sarcoidosis(redirected from Besnier Boeck disease)
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Causes and symptoms
- tender reddish bumps (nodules) or patches often appear on the skin
- the eyes may become red and teary, and the vision blurry
- the joints may become swollen and painful (arthritis)
- lymph nodes in the neck, armpits, and groin become enlarged and tender, lymph nodes within the chest, around the lungs, also become enlarged
- fluid may accumulate around the lungs (pleural effusion), making breathing increasingly difficult
- nasal stuffiness is common, as well as a hoarse sound to the voice
- cysts in the bone may cause pain in the hands and feet, or in other bony areas
- the bone marrow may decrease the production of all blood cells; decreased number of red blood cells causes anemia, fewer white blood cells increases the chance of infections, fewer platelets can increase the chance of bleeding
- the body's ability to process calcium often becomes abnormal, so that excess calcium passes through the kidneys and into the urine; this may cause kidney stones to form
- the liver may become enlarged
- the heart may suffer a variety of complications, including abnormal or missed beats (arrhythmias), inflammation of the covering of the heart (pericarditis), and an increasing tendency toward weak, ineffective pumping of the blood (heart failure)
- the nervous system may display the effects of sarcoidosis by hearing loss, chronic inflammation of the coverings of the brain and spinal cord (meningitis), abnormalities of the nerve that is involved in vision (optic nerve dysfunction), seizures, and the development of psychiatric disorders
As there is no specific test for sarcoidosis, diagnosis is based on clinical examination, x-rays, fiberoptic bronchoscopy, gallium-67 scan, serum angiotensin-converting enzyme, and a skin test called the Kveim test.
Blood studies may reveal blood dyscrasias such as anemia, leukopenia, eosinophilia, hypergammaglobulinemia, and a reduction in serum albumin. In a small percentage of patients there may be a transient hypercalcemia and hypercalciuria in the early stages of the disease. Patients have a delayed skin reactivity to tuberculin and other antigens such as candidiasis and mumps.
Patients are encouraged to keep appointments for follow-up medical evaluations. For self-monitoring they are taught symptoms to report such as shortness of breath, excessive tearing and inflamed eyes, chest pain, swollen joints, and an increased sense of being tired and listless. An irregular pulse rate or one that is under 50 or over 120 beats per minute should also be reported. Persons who do not know how to take their own pulse are taught how to do it. Periodic ophthalmologic examinations are imperative because of the possibility that sarcoidosis can affect the eye and cause uveitis, iritis, glaucoma, and cataracts.
Those receiving steroid therapy are taught the use and side effects of their medication. They are cautioned not to stop taking the prescribed drug, because this can produce a drop in blood pressure with fever, nausea, vomiting, and diarrhea.
Smoking is harmful because it aggravates impaired lung function; hence patients are encouraged to seek help if they need to quit smoking. Prolonged exposure to direct sunlight should be avoided because vitamin D aids absorption of calcium, which can contribute to elevated serum and urinary calcium levels and the formation of kidney stones. Well-informed patients can contribute a great deal to the management of their disease and enhance their sense of self-esteem and control, as well as avoid serious complications.
sarcoidosisImmunology An idiopathic multisystem disease characterized by nonnecrotizing–noncaseating granulomas Epidemiology Relatively more common in blacks, ♀, age 30 to 50, Clinical Cough, dyspnea, hemoptysis, endobronchial, upper respiratory tract and laryngeal 'bumpiness' due to mural granulomas, ↓ sputum; lungs are involved in ≥ 90%, followed by lymph nodes, skin, liver, eye, spleen, bone, joints, heart, muscle, CNS; lung function tests show restrictive, less commonly, obstructive disease; ocular disease occurs in ±25%–redness, photophobia, blurred vision; other findings: erythema nodosum, lymphadenopathy, hepato- and/or splenomegaly Immunology ↓ Cellular immunity–anergy; ↑ humoral immunity–polyclonal ↑ Igs, ↑ IgG in lung lavage fluid Imaging 10% of Pts have a normal CXR; 15% have pulmonary infiltrates sans lymphadenopathy; 40% have hilar lymphadenopathy; 30%–50% have infiltrates + lymphadenopathy Management Prednisone Diagnosis ↑ Angiotensin-converting enzyme Cause of death Heart, lungs. See Cardiac sarcoidosis, Scar sarcoidosis.
Synonym(s): Besnier-Boeck-Schaumann disease, Boeck disease, sarcoid.
sarcoidosis(săr″koyd-ō′sĭs) [″ + ″ + osis, condition]
The lungs are involved in 90% of cases of sarcoidosis and are the basis for the initial symptoms of fatigue, weight loss, anorexia, night sweats, shortness of breath, and a nonproductive cough. Hilar lymphadenopathy may precede the development of respiratory symptoms from alveolitis. Peripheral lymphadenopathy, iritis, skin lesions, splenomegaly, hepatomegaly, interstitial nephritis, peritoneal disease, involvement of other visceral organs, and skeletal changes are seen in patients with widespread disease. Immunological abnormalities include T-cell lymphocytopenia, increased blood monocyte count, and anergic reactions to skin tests for common allergens. In approx. 60% to 70% of patients, no permanent damage to the lungs or other organs occurs. Approx. 20% develop residual lung or eye damage, and 10% die of progressive pulmonary fibrosis or associated right-sided heart failure (cor pulmonale).
Diagnosis is made through a combination of clinical, radiographical, and histological findings. Sarcoidosis must be differentiated from other diseases that cause granulomas, such as tuberculosis, histoplasmosis, and some other fungal infections.
Sarcoidosis may progress insidiously or rapidly or may remit as the result of treatment with corticosteroids.
sarcoidosisA disease of unknown cause featuring GRANULOMAS in many parts of the body, especially in the lymph nodes, liver, lungs, skin and eyes. Sarcoidosis mainly affects young adults. Symptoms include fever, muscle and joint pain, ARTHRITIS, breathlessness and eye inflammation. There are enlarged lymph nodes in the neck and elsewhere, purplish swellings on the legs (erythema nodosum), a purplish rash on the face and areas of numbness. Treatment, when necessary, is with corticosteroid drugs. Most cases recover fully within 2 years without treatment.
Besnier,Ernest, French dermatologist, 1831-1909.
Patient discussion about sarcoidosis
Q. SARCOIDOSIS is it a lung disease?
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