serious side effects but may improve lung function in some people who aren’t helped by corticosteroids. More research is needed.

Pentoxifylline. This drug is an immune system suppressant. Stomach and gastrointestinal side effects are common. Early studies show that it has helped some people who have sarcoidosis in their lungs reduce their doses of prednisone while taking it. More research is needed.

Tetracycline. Tetracycline antibiotics are used to treat Lyme Disease, some types of pneumonia, and acne. A few small studies suggest that they may help in treating sarcoidosis in the skin. Research is ongoing.

Thalidomide. This immune system suppressant can cause bad side effects. It is effective against other conditions that involve granulomas of the skin (e.g., leprosy, tuberculosis). Scientists are studying this drug to see if it can be used to treat sarcoidosis in the skin.

What does the future hold?

Scientists worldwide are trying to learn more about sarcoidosis and how to improve its diagnosis and treatment. Some recent studies have led to possible new treatments, which, in turn, are being studied. Current research includes studies of:

The agent or agents that cause sarcoidosis

Why sarcoidosis seems to act differently in people of different races

Why sarcoidosis appears in some families

How genes, passed from one generation to another, may make some people more likely than others to develop sarcoidosis

How cells act and communicate with each other to cause sarcoidosis symptoms.

Sarcoidosis is thought to be chronic in up to 30% of patients, and 10 to 20% experience permanent sequelae. The disease is fatal in 1 to 5% of patients. Pulmonary fibrosis with respiratory failure is the most common cause of death worldwide, followed by pulmonary hemorrhage from aspergilloma. In Japan, however, infiltrative cardiomyopathy causing heart failure and arrhythmias is the most common cause of death.

Prognosis is worse for patients with extrapulmonary sarcoidosis and for blacks. Recovery occurs in 89% of whites and 76% of blacks with no extrathoracic disease and in 70% of whites and 46% of blacks with extrathoracic disease. The presence of erythema nodosum and acute arthritis are good prognostic signs. Uveitis, lupus pernio, chronic hypercalcemia, neurosarcoidosis, nephrocalcinosis, myocardial disease, and extensive pulmonary involvement are all poor prognostic signs. However, little difference is demonstrable in long-term outcome between treated and untreated patients, and relapse is common when treatment ends.

Treatment

Because sarcoidosis often spontaneously resolves, asymptomatic patients and those with mild symptoms do not require treatment, although they should be monitored for signs of deterioration. These patients can be followed with serial x-rays, pulmonary function tests (including diffusing capacity), and markers of extrathoracic involvement (eg, routine renal and liver function testing). Patients who require treatment regardless of stage include those with worsening symptoms; limitation of activity; markedly abnormal or deteriorating lung function; worrisome x-ray changes (cavitation, fibrosis, conglomerate masses, signs of pulmonary hypertension); heart, nervous system, or eye involvement; renal or hepatic insufficiency or failure; or disfiguring skin and joint disease.

Treatment is with corticosteroids. A standard protocol is prednisone Some Trade Names
DELTASONE
Click for Drug Monograph
0.3 to 1mg/kg po once/day depending on symptoms and severity of findings. Alternate-day regimens are also used (eg, prednisone Some Trade Names
DELTASONE
Click for Drug Monograph
40 to 60 mg po once every other day). It is rare to exceed 40 mg/day; however, higher doses may be needed to reduce complications in patients with ocular, myocardial, or neurologic disease. Response usually occurs within 2 to 4 wk, so symptoms and results of chest x-ray and pulmonary function tests may be reassessed between 4 and 12 wk. Chronic, insidious cases may respond more slowly. The drug is tapered to a maintenance dose (eg, prednisone Some Trade Names
DELTASONE
Click for Drug Monograph
≤ 10 mg every other day if possible) after evidence of response and is continued for a minimum of 12 mo if improvement occurs. The optimal duration of treatment is unknown. Premature taper can result in relapse. The drug is slowly stopped if response is absent or equivocal. Corticosteroids can ultimately be stopped in most patients, but because relapse occurs up to 50% of the time, monitoring should be repeated, usually every 3 to 6 mo. Corticosteroid treatment should be resumed for recurrence of symptoms and signs, including dyspnea, arthralgia, fever, hepatic insufficiency, cardiac arrhythmia, CNS involvement, hypercalcemia, ocular disease uncontrolled by local drugs, and disfiguring skin lesions.

Data on use of inhaled corticosteroids for pulmonary sarcoidosis are not definitive, but some evidence suggests that this route of administration can relieve cough in patients with endobronchial involvement. Topical corticosteroids may be useful in some cases of dermatologic and ocular disease.

