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Monday, March 31, 2008

SARCOIDOSIS

Sarcoidosis involves inflammation that produces tiny lumps of cells in various organs in your body. The lumps are called granulomas because they look like grains of sugar or sand. They are very small and can be seen only with a microscope.

These tiny granulomas can grow and clump together, making many large and small groups of lumps. If many granulomas form in an organ, they can affect how the organ works. This can cause symptoms of sarcoidosis.

Sarcoidosis can occur in almost any part of your body, although it usually affects some organs more than others. It usually starts in one of two places:

  • Lungs
  • Lymph nodes, especially the lymph nodes in your chest cavity.

Sarcoidosis also often affects your:

  • Skin
  • Eyes
  • Liver.

Less often, sarcoidosis affects your:

  • Spleen
  • Brain
  • Nerves
  • Heart
  • Tear glands
  • Salivary glands
  • Bones and joints.

Rarely, sarcoidosis affects other organs, including your:

  • Thyroid gland
  • Breasts
  • Kidneys
  • Reproductive organs.

Sarcoidosis almost always occurs in more than one organ at a time.

Sarcoidosis has an active and a nonactive phase:

  • In the active phase, the granulomas form and grow. In this phase, symptoms can develop, and scar tissue can form in the organs where the granulomas occur.
  • In the nonactive phase, the inflammation goes down, and the granulomas stay the same size or shrink. But the scars may remain and cause symptoms.

The course of the disease varies greatly among people.

  • In many people, sarcoidosis is mild. The inflammation that causes the granulomas may get better on its own. The granulomas may stop growing or shrink. Symptoms may go away within a few years.
  • In some people, the inflammation remains but doesn't get worse. You may also have symptoms or flare-ups and need treatment every now and then.
  • In other people, sarcoidosis slowly gets worse over the years and can cause permanent organ damage. Although treatment can help, sarcoidosis may leave scar tissue in the lungs, skin, eyes, or other organs. The scar tissue can affect how the organs work. Treatment usually does not affect scar tissue.

Changes in sarcoidosis usually occur slowly (e.g., over months). Sarcoidosis does not usually cause sudden illness. However, some symptoms may occur suddenly. They include:

  • Disturbed heart rhythms
  • Arthritis in the ankles
  • Eye symptoms.

In some serious cases in which vital organs are affected, sarcoidosis can result in death.

Sarcoidosis is not a form of cancer.

There is no known way to prevent sarcoidosis.

What Is Sarcoidosis?

Sarcoidosis involves inflammation that produces tiny lumps of cells in various organs in your body. The lumps are called granulomas because they look like grains of sugar or sand. They are very small and can be seen only with a microscope.

These tiny granulomas can grow and clump together, making many large and small groups of lumps. If many granulomas form in an organ, they can affect how the organ works. This can cause symptoms of sarcoidosis.

Sarcoidosis can occur in almost any part of your body, although it usually affects some organs more than others. It usually starts in one of two places:

  • Lungs
  • Lymph nodes, especially the lymph nodes in your chest cavity.

Sarcoidosis also often affects your:

  • Skin
  • Eyes
  • Liver.

Less often, sarcoidosis affects your:

  • Spleen
  • Brain
  • Nerves
  • Heart
  • Tear glands
  • Salivary glands
  • Bones and joints.

Rarely, sarcoidosis affects other organs, including your:

  • Thyroid gland
  • Breasts
  • Kidneys
  • Reproductive organs.

Sarcoidosis almost always occurs in more than one organ at a time.

Sarcoidosis has an active and a nonactive phase:

  • In the active phase, the granulomas form and grow. In this phase, symptoms can develop, and scar tissue can form in the organs where the granulomas occur.
  • In the nonactive phase, the inflammation goes down, and the granulomas stay the same size or shrink. But the scars may remain and cause symptoms.

The course of the disease varies greatly among people.

