STRONGER THAN SARCOIDOSIS

Learning that you have sarcoidosis can be scary. Learning to live with the diagnosis -- to manage not only the medical components, but the emotional toll it can take on literally every aspect of life -- can be especially daunting. That's why we're here - to share our experience, and to help make yours a little easier. You are not alone.

Welcome to Stronger Than Sarcoidosis

AS PER CLEVELAND CLINIC
What is Sarcoidosis? 
Sarcoidosis is an inflammatory disease that affects one or more organs but most commonly affects the lungs and lymph glands. As a result of the inflammation, abnormal lumps or nodules (called granulomas) form in one or more organs of the body. These granulomas may change the normal structure and possibly the function of the affected organ(s).

How is Sarcoidosis Diagnosed? ​
Because the symptoms and laboratory findings associated with sarcoidosis can occur in other diseases, there is no single test that can diagnose it. However, the classic sign of the disease is the formation of granulomas (abnormal masses or nodules consisting of inflamed tissue) in one or more of the major organs of the body. Sarcoidosis-related granulomas are not different from granulomas that occur in other diseases. As a result, a complete physical exam and medical history – including occupational history, medication history, and environmental exposures – must be made before concluding that the illness is, in fact, sarcoidosis. What types of tests are used to diagnose sarcoidosis? The main tools your doctor will use to diagnose sarcoidosis include:
Chest X-rays:
This test provides a picture of the lungs, heart and surrounding lymph nodes, and reveals where infection-fighting white blood cells have formed – often, the first indication of sarcoidosis. An X-ray can also show how much of the lungs are affected by the disease. Chest X-ray findings fall into one of the following 5 patterns described in the chart below. It is important to know that these X-ray patterns do not represent disease stages (in other words, they are not the sequential steps in the disease course). The categories simply allow doctors to classify the "types of disease." Chest X-ray patterns 0 = Normal: chest X-ray. I = Bilateral hilar lymphadenopathy (BHL): the X-ray shows an equal degree of enlargement of lymph nodes at the “root” of both sides of the lungs. This is a common presentation of sarcoidosis. II = BHL plus pulmonary infiltrations: the X-ray shows a disease process as described above with expansion into and throughout additional lung tissue. III = Pulmonary infiltration only (without BHL): the X-ray shows a disease process that is spread throughout the lung tissue (with no enlargement of lymph nodes). IV = Pulmonary fibrosis: the X-ray shows small lung fields, scarring, and “retraction” of both hila (the area at the “root” of the lungs). This type of disease is the most severe or permanent form of the disease.
Bronchoscopy:
Bronchoscopy involves passing a small tube (bronchoscope) down the trachea (windpipe) and into the bronchial tubes (airways) of the lungs. The purpose of this test is to inspect the bronchial tubes and to extract a biopsy (a small tissue sample) to look for granulomas, and to rule out infection. Bronchoscopy is a safe, low risk, outpatient procedure that provides your doctors with a good chance of making an accurate diagnosis. To prepare for this test, you will be asked not to eat or drink anything by mouth for 8 hours before the exam. Prior to the start of the exam, medications will be given to you to help you relax. Because these medications can make you groggy, an adult who can drive you home must accompany you. You will not be allowed to drive. The procedure usually lasts 15 to 45 minutes, with several additional hours for recovery. Your doctor may perform a bronchoalveolar lavage (washing out the air sacs), biopsies of the air sacs or airway wall using a forceps, or biopsy of a lymph node using a needle that is inserted through the bronchoscope. Guidance of the needle with an ultrasound probe attached to the bronchoscope (endobronchial ultrasound or EBUS) can almost always lead to a diagnosis of sarcoidosis when it is present. CT scan: This test is another form of X-ray that provides an even more detailed look at the lungs and lymph glands than that provided by a routine chest X-ray. This test does not hurt and simply involves lying on a table for about 10 minutes.
Mediastinoscopy:
This is a surgical procedure that involves a small incision at the base of the neck through which an instrument is passed to biopsy lymph nodes in the chest cavity. This test is performed under general anesthesia in the operating room of a hospital and takes 1 to 2 hours (same day procedure). The need for mediastinoscopy to make a diagnosis has gone down dramatically as EBUS-guided biopsies have become widespread.
Pulmonary function (breathing) tests:
These tests measure how well the lungs are working (expanding and exchanging oxygen and carbon dioxide in the blood). One pulmonary function test uses a device called a spirometer. This device records the changes in air flow as a person inhales and exhales, as well as the overall volume of air exhaled. The development of granulomas and fibrosis of the lung tissue stiffen the lung tissue and destroy the air sacs, making it more difficult for the lungs to perform these tasks.
Other biopsies:
In addition to the bronchoscopic biopsy or lymph node biopsy by mediastinoscopy, tissue samples can be taken from any other involved site including other lymph nodes, skin, and other sites to determine where granulomas have formed.
Blood tests:
Blood analyses evaluate the number and types of blood cells and blood proteins in the body, and how well the cells are functioning. They also track increases in calcium levels and abnormal liver function that sometimes accompany sarcoidosis. One blood test measures a substance called angiotensin-converting enzyme (ACE), which is secreted in large amounts by cells that make up granulomas. ACE levels, however, are not always high in sarcoidosis patients, and increased ACE levels can also show up in other illnesses. In short, there is no specific blood test to diagnose sarcoidosis. A newer blood test that is sometimes more useful than ACE is a measurement of the soluble interleukin 2 receptor levels (sIL2R).
Pulse oximetry:
This test measures the amount of oxygen in the blood by way of a sensor attached to a patient’s finger. If the oxygen level is low, your doctor may recommend the use of supplemental oxygen.
Electrocardiogram (EKG or ECG):
This is a routine office test that checks the electrical activity of the heart. For this test, electrodes with adhesive pads are attached to the skin of the patient’s chest, arms, and legs. The EKG machine creates a picture, on graph paper, of the electrical impulses traveling through your heart. This screening test helps doctors detect several abnormalities in the heart rhythm.
PET Scan:
In this test, a small amount of radioactive material called F-fluorodeoxyglucose is injected into a vein. This substance collects in the areas where the granulomas have collected. A scanner then detects and records the location and amount of inflammation in the body. This scan will detect inflammation created by conditions other than sarcoidosis so it will be used in combination with other testing.
Gallium scanning:
In this procedure, the radioactive chemical gallium-67 is injected into a vein. The gallium collects in inflamed body tissue. A scan of the body then indicates which tissues and how much tissue is affected. The scan will reveal any type of inflammation occurring in the body, however, and does not necessarily mean the patient has sarcoidosis. Because of this test’s limitations, it is not commonly performed.
Purified protein derivative:
This is a type of skin test that is used to help establish prior exposure or infection with tuberculosis (TB). Since TB is sometimes confused with sarcoidosis, this simple test is frequently performed. In sarcoidosis, this skin test is usually negative or non-reactive.
Slit-lamp examination:
This examination looks at the inside of the eye and is used to detect eye-related problems caused by sarcoidosis.

