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Overview

Name: Sarcoidosis

Description: NHLBI

This casebook is published and has been read 1163 times.

The author of this casebook has identified the following medical topics as being highly relevant to this casebook.

  • Sarcoidosis -- WHAT: Sarcoidosis: Sarcoidosis: a disorder of unknown etiology that affects many organ systems with noncaseating epithelioid cell granulomas. It has a special predilection for the lung and lymph tissues. WHY: Sarcoidosis can result in an acute arthritis commonly affecting the ankles and knees and less commonly the proximal interphalangeal joints, wrists, and elbows. The acute arthritis is symmetric and lasts for a few weeks. A less common chronic arthritis is destructive and may have dactylitis and telescoping digits. HOW: Sarcoidosis is a diagnosis of exclusion based on the clinical presentation and histology of biopsy tissue.
  • Pulmonary Sarcoidosis -- Sarcoidosis affecting predominantly the lungs, the site most frequently involved and most commonly causing morbidity and mortality in sarcoidosis. Pulmonary sarcoidosis is characterized by sharply circumscribed granulomas in the alveolar, bronchial, and vascular walls, composed of tightly packed cells derived from the mononuclear phagocyte system. The clinical symptoms when present are dyspnea upon exertion, nonproductive cough, and wheezing. (Cecil Textbook of Medicine, 19th ed, p431)
Notes

What Is Sarcoidosis?

Sarcoidosis (sar-koy-DO-sis) is a disease of unknown cause that leads to inflammation. It can affect various organs in the body.

Normally, your immune system defends your body against foreign or harmful substances. For example, it sends special cells to protect organs that are in danger.

These cells release chemicals that recruit other cells to isolate and destroy the harmful substance. Inflammation occurs during this process. Once the harmful substance is destroyed, the cells and the inflammation go away.

In people who have sarcoidosis, the inflammation doesn't go away. Instead, some of the immune system cells cluster to form lumps called granulomas (gran-yu-LO-mas) in various organs in your body.

Overview

Sarcoidosis can affect any organ in your body. However, it's more likely to occur in some organs than in others. The disease usually starts in the lungs, skin, and/or lymph nodes (especially the lymph nodes in your chest).

The disease also often affects the eyes and the liver. Although less common, sarcoidosis can affect the heart and brain, leading to serious complications.

If many granulomas form in an organ, they can affect how the organ works. This can cause signs and symptoms. Signs and symptoms vary depending on which organs are affected. Many people who have sarcoidosis have no symptoms or mild symptoms.

Lofgren's syndrome is a classic set of signs and symptoms that is typical in some people who have sarcoidosis. Lofgren's syndrome may cause fever, enlarged lymph nodes, arthritis (usually in the ankles), and/or erythema nodosum (er-i-THE-ma no-DO-sum).

Erythema nodosum is a rash of red or reddish-purple bumps on your ankles and shins. The rash may be warm and tender to the touch.

Treatment for sarcoidosis also varies depending on which organs are affected. Your doctor may prescribe topical treatments and/or medicines to treat the disease. Not everyone who has sarcoidosis needs treatment.

Outlook

The outcome of sarcoidosis varies. Many people recover from the disease with few or no long-term problems.

More than half of the people who have sarcoidosis have remission within 3 years of diagnosis. "Remission" means the disease isn't active, but it can return.

Two-thirds of people who have the disease have remission within 10 years of diagnosis. People who have Lofgren's syndrome usually have remission. Relapse (return of the disease) 1 or more years after remission occurs in less than 5 percent of patients.

Sarcoidosis leads to organ damage in about one-third of the people diagnosed with the disease. Damage may occur over many years and involve more than one organ. Rarely, sarcoidosis can be fatal. Death usually is the result of complications with the lungs, heart, or brain.

Poor outcomes are more likely in people who have advanced disease and show little improvement from treatment.

Certain people are at higher risk for poor outcomes from chronic (long-term) sarcoidosis. This includes people who have lung scarring, heart or brain complications, or lupus pernio (LU-pus PAR-ne-o). Lupus pernio is a serious skin condition that sarcoidosis may cause.

Research is ongoing for new and better treatments for sarcoidosis.

What Causes Sarcoidosis?

The cause of sarcoidosis isn't known. More than one factor may play a role in causing the disease.

Some researchers think that sarcoidosis develops when your immune system responds to a trigger, such as bacteria, viruses, dust, or chemicals.