  • In many people, sarcoidosis is mild. The inflammation that causes the granulomas may get better on its own. The granulomas may stop growing or shrink. Symptoms may go away within a few years.
  • In some people, the inflammation remains but doesn't get worse. You may also have symptoms or flare-ups and need treatment every now and then.
  • In other people, sarcoidosis slowly gets worse over the years and can cause permanent organ damage. Although treatment can help, sarcoidosis may leave scar tissue in the lungs, skin, eyes, or other organs. The scar tissue can affect how the organs work. Treatment usually does not affect scar tissue.

Changes in sarcoidosis usually occur slowly (e.g., over months). Sarcoidosis does not usually cause sudden illness. However, some symptoms may occur suddenly. They include:

  • Disturbed heart rhythms
  • Arthritis in the ankles
  • Eye symptoms.

In some serious cases in which vital organs are affected, sarcoidosis can result in death.

Sarcoidosis is not a form of cancer.

There is no known way to prevent sarcoidosis.

Sarcoidosis was once thought to be an uncommon condition. It's now known to affect tens of thousands of people throughout the United States. Because many people who have sarcoidosis have no symptoms, it's hard to know how many people have the condition.

Sarcoidosis was identified in the late 1860s. Since then, scientists have developed better tests to diagnose it and made advances in treating it.

What Causes Sarcoidosis?

The cause of sarcoidosis is not known. And, there may be more than one thing that causes it.

Scientists think that sarcoidosis develops when your immune system responds to something in the environment (e.g., bacteria, viruses, dust, chemicals) or perhaps to your own body tissue (autoimmunity).

Normally, your immune system defends your body against things that it sees as foreign and harmful. It does this by sending special cells to the organs that are being affected by these things. These cells release chemicals that produce inflammation around the foreign substance or substances to isolate and destroy them.

In sarcoidosis, this inflammation remains and leads to the development of granulomas or lumps.

Scientists have not yet identified the specific substance or substances that trigger the immune system response in the first place. They also think that sarcoidosis develops only if you have inherited a certain combination of genes.

You can't catch sarcoidosis from someone who has it.

More research is needed to discover what causes sarcoidosis.

Who Gets Sarcoidosis?

Sarcoidosis affects people of all ages and races worldwide.

It occurs mostly in:

  • Adults between the ages of 20 and 40
  • African Americans (especially women)
  • People of Asian, German, Irish, Puerto Rican, and Scandinavian origin.

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

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
  • Nonsmokers
  • Elementary and secondary school teachers
  • People exposed to agricultural dust, insecticides, pesticides, or mold
  • Firefighters.

Brothers and sisters, parents, and children of people who have sarcoidosis are more likely than others to have sarcoidosis.

What Are the Signs and Symptoms of Sarcoidosis?

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 (flem), 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 your neck and chest but sometimes those under your chin, in your arm pits, or in your groin.

Skin Symptoms

  • Various types of bumps, ulcers, or, rarely, flat areas of discolored skin, that appear mostly near your 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 your ankles and shins and can be warm, tender, red or purple-to-red in colour, and slightly raised. This is called erythema nodosum (er"i-the'mah nodo'sum). You 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 (loo'pus per'nio). 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 colour vision
  • Reduced visual clearness
  • Blindness (in rare cases).

Heart Symptoms

  • Shortness of breath
  • Swelling in your 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 your ankles, feet, and hands.
  • Joint pain.
  • Muscle aches (myalgias).
  • Muscle pain, a mass in a muscle, or muscle weakness.
  • Painful arthritis in your 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 your bones.
  • Painless swelling, most often in your fingers.
  • Anaemia that results from granulomas affecting your bone marrow. This usually should be treated.

Liver Symptoms

  • Fever
  • Fatigue
  • Itching
  • Pain in the upper right part of your abdomen, under the right ribs
  • Enlarged liver.

