Sarcoidosis Overview

What is Sarcoidosis?

Sarcoidosis is a disease where the immune system overreacts and makes too many small clusters of inflamed cells. These cells are called granulomas. These usually affect the lungs and lymph nodes. They can also appear in the skin, eyes, heart, nervous system and other organs. Sarcoidosis is a rare disease: According to the American Lung Association, about 27,000 people (Opens in a new window) develop it each year. Sarcoidosis is not contagious. There is no cure, but treatment can reduce organ damage and control the disease. 

Types of Sarcoidosis

Doctors may describe sarcoidosis by the main organ or system involved.

Cardiac (Heart). Sarcoidosis can disrupt the heart’s electrical system. It can cause abnormal heartbeats. It also can damage the heart muscle and lead to heart failure. There is a risk that one in four people (Opens in a new window) with this diagnosis will have heart issues too. Because of that risk, most patients are screened for heart problems. 

Cutaneous (Skin). The skin is the body’s biggest organ. About 30% (Opens in a new window) of patients will have cutaneous sarcoidosis. This type causes red or violet lumps on the lower arms, legs, face, ears, fingers, toes, scalp, back or buttocks. These bumps are sometimes painful.

Hepatic (Liver). Sarcoidosis in the liver can occur in from 6% to almost 80% (Opens in a new window) of patients. These patients often do not have symptoms. This type can cause jaundice, malaise, fatigue, enlarged liver and abnormal liver test results.

Lymph Nodes. These glands are found throughout the body. They make and store white blood cells. Sarcoidosis causes swollen lymph nodes.

Musculoskeletal. Sarcoidosis in the bones, muscles and joints can weaken them. It also can cause  painful limps, joint pain, swelling and stiffness.

Neurosarcoidosis (Brain & Nervous System). This type develops in the brain, spinal cord and optic nerve. It can damage the protective covering around nerves, which stops signals from traveling normally. Blocked nerve signals cause loss of body functions. If it affects nerves in the arms and legs, it can cause tingling, weakness or numbness. 

Ocular (Eye). The eyes can be affected in up to 79% (Opens in a new window)  of sarcoidosis patients. Any part of the eye and surround tissue can be affected. It can lead to eye pain, abnormal vision and other eye health issues.

Pulmonary (Lung). This is the most common type of sarcoidosis (Opens in a new window). Sometimes lung involvement is found by chance. It affects nearly 90% of patients. It can lead to severe cough, shortness of breath and difficulty with daily activities. In severe cases it can cause lung scarring, high blood pressure in the lungs, reduced lung function and infections. In rare cases it can cause respiratory failure.

Renal (Kidney). This type occurs in nearly one-third (Opens in a new window) of all patients. It can elevate calcium levels in the blood and urine. High calcium levels can cause dehydration, kidney stones and sometimes kidney failure.

Salivary gland. This type of sarcoidosis affects the mouth. It can lead to puffiness or fullness in the face. It also can cause dry mouth, swollen and tender glands, tooth decay, mouth infections and facial asymmetry. 

Sinus. Sarcoidosis in the sinuses can lead to loss of smell, congestion and facial pressure or pain. It also can cause nosebleeds, infections and runny nose. 

Spleen and bone marrow. This type can lead to anemia, spleen enlargement, leukopenia, clotting and other issues. 

Causes

The exact cause of sarcoidosis is unknown. It may be triggered by infections. It also could be caused by exposure dust, mold, smoke or chemicals. Experts believe that some people are genetically prone to this disease. In these people, an infection or environmental exposure may trigger an immune system reaction. This reaction causes inflammation and leads to granulomas forming. 

Risk Factors

This condition can affect anyone. Risk can be higher in certain populations. It also may be higher if a close family member has the disease.