Normally, your immune system defends your body against foreign or harmful substances. For example, it sends special cells to protect organs that are in danger.

These cells release chemicals that recruit other cells to isolate and destroy the harmful substance. Inflammation occurs during this process. Once the harmful substance is destroyed, the cells and the inflammation go away.

In people who have sarcoidosis, the inflammation doesn't go away. Instead, some of the immune system cells cluster to form lumps called granulomas in various organs in your body.

Genetics also may play a role in sarcoidosis. Researchers believe that sarcoidosis occurs if:

  • You have a certain gene (or certain genes) that raise your risk for the disease
  • —And—
  • You're exposed to something that triggers your immune system

Triggers may vary depending on your genetic makeup. Certain genes may influence which organs are affected and how severe your symptoms are.

Researchers continue to try to pinpoint the genes that are linked to sarcoidosis.

Who Is At Risk for Sarcoidosis?

Sarcoidosis affects people of all ages and races. However, it's more common among African Americans and Northern Europeans. In the United States, the disease affects African Americans somewhat more often and more severely than Whites.

Studies have shown that sarcoidosis tends to vary in different ethnic groups. For example, eye problems due to the disease are more common in Japanese people.

Lofgren's syndrome, a type of sarcoidosis, is more common in people of European descent. Lofgren's syndrome may involve fever, enlarged lymph nodes, arthritis (usually in the ankles), and/or erythema nodosum. Erythema nodosum is a rash of red or reddish-purple bumps on your ankles and shins. The rash may be warm and tender to the touch.

Sarcoidosis is somewhat more common in women than in men. The disease usually develops between the ages of 20 and 50.

People who have certain jobs also may be at higher risk for sarcoidosis. Examples include:

  • Health care workers
  • Elementary and secondary school teachers
  • People whose jobs expose them to agricultural dust, insecticides, pesticides, or mold
  • Suppliers of building materials, hardware, or gardening materials
  • Firefighters

People who have a family history of sarcoidosis also are at higher risk for the disease.

What Are the Signs and Symptoms of Sarcoidosis?

Many people who have sarcoidosis have no symptoms or mild symptoms. Often, the disease is found during a chest x ray done for another reason (for example, to diagnose pneumonia).

The signs and symptoms of sarcoidosis vary depending on which organs are affected. Signs and symptoms also may vary depending on your gender, age, and ethnic background. (For more information, see "Who Is At Risk for Sarcoidosis?")

Common Signs and Symptoms

In both adults and children, the disease most often affects the lungs. If granulomas (inflamed lumps) form in your lungs, you may wheeze, cough, feel short of breath, or have chest pain. Or, you may have no symptoms at all.

Some people who have sarcoidosis feel very fatigued (tired), uneasy, or depressed. Night sweats and weight loss are common symptoms of the disease.

Common signs and symptoms in children are fatigue, loss of appetite, weight loss, bone and joint pain, and anemia. Children who are younger than 4 years old may have a distinct form of sarcoidosis. It may cause enlarged lymph nodes in the chest (which can be seen on a chest x ray), skin lesions, and eye swelling or redness.

Other Signs and Symptoms

Sarcoidosis may affect your lymph nodes. The disease can cause enlarged lymph nodes that feel tender. Sarcoidosis usually affects the lymph nodes in your neck and chest. However, the disease also may affect the lymph nodes under your chin, in your armpits, or in your groin.

Sarcoidosis can cause lumps, ulcers, or areas of discolored skin. They may itch, but they don't hurt. These signs tend to appear on your back, arms, legs, and scalp. Sometimes they appear near your nose or eyes. These signs usually last a long time.

Sarcoidosis may cause a more serious skin condition called lupus pernio. Disfiguring skin sores may affect your nose, nasal passages, cheeks, ears, eyelids, and fingers. These sores tend to be ongoing. They can return after treatment is over.

Sarcoidosis also can cause eye problems. If you have sarcoidosis, it's important to have an annual eye exam. If you have changes in your vision and can't see as clearly or can't see color, call 9–1–1 or have someone drive you to the emergency room.

You should call your doctor if you have any new eye symptoms, such as burning, itching, tearing, pain, or sensitivity to light.

Signs and symptoms of sarcoidosis also may include an enlarged liver, spleen, or salivary glands.