Parotid (pah-rot'id) and Other Salivary Gland Symptoms

  • Swelling, which makes your cheeks look puffy
  • Excessive dryness in your mouth and throat.

Blood, Urinary Tract, and Kidney Symptoms

  • Increased calcium in your 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 your 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 your spinal cord.
  • Weakness, pain, or a "stinging needles" sensation in areas where many nerves are affected by sarcoidosis.

Pituitary (pi-tu'i-tar"e) 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 your blood and pain in your upper left abdomen. Platelets are needed to help your 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 you have these general symptoms but don't have symptoms from affected organs, you probably do not have sarcoidosis.

How Is Sarcoidosis Diagnosed?

Your doctor will find out if you have sarcoidosis 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 your organs
  • Rule out other causes of your symptoms
  • Determine the amount of damage to any of your affected organs
  • Determine whether you need treatment.

Medical History

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

Your doctor may also ask whether you have 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 your lungs that look like the granulomas that are caused by sarcoidosis but are actually signs of other conditions.

Physical Exam

Your doctor will look for symptoms of sarcoidosis, such as red bumps on your skin; swollen lymph nodes; an enlarged liver, spleen, or salivary gland(s); or redness in your eyes. He or she will also listen for abnormal lung sounds or heart rhythm. Your doctor also will check for other likely causes of your 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. Your 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 your heart and lungs. It may show granulomas or enlarged lymph nodes in your 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 your lungs
    • Stage 3: Chest x ray showing shadows in your 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 your symptoms and lung function are. But there are a lot of differences among people. If your x-ray results show Stages 0, 1, 2, or 3, you may not have symptoms or need treatment, and you 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 your blood. They also will show whether there are increases in your calcium levels or changes in your liver, kidney, and bone marrow that can occur with sarcoidosis.

  • Lung Function Tests. One test uses a spirometer (spi-rom'e-ter), a device that measures how much and how fast you can blow air out of your lungs after taking a deep breath. If there is a lot of inflammation and/or scarring in your lungs, you will not be able to move normal amounts of air in and out.

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

  • Electrocardiogram (ECG). This test will help show if your heart is affected by sarcoidosis.

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

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

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

  • Bronchoalveolar Lavage (brong"ko-al-ve'o-lar lah-vaje') (BAL). During bronchoscopy, your doctor may inject a small amount of salt water (saline) through the bronchoscope into your lungs. This fluid washes the lungs and helps bring up cells and other material from the air sacs deep in your lungs where the inflammation usually starts to develop. The cells and fluid are then examined for signs of inflammation.

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

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

    You 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.

  • Computerized Tomography (CT) Scan. This test provides a computer-generated image of your organs that has more detail than a regular chest x ray. It can provide more information about how sarcoidosis has affected an organ.

    Your doctor may do a CT scan to:

    • Obtain more information about how much of your lung is affected by sarcoidosis.
    • Detect sarcoidosis in your liver. A CT scan of your abdomen will show if your 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 your organs that your doctor doesn't want to risk doing a biopsy on. For example, an MR scan can be used to diagnose sarcoidosis in your brain, spinal cord, nerves, or heart.

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

    This test gives information on the tissue in your body that has been affected by sarcoidosis and the amount of damage to it. But since this test shows all inflammation in your 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 your 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 you may have ongoing symptoms.

Your treatment depends on:

  • What symptoms you have
  • How severe your symptoms are
  • Whether any of your vital organs (e.g., your lungs, eyes, heart, or brain) are affected
  • How the organ is affected.

Some organs must be treated, regardless of your symptoms. Others may not need to be treated. Usually, if you don't have symptoms, you don't need treatment, and you 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 these glands don't make enough of certain hormones. This requires treatment by an endocrinologist (en"do-kri-nol'o-jist), a doctor who specializes in the diagnosis and treatment of the endocrine glands. The endocrine glands include your adrenal and pituitary glands.
  • Aseptic (a-sep'tik) or avascular (ah-vas'ku-lar) necrosis (ne-kro'sis) of the hip, the development of cysts and hardened and dead tissue in the hip.

