Sarcoidosis is a chronic disease of unknown cause in which there is accumulation of inflammatory cells in various parts of the body. Most commonly affected organ is the lung. But other organs like skin, eyes, liver, and lymph nodes may also be involved. The normal architecture of these affected organs are destroyed and replaced by white blood cells like lymphocytes and monocytes and granulomas.
This disease affects young adults, more females than males. Sometimes there is a genetic component to it, and it has been described in all races throughout the world.
Symptoms
Symptoms can be acute or chronic. In some people, there may not be any symptoms. In acute forms of sarcoidosis, symptoms occur in one to two weeks.
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Fever
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Fatigue
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Malaise
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Anorexia or weight loss
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Respiratory symptoms include cough, shortness of breath, and vague chest discomfort behind the sternum or breastbone.
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Arthritis involving multiple joints
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Erythema nodosum or purplish raised nodules on face, skin, buttocks
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Enlargement of lymph nodes throughout the body
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Parotid enlargement
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Paralysis of the facial nerve (rare)
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Blurred vision
Chronic symptoms develop over months and maybe only associated with chronic cough.
Affects of the Disease
1) Lung-permanent damage of the lung tissue leading to fibrosis. Alveoli, bronchi and small blood vessels are all affected
2) Eyes- 25% percent of all cases, eyes are affecting. Blurred vision and photophobia may be present because of uveitis. Retinal vasculities may be present, conjunctival nodules may be seen, and there can be involved of the lacrimal gland, causing dry eyes.
3) Upper respiratory tract- 20% of cases have such symptoms. Nasal stuffiness, tonsilar enlargement, hoarseness of voice because of laryngeal involvement, difficulty in breathing, and airway obstruction can be seen.
4) Spleen- May be enlarged in 40% of cases.
5) Liver- 60-90% of cases, liver is enlarged. Cirrhosis can develop.
6) Central nervous system- 5% of cases have CNS involvement. Facial paralysis, optic nerve dysfunction, meningitis and rarely, seizures and psychiatry disturbances may be seen.
7) Musculoskeletal system- Bones, joints and muscles are involved in 3-13% of cases. Cysts may be seen in bones. Arthritis of the large joints may be present.
8) Heart-5% are affected. Left ventricle is involved. Irregular heart beat, pericarditis, and heart failure are all possible.
Diagnosis
1) History, and physical examination
2) X-ray of the chest may show large lymph nodes
3) Pulmonary function tests
4) Blood tests may show an increased calcium level or increased ACE level.
5) Gallium 67 scan
6) Bronchoscopy
7) Bronchial lavage
8) Tissue biopsy
Treatment
Ninety percent of cases resolve without any treatment. Steroids are recommended. Other drugs that are used are immunosuppressants such as methotrexate, azathioprine. These drugs have major side effects. Skin and nervous system symptoms are treated with hydroxyquinone.
Complications
1) Respiratory failure
2) Eye – blindness, which is rare
3) Cardiac- sudden death secondary to irregular heart beat
Prognosis when treated is good. Most cases are completely cured. Twenty percent remain chronic and 10% die. |