Although less common, sarcoidosis can affect the heart and brain. This can cause a number of symptoms, such as abnormal heartbeats, shortness of breath, headaches, and vision problems. If sarcoidosis affects the heart or brain, serious complications can occur.

Lofgren's Syndrome

Lofgren's syndrome is a classic set of signs and symptoms that appear in some people when they first develop sarcoidosis. Signs and symptoms may include:

  • Fever. This symptom only occurs in some people.
  • Enlarged lymph nodes (which can be seen on a chest x ray).
  • Arthritis, usually in the ankles. This symptom is more common in men.
  • Erythema nodosum. This is a rash of red or reddish-purple bumps on your ankles and shins. The rash may be warm and tender to the touch. This symptom is more common in women.

Sarcoidosis Signs and Symptoms

The illustration shows the major signs and symptoms of sarcoidosis (as described in this section) and the organs involved.

The illustration shows the major signs and symptoms of sarcoidosis and the organs involved.

How Is Sarcoidosis Diagnosed?

Your doctor will diagnose sarcoidosis based on your medical history, a physical exam, and the results from tests. He or she will look for granulomas (inflamed lumps) in your organs. Your doctor also will try to rule out other causes of your symptoms.

Medical History

Your doctor may ask you detailed questions about your medical history. For example, he or she may ask whether you have a family history of sarcoidosis. Your doctor also may ask whether you've had any jobs that may have raised your risk for the disease.

Your doctor also may ask whether you've ever been exposed to inhaled beryllium metal. This type of metal is used to make aircrafts and weapons. Your doctor also may want to know whether you've had contact with organic dust from birds or hay.

Exposure to these substances can cause inflamed lumps in your lungs that look like the granulomas from sarcoidosis. However, these lumps are signs of other conditions.

Physical Exam

Your doctor will examine you for signs and symptoms of sarcoidosis. Signs and symptoms may include red bumps on your skin; swollen lymph nodes; an enlarged liver, spleen, or salivary glands; or redness in your eyes. He or she will check for other causes of your symptoms.

Your doctor also may listen to your lungs and heart. Abnormal breathing and heartbeat sounds may be a sign that sarcoidosis is affecting your lungs or heart.

Diagnostic Tests

You may have tests to confirm a diagnosis and to find out how the disease is affecting you. Tests include chest x ray, lung function tests, biopsy, and other tests to assess organ damage.

Chest X Ray

A chest x ray is a painless test that creates pictures of the structures inside your chest, such as your heart and lungs. The test may show granulomas or enlarged lymph nodes in your chest. About 95 percent of people who have sarcoidosis have an abnormal chest x ray.

Lung Function Tests

Lung function tests measure the size of your lungs, how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. These tests may be used to find out whether sarcoidosis is affecting your lungs.

Biopsy

Your doctor may do a biopsy to confirm a diagnosis or rule out other causes of your symptoms. A biopsy involves taking a small sample of tissue from one of your affected organs.

Usually, doctors try to biopsy the organs that are easiest to access. Examples include the skin, tear glands, or the lymph nodes that are just under the skin.

If this isn't possible, your doctor may use a positron emission tomography (PET) scan to pinpoint areas for biopsy. For this test, a small amount of a radioactive substance is injected into a vein, usually in your arm.

The substance, which releases energy, travels through the blood and collects in organs or tissues. Special cameras detect the energy and convert it into three-dimensional pictures.

If lung function tests or a chest x ray shows signs of sarcoidosis in your lungs, your doctor may do a bronchoscopy (bron-KOS-ko-pee) to get a small sample of lung tissue.

During this procedure, a thin, flexible tube is passed through your nose (or sometimes your mouth), down your throat, and into the airways to reach your lung tissue. (For more information, see "What To Expect During Bronchoscopy.")

Other Tests To Assess Organ Damage

You also may have other tests to assess organ damage and find out whether you need treatment. For example, your doctor may recommend blood tests and/or an EKG (electrocardiogram).

Everyone who is diagnosed with sarcoidosis should see an ophthalmologist (eye specialist) for eye tests, even if they don't have eye symptoms. This is important because eye damage can occur without symptoms.

How Is Sarcoidosis Treated?

Not everyone who has sarcoidosis needs treatment. In some cases, the disease goes away on its own. Whether you need treatment and what type of treatment you need depend on your signs and symptoms, which organs are affected, and whether those organs are working well.

If the disease affects certain organs, such as your eyes, heart, or brain, you'll need treatment even if you don't have any symptoms.