Your doctor can usually help you manage these side effects.

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

You may also want to see an endocrinologist to make sure that your endocrine glands are making enough hormones. The endocrinologist may prescribe certain hormones for you to take until your endocrine glands are working well again.

Other Drugs Used To Treat Sarcoidosis

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

  • Your condition gets worse while you are taking prednisone
  • You can't stand the side effects of prednisone.

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

Most of these drugs also can cause serious side effects. Some also could increase your chances of getting cancer, especially if you take them at high doses.

You and your 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.

You 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 your body.

Drugs used for local therapy include:

  • Eye drops
  • Inhaled drugs for your 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 your chest.

Talk with your 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 your stomach.

    It also can cause eye problems. Before starting on this drug, you should see an ophthalmologist (of"thal-mol'o-jist), or eye doctor, for some baseline tests. Once you start taking it, you should have your 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 you take high doses. These include:

    • Nausea.
    • Mouth sores.
    • A decrease in infection-fighting white blood cells. You then have a greater chance of getting an infection. If you take this drug, you should have regular blood tests to check the levels of your white blood cells.
    • An allergic reaction in your lungs that goes away when you stop taking the drug. This is extremely rare.
    • Liver damage. This is the most serious side effect. If you take methotrexate you should be followed regularly by your physician.

If you are pregnant, you should not take this drug.

Taking folic acid can help you reduce your chances of having bad side effects from methotrexate.

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

    • Nausea
    • Reduced white blood cell levels, which increases your chances of getting an infection.

      This drug has caused cancer in some people, especially when they have taken it at high doses.

      If you are pregnant, you should not take this drug.

  • 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 your white blood cell levels than either methotrexate or azathioprine. Your doctor should check your white blood cell levels often while you are taking this drug to make sure you have a high enough level to fight infection.

    Cyclophosphamide can also irritate your bladder. Some people who have taken it for more than 2 years have developed bladder cancer.

    If you are pregnant, you should not take this drug.

    Cyclophosphamide can be given intravenously (through one of your 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 your eyes almost always responds well to treatment. Often, the only treatment you need is eye drops containing corticosteroids. You should have yearly eye exams, even if you think your eyes are doing well.

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

  • Liver. Sarcoidosis rarely causes permanent liver damage. As a result, your liver usually isn't treated unless it's causing major symptoms (e.g., fever). Drug treatment can usually reduce granulomas in your 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. You should check with your doctor to find out how often you need these tests.

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

  • Erythema nodosum. These painful bumps on your shins often go away in weeks to months without treatment. Your doctor probably will not give you medication unless you are very uncomfortable. Aspirin or ibuprofen, an anti-inflammatory drug that you can buy without a prescription, will usually help.

  • Heart. Sarcoidosis in your heart is usually treated with steroids. You may also be given heart drugs to improve your heart's pumping ability or to correct a disturbed heart rhythm.

    If you have a severe heart rhythm disturbance, your doctor may prescribe one of these devices:

    • A cardiac pacemaker, a small battery-operated device, often put under your skin, that regulates your heartbeat
    • A defibrillator, an implanted device that shocks your heart into a normal heartbeat or, if it has stopped, into beating.

    If your 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 your face, especially your cheeks and nose, can be distressing because it's in a very visible area. It often occurs with loss of your 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, your 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 (so-ri'ah-sis) or ankylosing spondylitis (ang"ki-lo'sing spon"di-li'tis), 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 your 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. More studies are needed.

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.

Living With Sarcoidosis

You should take steps to stay healthy. This includes:

  • Don't smoke.
  • Avoid substances like dusts and chemicals that can harm your lungs.
  • Try to follow a healthy eating plan.
  • Be as active as you can but don't strain yourself.

Joining a patient support group may help you adjust to living with sarcoidosis. Talking to others who have the same symptoms can help you see how they have coped with them.

