What is Sarcoidosis: Symptoms and Treatments

April is Sarcoidosis Awareness Month

The Foundation for Sarcoidosis Research  defines sarcoidosis as “an inflammatory disease characterized by the formation of granulomas – tiny clumps of inflammatory cells – in one or more organs of the body.”   Up to four in 10,000 people in the United States have sarcoidosis.

Symptoms of Sarcoidosis

There is no known cause of sarcoidosis, though some research shows it may be a type of autoimmune disease. Still, it is unknown what triggers the abnormal immune response.  Symptoms of sarcoidosis vary with the extent and severity of organ involvement.  For example, symptoms in the lungs include a persistent cough and shortness of breath and/or tightness/pain in the chest.  Other symptoms of sarcoidosis in other parts of the body may include fever, loss of appetite, skin rashes, red bumps on the shins, arms and/or face, and inflammation of the eyes.  A general feeling of being unwell may also be present.

Up to 30% of patients with sarcoidosis have eye disease, with the sarcoidosis affecting the membranes of the eye lids, cornea, retina and lens. Another 20-35% patients experience sarcoidosis of the skin, with raised patches of bumps on the legs, face, arms, and/or buttocks.  Arthritis in the elbows, hands, wrists, and ankles often accompanies the red bumps.

Sarcoidosis is more common in women than in men, and usually occurs between the ages of 20 and 40 years of age. For unknown reasons, it is more common in African-Americans, as well as those of Puerto Rican, Irish, German and Scandinavian origin.

If you suspect you may have this disease or are experiencing any of the symptoms listed above, you must speak to your physician and discuss the signs and symptoms you are experiencing in order to determine a clear diagnosis.

Diagnosing Sarcoidosis

Sarcoidosis is often difficult to diagnose because it can present itself as other diseases.  Your doctor will order tests like these to help diagnose sarcoidosis:

• Chest x-ray – will show swollen lymph nodes and cloudiness in the lungs.
• HRCT scan - for a detailed picture of the lymph nodes and lungs.
• Bronchoscopy – to extract a biopsy to examine for granulomas.
• Pulmonary function test – to measure your lung function.

There are other tests your doctor and/or team of specialists will possibly take to ensure a clear diagnosis and to help determine your course of treatment.

Treatment of Sarcoidosis

Symptoms of sarcoidosis may disappear on their own and, fortunately, most patients do not need treatment.  Once the symptoms go away the disease is considered inactive. Nonsteroidal anti-inflammatory drugs (NSAIDS) like ibuprofen and corticosteroid drugs, like prednisone, are the primary treatment for the granuloma formation and inflammation. Ultimately the treatment plan depends on which organ system is involved and the level of inflammation and discomfort.  Prednisone will not cure established scarring in the lungs, but it may help improve cough symptoms and improve pulmonary function tests.

Antimetabolites, also categorized as steroid-sparing agents, such as azathioprine, methotrexate, mycophenolate mofetil, and leflunomide may be used as successful alternatives to prednisone. 

Anitmalarial drugs such as hydroxychloroquine and chloroquine are sometime prescribed for this condition.  They were originally used to treat malaria – and still are used for this purpose today.

Drug therapy may also include immunosuppressants (like cyclophosphamide, cladribine, chlorambucil, and cyclosporine), immunomodulatory (pentoxifylline and thalidomide), and anti-tumor necrosis factor treatment (injectables such as infliximab, etanercept, golimumab, and adalimumab) and some other oral agents (e.g., ursodeoxycholic acid (Urso®), apremilast (Otezla®)). 

Sarcoidosis Facts

You may have a lot of questions about what life with sarcoidosis is like, so here are some facts to help you better understand this disease.

• Sarcoidosis is not contagious.
• Sarcoidosis is not a form of cancer.
• Skin rashes from sarcoidosis are not contagious.
• About 60% of people with sarcoidosis recover completely without treatment, while 30% may have persistent sarcoidosis that may need treatment.  Only 10 % with progressive sarcoidosis have serious organ and tissue damage.
• Approximately one in ten sarcoidosis patients have high blood calcium levels.  Check with your doctor to see if you need to reduce the amount of calcium rich foods you eat.

Do you think you may have sarcoidosis?

Sarcoidosis is difficult to diagnose because it does look like so many other diseases at first glance.  It is important to speak with your doctor about sarcoidosis and ask to be referred to a specialist for further diagnosis and treatment.  Your treatment may include several specialists because sarcoidosis may involve other organs of the body.  Remember to make regular appointments for check-ups with your doctors, as sarcoidosis may disappear and reappear over time.

To learn more about sarcoidosis, please visit the Foundation for Sarcoidosis Research at www.stopsarcoidosis.org.