In either case-whether you have symptoms or not-you should see your doctor for ongoing care. He or she will want to check to make sure that the disease isn't damaging your organs. For example, you may need lung function tests to make sure that your lungs continue to work well.

If the disease isn't worsening, your doctor may watch you closely to see whether the disease goes away on its own. If the disease does start to get worse, your doctor can prescribe treatment.

The goals of treatment are to:

  • Relieve symptoms
  • Improve organ function
  • Control inflammation and reduce the size of granulomas (inflamed lumps)
  • Prevent pulmonary fibrosis (lung scarring) if your lungs are affected

Your doctor may prescribe topical treatments and/or medicines to treat the disease.

Medicines

Prednisone

Prednisone, a type of steroid, is the main treatment for sarcoidosis. This medicine reduces inflammation. In most people, prednisone relieves symptoms within a couple of months.

Although most people need to take prednisone for 12 months or longer, your doctor may lower the dose within a few months after you start the medicine.

Long-term use of prednisone, especially at high doses, can cause serious side effects. Work with your doctor to decide whether the benefits of this medicine outweigh the risks. If your doctor prescribes this treatment, he or she will find the lowest dose that controls your disease.

When you stop taking prednisone, you should cut back slowly (as your doctor advises). This will help prevent flareups of sarcoidosis. Cutting back slowly also allows your body to adjust to not having the medicine.

If a relapse or flareup occurs after you stop taking prednisone, you may need a second round of treatment. If you remain stable for more than 1 year after stopping this treatment, the risk of relapse is low.

Other Medicines

Other medicines, besides prednisone, also are used to treat sarcoidosis. Examples include:

  • Hydroxychloroquine. This medicine works best for treating sarcoidosis that affects the skin or brain. Your doctor also may prescribe this medicine if you have a high level of calcium in your blood due to sarcoidosis.
  • Methotrexate. This medicine works best for treating sarcoidosis that affects your lungs, eyes, skin, or joints.

Your doctor may prescribe these medicines if your sarcoidosis worsens while you're taking prednisone or if you can't handle prednisone's side effects.

If you have Lofgren's syndrome with pain or fever, your doctor may prescribe nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen.

If you're wheezing and coughing, you may need inhaled medicine to help open your airways. You take inhaled medicine using an inhaler. This device allows the medicine to go right to your lungs.

Ongoing Research

Researchers continue to look for new and better treatments for sarcoidosis. They're currently studying treatments aimed at the immune system. Researchers also are studying antibiotics as a possible treatment for sarcoidosis that affects the skin.

Living With Sarcoidosis

Sarcoidosis has no cure. However, you can take steps to manage the disease. Get ongoing care and follow a healthy lifestyle. Talk to your doctor if you’re pregnant or planning a pregnancy.

Ongoing Care

Getting ongoing care is important, even if you don't take medicine for your sarcoidosis. New symptoms can occur at any time. Also, the disease can slowly worsen without your noticing.

How often you need to see your doctor will depend on how severe your symptoms are, which organs are affected, what treatments you're using, and whether you have any side effects from treatments. Even if you don’t have symptoms, you should see your doctor for ongoing care.

Your doctor may recommend routine tests, such as lung function tests and eye exams. He or she will want to check to make sure that the disease isn’t damaging your organs.

Discuss with your doctor how often you need to have followup visits. You may have some followup visits with your primary care doctor and others with one or more specialists.

Lifestyle Changes

Making lifestyle changes can help you manage your health. For example, follow a healthy diet and be as physically active as you can. A healthy diet includes a variety of fruits, vegetables, and whole grains.

It also includes lean meats, poultry, fish, beans, and fat-free or low-fat milk or milk products. A healthy diet is low in saturated fat, trans fat, cholesterol, sodium (salt), and added sugar.

For more information on following a healthy diet, see the National Heart, Lung, and Blood Institute's Aim for a Healthy Weight Web site, "Your Guide to a Healthy Heart," and "Your Guide to Lowering Your Blood Pressure With DASH." All of these resources include general information about healthy eating.

If you smoke, quit. Talk to your doctor about program and products that can help you quit. Also, try to avoid other lung irritants, such as dust, chemicals, and secondhand smoke.

Emotional Issues

Living with a chronic disease may cause fear, anxiety, depression, and stress. It’s important to talk about how you feel with your health care team. Talking to a professional counselor also can help. If you’re feeling very depressed, your health care team or counselor may prescribe medicines to make you feel better.