Your regular doctor may be able to diagnose and treat your sarcoidosis, but diagnosis and treatment by a doctor who specializes in sarcoidosis is recommended. If you prefer to use your regular doctor, you should see a doctor who specializes in the organs that are affected by your sarcoidosis at least once. For example, see an ophthalmologist if your eyes are affected or a pulmonologist if you have sarcoidosis in your lungs. These specialists are often found at major medical centers. They will work with your 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 you can continue corticosteroid treatment through your pregnancy. None of the other drugs are recommended for use during pregnancy.

Sometimes your 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 you to discuss this issue with your doctor. If you become pregnant, you 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 you aren't taking medication for your sarcoidosis. New symptoms can occur at any time, and your condition can get worse slowly, without your noticing.

Followup exams usually include:

  • A review of your symptoms
  • A physical exam
  • A chest x ray and CT scan
  • Breathing tests
  • An eye exam
  • Blood tests
  • An electrocardiogram (EKG).

How often you have your examinations and tests depends on:

  • How severe your symptoms are
  • Which organs were affected at diagnosis
  • What treatment you are using
  • Any complications that may develop during treatment.

You will probably need routine followup care for several years. Whether you see your regular doctor or a sarcoidosis specialist for this depends on your symptoms during the first year of followup.

Here are some examples of how your followup care can be managed. They are based on either your condition when you were diagnosed with sarcoidosis or the treatment used.

Followup After Initial Diagnosis

  1. If at diagnosis, you have no symptoms, a normal breathing test, and an abnormal chest x ray:
  • You should plan on having a followup exam every 6 to12 months until your condition is stable or improving.

  • Your breathing test may need to be repeated. The need to repeat it depends on your symptoms and ability to be active.

If at your first followup visit, you have no new symptoms and your chest x ray is normal, you can go to your regular doctor for future followup care.

  1. If at diagnosis, you have some symptoms, an abnormal chest x ray, but you don't need treatment:
  • You should plan on having a followup exam in 3 to 6 months.

If at your followup exam, your condition has gotten worse (i.e., you now have more symptoms, an abnormal x ray, or abnormal lab tests) you may need treatment.

  • If treatment is started, you may need followup tests more often.

Followup Based on Your Drug Treatment

If treatment is begun with prednisone:

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

If treatment is begun with hydroxychloroquine:

  • You should have an eye exam every 6 months while taking this drug.

If treatment is begun with methotrexate:

  • You should have blood tests every month or every other month to see if you have anaemia, low white blood cell or platelet levels, or liver inflammation.

Other Followup Tests

Depending on how serious your condition is and what organs are affected, you may also need to have certain tests 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 you may have eye damage even if you don't have symptoms.

These tests may include:

  • A slit lamp examination. Your doctor uses an instrument with a high-intensity light source to look at the front of your eyes.
  • A visual fields examination. Your doctor will ask you to you to look at a light through an instrument.
  • Inspection of your retina and optic nerve.

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

You should also have regular eye exams if you are being treated with:

  • Chloroquine or hydroxycholoroquine (Plaquenil)
  • Corticosteroids.

Breathing Tests

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

Blood Tests

A blood test for calcium should be done. If your calcium level is high, you probably will need to be treated. You also should not take vitamin and mineral supplements containing calcium or vitamin D, and you should avoid too much exposure to the sun.

Electrocardiogram

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

Key Points

  • Sarcoidosis involves inflammation that produces tiny lumps of cells called granulomas in various organs in your body. These granulomas can grow and clump together, making many large and small groups of lumps. If many granulomas form in an organ, they may affect how the organ works and cause symptoms.

  • Sarcoidosis can occur in almost any part of your body, although it usually starts in either your lungs or lymph nodes. It also often affects your skin, eyes, and liver.

  • Sarcoidosis can also affect your spleen, brain, nerves, and heart.

