Health Encyclopedia

Sarcoidosis

Definition

Sarcoidosis is a disease of unknown cause in which inflammation occurs in the lymph nodes, lungs, liver, eyes, skin, or other tissues.

Causes, incidence, and risk factors

The cause of the disease is unknown. Sarcoidosis is marked by abnormal inflammatory masses (granulomas) in certain organs of the body. Granulomas are clusters of immune cells (macrophages, lymphocytes, and multinucleated giant cells). The disease can affect almost any organ of the body, although it most commonly affects the lungs. Sarcoidosis can be acute, subacute, or chronic.

Possible causes of sarcoidosis include:

Hypersensitivity to environmental factors
Genetics
Extreme immune response to infection

The incidence varies widely according to race and sex.

It is more common in African Americans than Caucasians. Females are usually affected more frequently than males. Onset of the disease typically occurs between the ages of 20 and 40. Sarcoidosis is very rare in young children.

Symptoms

General discomfort, uneasiness, or ill feeling (malaise)
Fever
Shortness of breath
Cough
Skin lesions
Skin rash
Headache
Visual changes
Neurological changes
Enlarged lymph glands (armpit lump)
Enlarged liver
Enlarged spleen
Dry mouth
Fatigue (one of the most common symptoms in children)
Weight loss (one of the most common symptoms in children)

Additional symptoms of this disease:

Note: There may be no symptoms. Most of the time, the disease is found in patients with no symptoms who have an abnormal chest x-ray.

Signs and tests

CBC
Chem-7 or chem-20
ACE levels
Chest x-ray to see if the lungs are involved or lymph nodes are enlarged
CT scan
Lymph node biopsy
Skin lesion biopsy
Bronchoscopy
Open lung biopsy
Liver biopsy
Kidney biopsy
EKG to see if the heart is involved

This disease may also alter the results of the following tests:

Quantitative immunoglobulins (nephelometry)
PTH
Serum phosphorus
Nerve biopsy
Mediastinoscopy with biopsy
Lung gallium (Ga.) scan
Immunoelectrophoresis - serum
Calcium - urine
Calcium - ionized
Calcium - serum
Liver function tests

Treatment

Sarcoidosis symptoms often resolve on their own gradually without treatment.

Severely affected patients may need treatment with corticosteroids. Therapy may continue for one or two years. Some of the most severely affected patients may require life-long therapy.

Immunosuppressive agents, such as methotrexate, azathioprine, and cyclophosphamide, are sometimes used in addition to corticosteroids. Rarely, some individuals with irreversible organ failure require an organ transplant.

Expectations (prognosis)

Many people are not seriously ill, and the disease may resolve without treatment. About 30 - 50% of cases resolve without treatment in 3 years. About 20% of those with lung involvement will develop lung damage. Death from sarcoidosis is rare.

Complications

Diffuse interstitial pulmonary fibrosis
Pulmonary hypertension
Anterior uveitis
Glaucoma and blindness (rare)
Cardiac arrhythmias
Cranial or peripheral nerve palsies
Kidney stones
Organ failure, leading to the need for a transplant

Calling your health care provider

Call your health care provider if you have difficulty breathing, vision changes, palpitations, or other symptoms of this disorder.

References

Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa:Saunders; 2003.

Cox CE, Donohue JF, Brown CD, Kataria YP, Judson MA. Health-related quality of life of persons with sarcoidosis. Chest. March 2004;125:997-1004.

Cox CE, Davis-Allen A, Judson MA. Sarcoidosis. Med Clin North Am. July 2005;89:817-828.

Review Date:3/1/2007
Reviewed By:David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.

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