About 10% of patients requiring therapy are unresponsive to tolerable doses of a corticosteroid and should be given a 6-mo trial of methotrexate Some Trade Names
RHEUMATREX
Click for Drug Monograph
starting at 2.5 mg po once/wk and increasing in increments of 2.5 mg/wk to a total of 10 to 15 mg/wk as tolerated to keep WBC > 3000/μL. Initially, methotrexate Some Trade Names
RHEUMATREX
Click for Drug Monograph
and corticosteroids are both given; over 8 wk, the corticosteroid dose can be tapered and, in many cases, stopped. The maximal response to methotrexate Some Trade Names
RHEUMATREX
Click for Drug Monograph
, however, may take 6 to 12 mo. In such cases, prednisone Some Trade Names
DELTASONE
Click for Drug Monograph
must be tapered more slowly. Serial blood counts and liver enzyme tests should be performed every 1 to 2 wk initially and then every 4 to 6 wk once a stable dose is achieved. Folic acid (1 mg po once/day) is recommended for patients treated with methotrexate Some Trade Names
RHEUMATREX
Click for Drug Monograph
.

Other drugs reported to be effective in small numbers of patients who are corticosteroid-resistant or who experience complicating adverse effects include azathioprine Some Trade Names
IMURAN
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, cyclophosphamide Some Trade Names
CYTOXAN
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, chlorambucil Some Trade Names
LEUKERAN
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, chloroquine Some Trade Names
ARALEN
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or hydroxychloroquine Some Trade Names
PLAQUENIL
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, thalidomide Some Trade Names
THALOMID
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, pentoxifylline Some Trade Names
TRENTAL
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, and infliximab Some Trade Names
REMICADE
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.

Hydroxychloroquine Some Trade Names
PLAQUENIL
Click for Drug Monograph
200 mg po bid to tid can be as effective as corticosteroids for treatment of disfiguring skin sarcoidosis and in treatment of hypercalciuria. Although immunosuppressants are often more effective in refractory cases, relapse is common after cessation.

No available drugs have consistently prevented pulmonary fibrosis.

Lung transplantation is an option for patients with end-stage pulmonary involvement, although disease may recur in the transplanted organ.

Median estimated glomerular filtration rate (eGFR) was 20.5 mL/min per 1.73 m(2) (range, 4-93 mL/min per 1.73 m(2)). Moderate proteinuria was found in 31 (66%) patients (median, 0.7 g/24 h; range, 0-2.7 g/24 h), microscopic hematuria in 11 (21.7%) patients, aseptic leukocyturia in 13 (28.7%) patients. Fifteen of 47 (32%) patients had hypercalcemia (>2.75 mmol/L). Eleven of the 22 (50%) patients diagnosed between June and September had hypercalcemia compared with only 4 of the 25 (16%) cases diagnosed during the other months (p < 0.001).

Thirty-seven patients presented with noncaseating granulomatous interstitial nephritis (GIN), and 10 with interstitial nephritis without granulomas. Apart from hypercalcemia, the clinical phenotype was also remarkable for the high frequency of fever at presentation. All patients initially received prednisone (median duration, 18 mo), 10 received intravenous pulse methylprednisolone. eGFR increased from 20 +/- 19 to 44 +/- 24.7 mL/min per 1.73 m(2) at 1 month (p < 0.001, n = 38), to 47 +/- 19.9 mL/min per 1.73 m(2) at 1 year (p < 0.001, n = 46), to 49.13 +/- 25 mL/min per 1.73 m(2) at last follow-up (p < 0.001, n = 47). A complete response to therapy at 1 year and at last follow-up was strongly correlated with complete response at 1 month (p < 0.01).

Renal function improvement was inversely related to initial histologic fibrosis score. A complete response to therapy at 1 year was strongly correlated with hypercalcemia at presentation (p = 0.003). Relapses were purely renal (n = 3) and purely extrarenal (n = 10) or both (n = 4), often a long time after presentation, with in some cases severe cardiac or central nervous system involvement.

We conclude that hypercalcemia and fever at presentation are often associated with RS; RS is most often and permanently responsive to corticosteroid treatment, but some degree of persistent renal failure is highly frequent and its degree of severity in the long run is well predicted from both histologic fibrotic renal score and response obtained at 1 month.

Potential Benefits:

* Free medical care & laboratory studies.
* Receive treatments before they are offered to others.
* You may or may not receive direct benefit for yourself and your condition as a result of participating in research, however, you will know that the knowledge learned by researchers may help others.

Know the Risks:

* Some risks are involved in clinical research, just as in routine medical care and activities of daily living.  These may include unpleasant, serious or even life-threatening side effects to experimental treatment or the experimental treatment may not be effective.
* Risks associated with any research study should be described in detail in a written consent document and by a member of the research team.
* Carefully weigh these risks against possible benefits.

Before Participating in a Study:
You should know as much as possible about the study and feel comfortable asking the members of the health care team questions about it, the care expected while in a trial and the cost of the trial.