  • The course of sarcoidosis varies greatly among people. Sometimes, it's mild, and the symptoms may go away within a few years, even without treatment. But sometimes sarcoidosis slowly gets worse over the years and can cause permanent organ damage.

  • The cause of sarcoidosis is unknown. Doctors think that it may result when your immune system overreacts to some kind of foreign substance. Scientists also think that your immune system acts this way only if you have inherited a certain mix of genes.

  • Sarcoidosis affects men and women of all ages and races worldwide. It occurs mostly in people ages 20 to 40; African Americans, especially women; and people of Asian, German, Irish, Puerto Rican, and Scandinavian origin.

  • Many people with sarcoidosis have no symptoms. Symptoms usually depend on which organs the disease affects. Symptoms from sarcoidosis in the lungs and lymph nodes include shortness of breath, a dry cough, wheezing, and enlarged and sometimes tender lymph nodes.

  • Your doctor will determine if you have sarcoidosis by taking a detailed medical history and conducting a physical exam and several tests. The tests may include a chest x ray, blood and lung function tests, an electrocardiogram, magnetic resonance scan, and scans using radioactive elements.

  • Your doctor may also insert a long, narrow, flexible tube with a light on the end, called a bronchoscope, through your nose or mouth into your lungs to look at your airways and to obtain samples of cells and other tissue for examination under a microscope. You most likely would have this procedure as an outpatient in a hospital under local anaesthesia.

  • Treatment for sarcoidosis depends on your symptoms and how severe they are, whether any of your critical organs (e.g., your lungs, eyes, heart, brain) are affected, and how they are affected.

  • The main treatment for sarcoidosis is prednisone, a corticosteroid or anti-inflammatory drug. It's usually given for many months, sometimes even for a year or two. When used for a long time at high doses, prednisone can cause serious side effects, including diabetes, high blood pressure, depression, heartburn, acne, osteoporosis, cataracts, and glaucoma. It can also affect the body's production of certain hormones.

  • Other drugs may be used to treat sarcoidosis if your condition gets worse while you are taking prednisone or you can't stand its side effects. Most of these other drugs are immune system suppressants that can cause serious side effects.

  • Local therapy is the safest way to treat sarcoidosis. Localized drugs include eye drops, inhaled drugs for your lungs, and skin creams.

  • Research is being done to improve the diagnosis and treatment of sarcoidosis. Scientists are studying drugs that are used for other conditions to see if they can help people who have sarcoidosis. These drugs include several strong immune system suppressants, tetracycline antibiotics, and thalidomide.

  • Scientists also are conducting research to find out more about what causes sarcoidosis, why it seems to act differently in people of different races, and what genes are involved.

  • If you have sarcoidosis, you should take steps to stay healthy, including don't smoke, avoid substances like dusts and chemicals that can harm your lungs, follow a healthy eating plan, and be as active as you can without straining yourself.

  • Joining a patient support group may help you adjust to living with sarcoidosis.

  • Your regular doctor may be able to diagnose and treat your sarcoidosis, but diagnosis and treatment by a doctor who specializes in sarcoidosis is recommended. These specialists are often found at major medical centers. Even if you want to be treated mainly by your regular doctor, you should see a doctor who specializes in the organs that are affected by your sarcoidosis at least once. He or she can work with your regular doctor to make sure you have a good treatment plan.

  • If you are thinking about getting pregnant, you should talk to your doctor about how sarcoidosis may affect your pregnancy. Pregnancy usually doesn't affect the course of sarcoidosis, but it may flare up after delivery. You can continue taking corticosteroids during your pregnancy. None of the other drugs that are used to treat sarcoidosis are recommended for use then.

  • If you are pregnant, you should have good prenatal care and sarcoidosis checkups during and after pregnancy.

  • Regular followup care is important, even if you aren't taking medication, as new symptoms can occur at any time, and your condition can get worse slowly, without your noticing.

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