• African Americans are 10 times more at risk than Caucasians
• People of European descent (especially Scandinavian) 
• Family history of sarcoidosis or similar immune disease. 
• Ages of 20 and 40 
• Women are more at risk than men

Prevention

There is no clear way to prevent sarcoidosis. You may lower your risk and help protect your overall lung and immune health with these tips:

• Avoid exposure to air pollution, chemical fumes, dust, mold and smoke.  
• Limit time outdoors on bad air pollution days and do not exercise during peak exposures.
• Wear a protective mast or ventilation if you work around irritants.
• Do not smoke or vape.
• Eat a balanced diet.
• Get quality sleep.
• Exercise regularly.
• Manage stress.
• Stay up to date on your vaccinations to prevent infections.
• Avoid people who are sick, especially with respiratory infections.

Signs and Symptoms

Sarcoidosis symptoms depend on which organs are involved. Each person’s experience is different. About 50% (Opens in a new window) of those with the disease do not have symptoms when first diagnosed. This condition has general symptoms and symptoms by organ. 

General symptoms

• Weight loss
• Night sweats
• Fatigue
• Low grade fever

Symptoms by Organ

The lungs are the most common organ affected by sarcoidosis. However, any organ, and more than one organ, can be affected

Learn more from our infographic. 

When to Call a Doctor

Seek medical care if you have symptoms of sarcoidosis. It’s important that you have a doctor check a long-lasting cough, shortness of breath, new vision changes, heart palpitations, fainting or nervous system symptoms.  

Diagnosis

Sarcoidosis is diagnosed only after other conditions have been eliminated as the cause. A medical evaluation, diagnostic procedures and testing are used to rule out other causes. During an evaluation, the skin, eyes and lymph nodes are checked for signs of the disease. Then, a biopsy or lavage is often done to look for evidence of granulomas. Because the granulomas in sarcoidosis can look like other diseases, testing is often ordered to rule out other causes. 

Getting diagnosed when symptoms first appear is important. Starting treatment early can help prevent damage to the lungs, eyes, heart and other organs. It is also important to continue to follow up with the doctor to watch if disease is progressing.

Breathing and Exercise Tests

Pulmonary Function Tests: These tests show how well the lungs are working and the lung disease severity level. 

Cardiac Tests

Electrocardiogram (EKG): This test checks the heart’s electrical system. It detects changes in how the heart’s signals work. A continuous EKG or Holter monitor can be worn to record heart activity over a few days. 

Imaging Tests

Cardiac MRI: A cardiac MRI is the best way to see if the heart has scar tissue. The buildup of scar tissue can lead to complications. An MRI also measures the heart’s size and determines how well it is pumping.

Chest X-ray: This test shows evidence of enlarged lymph nodes and spots of cell clusters on the lungs. It can also be used to stage the lung involvement. A scale of 0 – 4 is often used, with 4 showing the most lung involvement.

CT (Computerized Tomography) Scan: A CT scan is a detailed type of X-ray. The scan shows lymph nodes and scars in the lungs that regular X-rays do not see.

Echocardiogram: This test evaluates the structure and function of the heart.

PET (Positron Emission Tomography) Scans (CT or MRI): A PET scan shows areas in the body that have active inflammation. 

Single Photo Emission Computed Tomography (SPECT): This test shows how blood flows to tissues and organs in the body. It also shows changes in the heart muscle. A SPECT test also can determine the extent of sarcoidosis in muscles, bones and joints. It also can be used to find the best place in the body to take a tissue sample for biopsy. 

Lab Tests

Calcium Levels in Blood and Urine: This condition can cause too much calcium in the blood and/or urine. A 24-hour urine sample can measure the calcium level in the urine. A blood sample can be drawn for levels of calcium in the blood. High calcium levels can cause fatigue, abdominal pain, constipation, mental fogginess and kidney dysfunction.

Procedures

Bronchoalveolar Lavage: During this procedure, a small part of the lung is washed (lavaged) with a sterile saline solution. This lets the doctor see which immune system cells are in the lung. Counting the types of cells helps estimate the level of inflammation. A lavage can also determine if the inflammation looks like sarcoidosis.

