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:

• Tearing, decreased
• Seizures
• Nosebleed - symptom
• Joint stiffness
• Hair loss
• Eye burning, itching, and discharge
• Abnormal breath sounds (such as rales)

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.

Support Groups

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.

Prevention

Images

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.

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