Sarcoidosis Treatments 

How is sarcoidosis treated?

There is no cure for sarcoidosis, but the disease may get better on its own over time. Many people with sarcoidosis have mild symptoms and do not require any treatment at all. Treatment, when it is needed, generally falls into two categories—maintenance of good health practices and drug treatment.
Good health practices include:
Getting regular check-ups with your health care provider.
Eating a well-balanced diet with a variety of fresh fruits and vegetables.
Drinking 8 to 10 8-ounce glasses of water a day.
Getting 6 to 8 hours of sleep each night. Exercising regularly, and managing and maintaining your weight.
Quitting smoking.
Avoiding exposure to dust, chemicals, fumes, gases, toxic inhalants and other substances that can harm your lungs.
Avoiding excessive amounts of calcium-rich foods (such as dairy products, oranges, canned salmon with bones), vitamin D and sunlight. Daily sunbathing is an example of excessive sunlight and should be avoided; sunlight received from activities of everyday living is acceptable. (The advice in this bullet point is limited to patients with high blood or urine levels of calcium.)

Drug treatments are used to relieve symptoms, reduce the inflammation of the affected tissues, reduce the impact of granuloma development, and prevent the development of lung fibrosis and other irreversible organ damage.

Corticosteroids

Are particularly effective in reducing inflammation, and are typically the first drugs used in treating sarcoidosis. The oral corticosteroid prednisone is the most commonly used corticosteroid.

For patients with no symptoms or very mild symptoms, the side effects of prednisone therapy may outweigh possible benefits, so treatment is usually not recommended for this disease stage. Corticosteroids are more typically reserved for patients with disease that is of moderate severity. Symptoms, especially cough and shortness of breath, generally improve with steroid therapy.

Corticosteroid treatment controls the disease rather than cures it. The symptoms respond to treatment in the majority of patients. A relatively high dose is usually prescribed at first, followed by a slow taper to the lowest effective dose. Fortunately, disease relapses --when they occur -- usually respond to retreatment with steroids. Patients who improve and remain stable for more than one year after stopping treatment have a low rate of relapse.

Results of some long-term studies indicate patients can expect about a 10% improvement in symptoms up to five years after quitting corticosteroid treatment. Whether or not this is a large enough benefit to outweigh the risks of corticosteroid treatment remains an issue debated by doctors. If steroids are prescribed, the patient should see his or her doctor at regular intervals so that the disease and side effects of treatment can be monitored.