Slit Lamp Procedure: This procedure is done by an medical eye doctor, called an ophthalmologist. It looks for signs of active inflammation on the eye. 

Skin or Tissue Biopsy: This test takes a tissue sample from the skin, lungs or other affected organs to look for cell clusters (granulomas). 

Lumbar Puncture: This test looks at the spinal fluid. It can show evidence of inflammation in the central nervous system.

Treatment

Not everyone with sarcoidosis needs treatment right away. If symptoms are mild and organs are functioning well, your doctor may recommend monitoring. Almost half of those diagnosed improve without treatment. When treatment is needed, medications can reduce inflammation. Many people will recover. Some people will get worse or will have other areas affected. Since this disease can affect different organs, it is important to be treated by a multidisciplinary team.

The goals of treatment are to:

• Maintain good organ function
• Control and lessen symptoms
• Prevent organ damage 
• Improve outcomes

Medications

Corticosteroids: This is a steroid medicine that reduces inflammation. It is the main treatment for sarcoidosis. Prednisone is the most commonly used steroid. It can reduce symptoms and granuloma formation. It also can improve lung function. Prednisone may also help reduce scarring in the lungs. Because this drug can cause side effects, your doctor will monitor you closely.  

Immune System Suppressants: This type of medicine calms an overactive immune system. It helps reduce symptoms. Common options include methotrexate, azathioprine and mycophenolate mofetil (CellCept). If these do not work, doctors may use biologics such as rituximab, abatacept or adalimumab. In severe cases, stronger medicines like cyclophosphamide or other TNF-blockers may be used. 

Other Medicines: If corticosteroids and methotrexate are not effective, other drugs may be used. These medicines are used cautiously since their effect on sarcoidosis is not as well understood. They also can have side effects. These medicines can include:

• Humira® (adalimumab)
• Plaquenil® (hydroxychloroquine)
• Remicade® (infliximab)

Oxygen Therapy

Oxygen therapy may be an important part of a treatment plan for severe sarcoidosis. Oxygen can help reduce long-term heart and lung problems caused by low oxygen levels. Here are more resources about using oxygen.

Procedures

Permanent pacemaker: This device is placed during a minimally invasive procedure. It helps keep a regular heartbeat.

Internal cardiac defibrillator: This device notices abnormal heartbeats. When needed, it delivers a shock to restore a regular heartbeat. It is placed in the chest during surgery.

Pulmonary Rehabilitation

For people with chronic, progressive sarcoidosis, pulmonary rehabilitation can be helpful. This rehab program includes exercise, healthy eating and education.

Clinical Trials

Clinical trials help determine new treatment options for diseases and conditions. Patients with sarcoidosis have access to clinical trials and should speak with their doctor to determine what trials might work best for them.

Lifestyle Management

In addition to medical treatments, lifestyle changes can also help manage this condition. 

• Quit smoking
• Avoid lung irritants such as dust, air pollution, etc.
• Get regular exercise
• Maintain a healthy diet
• Get quality sleep 
• Manage stress
• Take calcium and vitamin D supplements (if recommended by your doctor)

Many people living with sarcoidosis improve over time. Others have chronic disease that needs long-term monitoring and treatment. Practical steps to living with this condition include:

• Keep follow-up appointments and testing
• Track symptoms
• Tell your doctor about any drug side effects
• Ask if routine eye exams is recommended for you. 

Learn more about living with sarcoidosis.  

When to See a Specialist

Since this condition mostly affects the lungs, if you or a loved one has symptoms or a family history of sarcoidosis, it is important to be evaluated by a pulmonologist who specializes in this disease.

At National Jewish Health in Denver, Colorado, we have one of the world’s leading sarcoidosis treatment programs. Learn more about our program or use the button below to make an appointment.