Sarcoidosis

• Illustrations
• Definition
• Causes, incidence, and risk factors
• Symptoms
• Signs and tests

• Treatment
• Expectations (prognosis)
• Complications
• Calling your health care provider
• References

Definition    Return to top

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    Return to top

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    Return to top

• 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:

• Tearing, decreased
• Seizures
• Nosebleed - symptom
• Joint stiffness
• Hair loss
• Eye burning, itching and discharge
• Rales or other abnormal breath sounds

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    Return to top

• 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    Return to top

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)    Return to top

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    Return to top

• 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    Return to top

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

References    Return to top

Murray JF, Nadel JA. Sarcoidosis. In: Textbook of Respiratory Medicine. 3rd ed.W. B. Saunders Company, 2000; 1717-1732.