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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 the pediatric population)
Weight loss (one of the most common symptoms in the pediatric population)

Additional symptoms that may be associated with this disease:

Note: There may be no symptoms. Most of the time, the disease is found in asymptomatic patients 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)
Serum calcium
Liver function tests

Treatment

Sarcoidosis symptoms often gradually resolve on their own without treatment.

Severely affected patients may require treatment with corticosteroids. Therapy may continue for one or two years, although some of the most severely affected 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 organ transplantation.

Expectations (prognosis)

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

Complications