The common side effects of corticosteroids include:

Excessive weight gain.
Insomnia.
Acne.
Diabetes in susceptible people.
High blood pressure.
Glaucoma.
Cataracts.
Osteoporosis.
Depression and emotional irritability.
Skin bruising.
Increased risk of infections.

Are there alternative treatments to corticosteroids?
Other treatments are available for patients who cannot tolerate steroids either because they are contraindicated or because side effects cannot be tolerated. Patients whose disease does not respond to steroids or who wish to lower the dose of steroids and use another drug in combination have additional treatment options as well. It's important to keep in mind that all of the following treatments have been used and studied much less extensively than corticosteroids. Doctors with special expertise in sarcoidosis should manage patients who are on regular prednisone therapy or any of the following alternatives:
Methotrexate, leflunomide or azathioprine:
These medications, most often used in rheumatoid arthritis, have been used in place of or in addition to corticosteroids to treat pulmonary sarcoidosis and chronic sarcoidosis. Methotrexate can be taken as pills or an injection under the skin, once per week. All of these medications can suppress the immune system and have the potential to cause liver toxicity so blood work needs to be monitored every 4-8 weeks. In addition, these drugs have the potential to harm a fetus (unborn child) so pregnancy needs to be avoided. Patients who take methotrexate must not drink alcohol.
Hydroxychloroquine and chloroquine:

These oral antimalarial drugs have been used to treat sarcoidosis of the skin, lungs, and nervous system. They are used to treat the hypercalcemia seen with sarcoidosis. Patients on these drugs need occasional monitoring of their eyes by an ophthalmologist.
Cyclophosphamide or chlorambucil:

These medications are usually used for disease that has reached the severe stage and after other therapies have failed. Cyclophosphamide is associated with many severe side effects including bone marrow suppression and kidney damage.
Pentoxifylline and thalidomide:

Recent studies have reported beneficial effects of these drugs in treatment-resistant lupus pernio.
Infliximab or adalimumab:

These medications, which are given as an intravenous injection every 4-8 weeks or under the skin (subcutaneous injection) every 1-2 weeks, have been used recently for patients with severe sarcoidosis. They are usually used after other options are not successful or not tolerated. They significantly increase the risk for infections.
Various NSAIDs (nonsteroidal anti-inflammatory drugs [such as ibuprofen or aspirin]):

These drugs may help reduce acute inflammation and relieve arthritis and fever. Topical corticosteroids: These agents could be used in several preparations (for example, eye drops, skin creams, and respiratory sprays) for mild local symptoms of sarcoidosis. Although they are a lot safer than steroid pills, they are also less effective.
Organ transplantation:

This option is rarely considered in patients with end-stage disease, where the kidneys or lungs have failed.
When is treatment started?

Many questions exist regarding the appropriate timing and duration of treatment for sarcoidosis. The decision to begin treatment generally depends on the organ system involved and the severity of disease.
There are several situations, however, under which some form of treatment is usually given. These include patients with neurological, heart, and sight-threatening disease; those with serious pulmonary symptoms and/or worsening lung function; and those with kidney involvement--specifically, hypercalcemia. Because of the serious effects that can occur when these systems are involved, treatment is started even if symptoms are mild.
Other indications for which treatment could be considered include an inability to work as a result of fever, weakness, fatigue, joint pain, nervous system changes, respiratory symptoms (especially shortness of breath and cough), and disfiguring skin disease.

What can happen as the disease progresses?

In many people with sarcoidosis, the disease appears briefly and then disappears without the person even knowing they have the disease. When sarcoidosis seriously affects the ability of the lungs to function normally, patients may require supplemental oxygen (supplied in a small portable oxygen tank and administered through plastic tubing clipped to the nose) to help them breathe.
Twenty percent to 30% of people have some permanent lung damage. For 10% to 30%, sarcoidosis is a chronic condition, with symptom progression despite treatment that has continued for more than two years. In some people, the disease may result in the deterioration of the affected organ.

When the granulomas or fibrosis seriously affect the function of a vital organ -- such as the lungs, heart, nervous system, liver, or kidneys -- sarcoidosis can be fatal. Death occurs in 1% to 6% of all patients with sarcoidosis and in 5% to 10% of patients with chronic progressive disease. The leading cause of sarcoidosis-related death in the United States is irreversible pulmonary fibrosis.

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OUR MISSION

Our mission is to raise awareness, advocate, and educate about this rare disease called sarcoidosis. We aim to strengthen the sarcoidosis community with information, support, connection, and interactive events that focus on the physical, mental, and emotional aspects of living with this disease.
Stronger Than Sarcoidosis was created by sarcoidosis patients, for sarcoidosis patients, and we will always be patient-centered. We aim to support the sarcoidosis community, ensuring that all sarcoidosis patients know they are not alone. Together, we are Stronger Than Sarcoidosis.

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