Joining a patient support group may help you adjust to living with sarcoidosis. You can see how other people who have the disease have coped with it. Talk to your doctor about local support groups or check with an area medical center.

Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.

Pregnancy

Many women who have sarcoidosis give birth to healthy babies. Women who have severe sarcoidosis, especially if they’re older, may have trouble becoming pregnant. In some cases, sarcoidosis may get worse after the baby is delivered.

If you have sarcoidosis and are pregnant or planning a pregnancy, talk to your doctor about the risks. Also, if you become pregnant, it’s important to get good prenatal care and regular sarcoidosis checkups during and after pregnancy.

Some sarcoidosis medicines are considered safe to use during pregnancy; others are not recommended.

Key Points

  • Sarcoidosis is a disease of unknown cause that leads to inflammation. It can affect various organs in the body.
  • Sarcoidosis is more likely to occur in some organs than in others. The disease usually starts in the lungs, skin, and/or lymph nodes (especially the lymph nodes in your chest). The disease also often affects the eyes and the liver.
  • In people who have sarcoidosis, immune system cells cause inflammation and cluster to form lumps called granulomas.
  • If many granulomas form in an organ, they can affect how the organ works. This can cause signs and symptoms. Signs and symptoms vary depending on which organs are affected. Many people who have sarcoidosis have no symptoms or mild symptoms.
  • Some researchers think that sarcoidosis develops when your immune system responds to a trigger, such as bacteria, viruses, dust, or chemicals. Genetics also may play a role in sarcoidosis.
  • Sarcoidosis affects people of all ages and races. In the United States, the disease affects African Americans somewhat more often and more severely than Whites. The disease also is slightly more common in women than in men. It usually develops between the ages of 20 and 50.
  • In both adults and children, the disease most often affects the lungs. If granulomas form in your lungs, you may wheeze, cough, feel short of breath, or have chest pain. Some people who have sarcoidosis feel very fatigued (tired), uneasy, or depressed. Night sweats and weight loss are common symptoms of the disease.
  • Lofgren's syndrome is a classic set of signs and symptoms that appear in some people when they first develop sarcoidosis. Signs and symptoms may include fever, enlarged lymph nodes, arthritis, and erythema nodosum (a rash of red or reddish-purple bumps on your ankles).
  • Your doctor will diagnose sarcoidosis based on your medical history, a physical exam, and the results from tests. He or she will look for granulomas (inflamed lumps) in your organs. Your doctor also will try to rule out other causes of your symptoms.
  • Whether you need treatment and what type of treatment you need depend on your signs and symptoms, which organs are affected, and whether those organs are working well.
  • Your doctor may prescribe topical treatments and/or medicines to treat the disease. Not everyone who has sarcoidosis needs treatment. In some cases, the disease goes away on its own.
  • If the disease affects certain organs, such as your eyes, heart, or brain, you may need treatment even if you don't have any symptoms.
  • Sarcoidosis has no cure. However, you can take steps to manage the disease. Get ongoing care and follow a healthy lifestyle. Talk to your doctor if you're pregnant or planning a pregnancy.
  • Getting ongoing care is important, even if you don't take medicine for your sarcoidosis. New symptoms can occur at any time. Also, your condition can slowly worsen without your noticing.
  • The outcome of sarcoidosis varies. Many people recover from the disease with few or no long-term problems.
  • Rarely, sarcoidosis can be fatal. Death usually is the result of complications with the lungs, heart, or brain. Poor outcomes are more likely in people who have advanced disease and show little improvement from treatment.
Bookmarks The following information, which has been distilled by the casebook author from this and other websites is particularly relevant to this casebook.
Bookmarks - Web
Web Page Notes Concepts
 What is sarcoidosis? (Sarcoidosis)
 
 Sarcoidosis: MedlinePlus (Sarcoidosis)
 
 Sarcoidosis Signs & Symptoms - Sarcoidosis - Pulmonology Channel Pulmonary Channel (Sarcoidosis)
 
 FSR - Diagnosis of Sarcoidosis Foundation for Sarcoidosis Research (Sarcoidosis)
 
 Sarcoidosis Treatment Options Cleveland Clinic (Sarcoidosis)
 
 Sarcoidosis - MayoClinic.com Mayo Clinic (Pulmonary Sarcoidosis)
 

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