Sarcoidosis Information - Symptoms and It's Treatment

Sarcoidosis (sar"koi-do'sis) involves inflammation that produces tiny lumps of cells in various organs in your body. The lumps are called granulomas (gran"u-lo'mahs) because they look like grains of sugar or sand. They are very small and can be seen only with a microscope.

Sarcoidosis is a multisystem, granulo, matous disorder that characteristically produces lymphadenopathy, pulmonary infiltration, and skeletal, liver, eye, or skin lesions. It occurs most commonly 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.

Pulmonary sarcoidosis can develop into pulmonary fibrosi, which distorts the structure of the lungs and can interfere with breathing. Bronchiectasis, a lung disease in which pockets form in the air tubes of the lung and become sites for infection, can also occur .

What causes Sarcoidosis?

The cause of sarcoidosis is unknown. but the following factors may playa role:

• hypersensitivity response (possibly from 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 and beryllium, that can lead to illnesses resembling sarcoidosis, suggesting an extrinsic cause for this disease.

Signs and symptoms of Sarcoidosis

Initial symptoms 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 the fibrosis:

• respiratory - breathlessness, cough (usually nonproductive), substernal pain; pulmonary hypertension and cor pulmonale (complications in advanced pulmonary disease)
• Cutaneous - erythema nodosum, subcutaneous skin nodules with maculopapular eruptions and extensive nasal mucosal lesions
• ophthalmic - anterior uveitis (common), glaucoma, and blindness (rare)
• lymphatic - bilateral hilar and right paratracheal lymphadenopathy and splenomegaly
• musculoskeletal - muscle weakness, polyarthralgia, pain, and punched-out lesions on phalanges
• hepatic - granulomatous hepatitis. which usually produces no symptoms . genitourinary - hypercalduria
• cardiovascular - arrhythmias (premature beats, bundle-branch or complete heart block) and rarely, cardlomyopathy
• central nervous system (CNS) -cranial or peripheral nerve palsies, basilar meningitis, seizures, and pituitary and hypothalamic lesions producing diabetes insipidus.

Diagnosis information

Diagnosis of sarcoidosis is one of exclusion. Once differential diagnoses are excluded. other tests may be ordered. Typical clinical features with appropriate laboratory data and X-ray findings suggest sarcoidosis. A positive Kveim skin test supports the diagnosis. In this test. the patient receives an intradermal injection of an antigen prepared from human sarcoidasis spleen or lymph nodes from patients with sarcoidosis. If the patient has active sarcoidosis, granuloma develops at the injection site in 2 to 6 weeks. This reaction is considered positive when a biopsy of the skin at the injection site shows discrete epithelioid cell granuloma.

Other relevant findings include:

• chest X-ray - bilateral hilar and right paratracheal adenopathy with or without diffuse interstitial infiltrates and occasionally, large nodular lesions present in lung parenchyma.
• lymph node, skin, or lung biopsy noncaseating granulomas with negative cultures for mycobacteria and fungi
• other laboratory data - rarely, increased serum caldum, mild anemia, leukocytosis, and hyperglobulinemia
• pulmonary function tests - decreased total lung capacity and compliance and decreased diffusing capacity
• arterial blood gas (ABG) analysis decreased arterial oxygen saturation.

Negative tuberculin skin test, fungal serologies, and sputum cultures for mycobacteria and fungi as well as negative biopsy cultures help rule out infection.

Treatment of Sarcoidosis

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 measures include a low-calcium diet and avoidance of direct exposure to sunlight in patients with hypercalcemia.

Special considerations and Prevention

• Watch for and report complications. Be aware of any abnormal laboratory results (anemia, for example) that could alter patient care.
• For the patient with arthralgia, administer analgesics as ordered. Record signs of progressive muscle weakness.
• Provide a nutritious, high-calorie diet and plenty of fluids. If the patient has hypercalcemia, suggest a low calcium diet. Weigh the patient regularly to detect weight loss.
• Monitor respiratory function. Check chest X-rays for the extent of lung involvement; note and record any bloody sputum or increase in sputum. If the patient has pulmonary hypertension or end-stage cor pulmonale, check ABG levels, observe for arrhythmias, and administer oxygen as needed.
• Because steroids may induce or worsen diabetes mellitus, perform finger stick glucose tests at least every 12 hours at the beginning of steroid therapy. Also, watch for other steroid adverse effects, such as fluid retention, electrolyte imbalance (especially hypokalemia), moon face, hypertension, and personality change. Remember that the patient on long-term or high dose steroid therapy is vulnerable to infection.
• During or after steroid withdrawal (particularly in association with infection or other types of stress), watch for and report vomiting, orthostatic hypotension, hypoglycemia, restlessness, anorexia, malaise, and fatigue.
• When preparing the patient for discharge, stress the need for compliance with prescribed steroid therapy and regular, careful follow-up examinations and treatment. Refer the patient with failing vision to community support and resource groups and the American Foundation for the Blind, if necessary.