* The purpose of the study.
* Who is conducting the study.
* How long the study will last and how participation might affect your daily life.
* The kinds of tests and experimental treatments involved.
* How the possible risks, side effects and benefits compare with your current treatment.
* Who will pay for the experimental treatment and other related expenses.
* What type of long-term follow up care is part of this study.

Understanding Clinical Trial Keywords:

* placebo:  an inactive pill, liquid or powder that has no treatment value. In clinical trials, experimental treatments are often compared with placebos to assess the effectiveness.
* double-blind study:  a clinical trial design in which neither the participants nor the study staff knows who receives the experimental drug or the placebo, to produce objective results.
* randomized trial:  participants are randomly (by chance) assigned to one of two or more treatment arms of a clinical trial, some may receive a placebo.
* controlled trial:  a control is a standard against which experimental observations may be evaluated. In clinical trials, one group of participants is given an experimental drug, while another group (the control group) is given either a standard treatment or a placebo.
* observational study:  participants are observed or certain outcomes are measured but no treatment is given.

For those of us coping on a daily basis with the symptoms of the disease and the side effects of medications, research efforts seem not to be enough. Sometimes it is useful to understand where we have been to better appreciate where we are. This brief review of the history of sarcoidosis research illustrates that wide-scale research efforts are fairly new.

While Jonathan Hutchinson, an English physician, is credited with documenting the condition that later became known as sarcoidosis, we can imagine that the mystery has been with us since the beginning of time. WASOG describes sarcoidosis are a “systemic granulomatous disease that primarily affects the lung and lymphatic systems.” We can thank scientists for getting us from Hutchinson’s observations in 1877 of “multiple, raised, purplish cutaneous patches on the hands and feet…” thought initially to be attributed to gout, and later to represent ” a form of skin disease,” to WASOG’s definition 122 years later.

Caesar Boeck, the nephew of a Norwegian dermatologist, Carl Boeck, who first illustrated sarcoid lesions in sailors, first used the term ’sarkoid” in an 1899 report describing skin lesions that resembled benign sarcoma. But, it was his subsequent research and publications that gradually revealed the systemic nature of the disorder. Boeck, along with other dermatologists and scientists from other specialties and parts of the world, including France, Germany, Denmark, and Sweden, made their discoveries known in the late 19th and early 20th Centuries.

The mid-20th Century ushered in the age of steroid treatment for sarcoidosis, followed by 30 years of research on its effectiveness in treating pulmonary sarcoidosis. This was made possible by the development of the radiographic staging system, which helped to account for the spontaneous remissions often observed in early stages of some of the syndromes. The invention of fiberoptic bronchoscopy in the 1970’s allowed for the diagnosis of sarcoidosis to be confirmed.

Carl Boeck attempted the first international collaboration in the late 1800’s when he sent his drawings of the lesions of Norwegian sailors to Hutchinson, who failed to publish them. It was not until 1958 that the first International Meeting was held, with very poor attendance. Five years later, an International Committee on Sarcoidosis was formed, resulting in increased collaboration, research, and publication. A Medline search reveals 6500 publications related to sarcoidosis since 1965. The International Committee began publication of the Sarcoidosis Journal in 1984, furthering the dissemination of ideas and research related to sarcoidosis. WASOG was formed in 1987 and published the Statement on Sarcoidosis in 1999. It’s 7th Congress is scheduled for June 2002 in Sweden.

In 1984 when my diagnosis of sarcoidosis was confirmed with bronchoscopy, little information was available to consumers. I was able to locate brief descriptions in medical texts. Fortunately, all that has changed. In the last decade, numerous support, information, education, and research organizations have been formed for consumers and researchers alike, many of international scope. Information on the latest medications and treatments are readily available from consumer and health organizations and publications, pharmaceutical companies, medical publications, and the Internet.

The National Institutes of Health (NIH) is the premier national, government agency coordinating sarcoidosis research. The National Lung, Heart, and Blood Institute (NLHBI) of NIH is currently collaborating with ten of the best medical institutions in the country on sarcoidosis research. They are conducting clinical trials in four areas: 1) the relationship of genetics and the environment for African Americans, 2) treatment of pulmonary sarcoidosis with pentotoxifylline, 3) the genetics involved in the development of lung disease (sarcoidosis is one of five conditions being studied), and 4) evaluation and treatment of patients with inflammatory eye disease.

NIH’s Clinical Center is a good place to start to learn about research and clinical trials. The site explains standards for studies, roles of participants and researchers, and other useful information, and, includes a link to the clinical trials website. This website (clinicaltrials.gov) explains all the clinical trials in detail and offers links to WebPages and protocols. You will find specifics on how to participate, such as eligibility, need for referral letter from physician, as well as phase and type of study. The four studies are in different phases and continue to recruit for participants. You can cross-reference information on studies by visiting the websites of participating medical institutions and the NIH.

