Understanding Sarcoidosis

What is Sarcoidosis?

Sarcoidosis is an inflammatory disease that can affect almost any organ in the body. It most commonly starts in the lungs or lymph nodes. Although no one knows exactly what causes sarcoidosis, it is clear that the disease is characterized by the formation of granulomas which are small collections of tissue and inflammatory cells. Granulomas collect in various organs of the body and can lead to chronic inflammation and scarring.

Sarcoidosis and African American Women

Sarcoidosis is a relatively common disease affecting people of all ages and sexes. It usually affects people between the ages of 20 and 40, but it can occur in children and in the elderly.

In the United States, sarcoidosis is approximately 10-15 times more common in African Americans than Whites. African American females get sarcoidosis 2 times more commonly than African American males.

In Europe, the disease affects mostly Whites, with rates as high as 64 per 100,000 in Sweden.

Signs and Symptoms of Sarcoidosis

People with sarcoidosis present for medical attention with a variety of symptoms. Here are some of the more common symptoms reported: (please remember that these symptoms could be manifestations of many different illnesses).

• Shortness of breath.

• Cough that will not go away.

• Enlarged lymph nodes.

• Weight loss, night sweats, and/or fevers.

• Fatigue.

Clinical Manifestations

As stated above, sarcoidosis is a disease that can affect a number of organs in the body. Here is a list of some of the areas most commonly affected:

Lungs: The lungs are usually the first organ system involved. Patients often complain of cough and shortness of breath. 90% of people with sarcoidosis have an abnormal chest x-ray at some time during their illness.

Skin: Sarcoidosis involves the skin in about 25% of cases. A common lesion encountered is called Erythema Nodosum, which is characterized by tender red nodules on the anterior surface of the legs. Also seen are Skin Plaques which are purple, often raised lesions on the face, buttocks, and extremities.

Lymph Nodes: Enlarged lymph nodes (lymphadenopathy) are very common findings in sarcoidosis. Many of the lymph nodes inside of the chest are enlarged. Lymphadenopathy rarely causes a problem unless the amount is massive.

Sinuses: Up to 20% of patients experience nasal stuffiness and/or other symptoms of upper respiratory tract involvement.

Eye: Eye involvement also occurs in about 25% of patients and, without therapy, can lead to blindness. Other eye problems can include blurred vision, tearing and photophobia (bright light in the eyes is uncomfortable and/or painful).

Heart: Approximately 5% of patients have significant heart involvement.

Nervous System: All parts of the nervous system can be involved in sarcoidosis. About 5% of sarcoidosis patients experience nervous system involvement. Unilateral (one-sided) facial paralysis is the most common manifestation and has sometimes been misdiagnosed as Bell's Palsy or stroke.

Medical Evaluation and Diagnosis

Sarcoidosis is often a difficult diagnosis to make since the signs and symptoms of the disease mimic many common illnesses. Often, patients are diagnosed with a chronic cough or bronchitis and it is not until several months have passed and a series of tests are done until the diagnosis is made.

No blood tests are absolutely diagnostic or specific for sarcoidosis, although there are a couple of lab findings (elevated angiotensin-converting enzyme level and elevated urine calcium level) which are often found in association with the disease. The chest x-ray is often helpful in making the diagnosis of sarcoidosis. A finding of “bilateral hilar adenopathy” is the hallmark of sarcoidosis, but is occasionally seen in other illnesses.

The only certain way to diagnose sarcoidosis is with a biopsy of an affected organ, in conjunction with a careful examination of a person’s medical history, x-ray findings, and physical examination. During a biopsy, a small portion of tissue is taken from the body (often the lung, a lymph node or skin) and analyzed under the microscope. Often, lung biopsies are completed in conjunction with a lung bronchoscopy.

Often, however, doctors will make the diagnosis without having the patient undergo the biopsy in cases when the collection of findings is overwhelmingly suggestive of sarcoidosis.

Prognosis

The overall prognosis for people with sarcoidosis is quite good. The majority of individuals who are diagnosed with sarcoidosis recover completely and are not left with any significant impairments. About 50% of patients have some permanent organ damage, but for most this is mild and rarely progresses.

In approximately 15-20% of cases, the sarcoidosis remains active or recurs intermittently. In another 10% of those affected with sarcoidosis, the disease is extremely severe and can lead to death.

Treatment

Glucocorticoids are the mainstay of treatment for sarcoidosis and the majority of patients in the United States are treated with Prednisone, an oral steroid that is used for a variety of illnesses. The major question is not what to give to patients with sarcoidosis but when to give it. Some feel that unless a patient is experiencing severe, active pulmonary sarcoidosis that is safe to observe without therapy for 2 to 3 months. If the inflammation does not subside by itself, then therapy is started. Each case is different so it is important to ask your doctor about the risks and benefits of therapy in your particular case.

Empowerment Points

• Sarcoidosis is an inflammatory disorder of unknown cause that is found most commonly in the U.S. in African American women.

• This disease affects a variety of organs in the body including the lungs, skin, eyes and lymph nodes.

• Common symptoms of sarcoidosis include shortness of breath, persistent cough, enlarged lymph nodes, weight loss, fever and fatigue. (These symptoms are not specific to sarcoidosis, however).

• Sarcoidosis can sometimes be difficult to diagnose because its presentation is similar to several other illnesses.

• If you are diagnosed with sarcoidosis, don’t give up!! Most people do quite well and recover without significant long-term problems.

Resources

1. National Sarcoidosis Resource Center www.nsrc-global.net

2. World Sarcoid Society www.worldsarcsociety.com

References

1. National Institutes of Health (N.I.H.) www.nhlbi.nih.gov (look under health topics).

2. Harrison’s Principles of Internal Medicine, 13^th Edition.