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Sarcoidosis - Symptoms & Treatment

A multisystemic, granulomatous disorder, sarcoidosis characteristically produces lymphadenopathy, pulmonary infiltration, and skeletal, liver, eye, or skin lesions. It occurs most often in young adults (ages 20 to 40). In the United States, sarcoidosis occurs predominantly among blacks and affects twice as many women as men.

Acute sarcoidosis usually resolves within 2 years. Chronic, progressive sarcoidosis, which is uncommon, is associated with pulmonary fibrosis and progressive pulmonary disability.

Sarcoidosis remains something of a medical mystery, but it's believed to result from an abnormal immune response. Just what triggers this response isn't known. Doctors do know that the disease often goes away on its own without treatment - usually within 2 to 3 years. Some people may have symptoms for a lifetime, however. And in rare cases sarcoidosis can be fatal.

If you have no signs or symptoms of sarcoidosis or only minor ones, your doctor may choose to take a wait-and-see approach. Treatment for severe cases is usually with the corticosteroid drug prednisone.

Causes

Although the cause of sarcoidosis is unknown, the following possible causes have been considered:

• hypersensitivity response (possibly from a T-cell imbalance) to such agents as atypical mycobacteria, fungi, and pine pollen
• genetic predisposition (suggested by a slightly higher incidence of sarcoidosis within the same family)
• chemicals, such as zirconium or beryllium, can lead to illnesses resembling sarcoidosis, suggesting an extrinsic cause for this disease.

Signs and symptoms

Initial signs of sarcoidosis include arthralgia (in the wrists, ankles, and elbows), fatigue, malaise, and weight loss. Other clinical features vary according to the extent and location of fibrosis:

• respiratory- breathlessness, cough (usually nonproductive), substernal pain; complications in advanced pulmonary disease include pulmonary hypertension and cor pulmonale
• cutaneous- erythema nodosum, subcutaneous skin nodules with maculopapular eruptions, extensive nasal mucosal lesions
• ophthalmic-anterior uveitis (common); glaucoma, blindness (rare)
• lymphatic-bilateral hilar and right paratracheal lymphadenopathy and splenomegaly
• musculoskeletal- muscle weakness, polyarthralgia, pain, punched-out lesions on phalanges
• hepatic-granulomatous hepatitis, usually asymptomatic                                
• genitourinary- hypercalciuria
• cardiovascular-arrhythmias (premature beats, bundle branch block, or complete heart block); cardiomyopathy (rare)
• central nervous system (CNS) - cranial or peripheral nerve palsies, basilar meningitis, seizures, pituitary and hypothalamic lesions producing diabetes insipidus.

Diagnosis

During a physical exam, physicians check for:

• Enlarged, tender lymph glands.
• Dry cough
• Erythema Nodosum (redness of the skin)
• Eye problems

Tests include:

• Biopsies of skin lesions, lymph nodes or lungs
• Chest X-ray
• Blood tests

Treatment

Asymptomatic sarcoidosis requires no treatment. However, sarcoidosis that causes ocular, respiratory, CNS, cardiac, or systemic symptoms (such as fever and weight loss) requires treatment with systemic or topical steroids, as does sarcoidosis that produces hypercalcemia or destructive skin lesions. Such therapy is usually continued for 1 to 2 years, but some patients may need lifelong therapy.

Other treatment includes a low-calcium diet and avoidance of direct exposure to sunlight in patients with hypercalcemia.

Prevention

Many people with sarcoidosis recover completely. Others have only minor symptoms. But some people have more severe problems, including damage to their lungs or other organs. These suggestions may help make it easier to live with sarcoidosis:

• Protect your lungs. To help keep your lungs healthy, don't smoke, and avoid contact with chemicals, dust and other irritants.
• Get regular checkups. If you've received a diagnosis of sarcoidosis, see your doctor often so that your condition can be monitored.
• Maintain a strong support system. A number of organizations and support groups exist to help people cope with sarcoidosis. You might want to consider joining a sarcoidosis support group to share your experiences, feelings and concerns. Although support groups aren't for everyone, they can be a good source for practical information as well as for encouragement and understanding. Your doctor or local chapter of the American Lung Association can help you find a sarcoidosis support group in your community.