Both private and non-government sponsored public institutions also conduct research. Pharmaceutical companies conduct an endless array of research on drugs and have recently begun to market drugs directly to the public rather than via the physician. The Sarcoidosis Journal and other professional publications report on research being conducted at the international level.

There are several ways to participate in research as a consumer/patient. A clinical trial is one way. Several organizations and foundations have been established to support research efforts and disseminate research results to the public. This is not an endorsement of any particular organization. You will need to do your own research before making financial or other contributions. Some pharmaceutical companies solicit survey participants.

Finally, some of the best research results from what we do as consumers of information, medications, and other sarcoidosis treatments. Following a treatment regimen, monitoring the effects of the regimen, and reporting your responses to your physician is one way for scientists to collect data about the effectiveness of the treatments provided. And, for you to become an educated consumer with more control over your health.

Article Source: Suite101

In general, sarcoidosis appears briefly and heals naturally in 60%-70% of the cases, often without the patient knowing or doing anything about it. From 20%-30% of sarcoidosis patients are left with some permanent lung damage. In 10%-15% of the patients, sarcoidosis can become chronic.

When either the granulomas or fibrosis seriously affect the function of a vital organ—the lungs, heart, nervous system, liver, or kidneys, for example—sarcoidosis can be fatal. This occurs 5%-10% of the time. Some people are more at risk than others; no one knows why.

No one can predict how sarcoidosis will progress in an individual patient. The patient’s symptoms, race, and the doctor’s findings can give some clues. For example, a sudden onset of general symptoms such as weight loss of feeling poorly are usually taken to mean that the course of sarcoidosis will be relatively short and mild. Dyspnea and possibly skin sarcoidosis often indicate that the sarcoidosis will be more chronic and severe.

White patients are more likely to develop the milder form of the disease. Black people tend to develop the more chronic and severe form.

Sarcoidosis rarely develops before the age of 10 or after the age of 60. However, the illness—with or without symptoms—has been reported in younger as well as in older people. When symptoms do appear in these age groups, the symptoms are those that are more general in nature, for example, tiredness, sluggishness, coughing, and a general feeling of ill health.

Article Source: MedicineNet.com

Reiter’s Syndrome

This disease occurs almost entirely in men. The cutaneous lesions of Reiter’s syndrome are essentially identical with those that occur in psoriasis. Pustular plaques on the palms and soles, together with small red scaling plaques on the glans penis, are characteristically present. Ocular inflammatory disease, sacroiliitis, and arthritis of the spine and larger peripheral joints complete the syndrome. Most patients with Reiter’s disease have an HLA haplotype that includes B27. Episodes of disease activity may be triggered by a variety of infectious etiologies.

Behcet’s Syndrome

Behcet’s syndrome is characterized by the presence of aphthous-like ulcers of the mouth and genitalia. These changes are usually accompanied by arthritis, uveitis, and a variety of neurologic changes. Thrombophlebitis, gastrointestinal involvement, erythema nodosum, and a peculiar pustular response to skin trauma (pathergy) are occasionally present.

Sarcoidosis

Sarcoidosis is characterized by the presence of noninfectious, noncaseating granulomas of many organs, including the skin. The most distinctive skin lesions consist of clusters of small, nonscaling, violaceous to skin-colored, dome-shaped papules. These papules are most often found on the face and neck. Coalescence of the papules to form annular plaques is frequently noted. Larger nodules and plaques are sometimes found on the trunk and extremities. Involvement of cutaneous scar tissue with sarcoidal granulomas is highly characteristic. Erythema nodosum of the lower legs frequently accompanies active lesions of sarcoid occurring in the lungs. Biopsy of the skin lesions (except those of erythema nodosum) reveals the characteristic granulomas and allows confirmation of diagnosis to be made on clinical examination. Recently, a mycobacterial origin for sarcoidosis has been postulated.

Acquired Immuno dificiency Syndrome (AIDS)

Several types of skin lesions are found in AIDS and, in fact, are often of help in making this diagnosis. The lesions of Kaposi sarcoma occur in about 25% of the patients. They consist of red to violaceous, smooth-surfaced papules and nodules. Lesions may be found anywhere on the body, but the most frequent distribution includes the face, arms, trunk, and mucous membranes. The lesions of AIDS-related Kaposi’s sarcoma differ from those of classical Kaposi’s sarcoma in that the former are smaller in size, are violaceous rather than blue-black, and are distributed on the upper half of the body instead of the legs. Less characteristic findings in AIDS include oral candidiasis, oral hairy leukoplakia seborrheic dermatitis of the face, minute pruritic erythematous papules and pustules of the trunk, and various other types of cutaneous opportunistic infections.

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By Robin Kumar Lim