sarcoidosis

Sarcoidosis

 

Definition

Sarcoidosis is a disease which can affect many organs within the body. It causes the development of granulomas. Granulomas are masses resembling little tumors. They are made up of clumps of cells from the immune system.

Description

Sarcoidosis is a very puzzling disorder. In addition to having no clear-cut understanding of the cause of sarcoidosis, researchers are also puzzled by its distribution in the world population. In the United States, for example, 10-17 times as many African-Americans are affected as white Americans. In Europe, whites are primarily affected.

Prevalence is a way of measuring the number of people affected per 100,000 people in a given population. The prevalence figures for sarcoidosis are very unusual. In the United States, prevalence figures range from five (5/100,000 in the United States) for whites to 40 for blacks. In Europe, prevalence ranges from three in Poland, to 10 in France, to 64 in Sweden, to 200 for Irish women living in London. Furthermore, a person from a group with very low prevalence who leaves his or her native land for a second location with a higher prevalence will then have the same risk as anyone living in that second location.

Sarcoidosis affects both men and women, although women are more likely to have the disorder. The average age for diagnosis is around 20-40 years.

Causes and symptoms

The cause of sarcoidosis is not known. Because the granulomas are primarily made up of cells from the immune system (macrophages and lymphocytes), an immune connection is strongly suspected. One of the theories which has been put forth suggests that exposure to some toxic or infectious material starts up an immune response. For some reason, the body is unable to stop the response, and it spreads from the original organ to other organs.

Because sarcoidosis has been noted to occur in family groups, a genetic cause has also been suggested. Research shows that identical twins are more likely to both have sarcoidosis than are nonidentical twins or other siblings.

Some cases of sarcoidosis occur without the patient even noting any symptoms. These cases are often discovered by chance during routine chest x rays. Most cases of sarcoidosis, however, begin with very nonspecific symptoms, such as decreased energy, weakness, and a dry cough. Occasionally, the cough is accompanied by some mild pain in the breastbone (sternum). Some patients note that they are having unusual shortness of breath while exercising. Some patients develop fever, decreased appetite, and weight loss.

Virtually every system of the body has the potential to suffer the effects of sarcoidosis:

• 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

Any, all, or even none of the above symptoms may be present in sarcoidosis.

Diagnosis

Diagnosis depends on information from a number of sources, including the patient's symptoms, the physical examination, x-ray pictures of the chest, and a number of other laboratory examinations of blood or other tissue. None of these categories of information are sufficient to make the diagnosis of sarcoidosis. There is no one test or sign or symptom which clearly points to sarcoidosis, excluding all other types of diseases. This is because nearly all of the symptoms and laboratory results in sarcoidosis also occur in other diseases. Diagnosis, then, requires careful consideration of many facts.

The physical examination in sarcoidosis may reveal the characteristic skin lesions. Wheezes may be heard throughout the lungs. The liver may be enlarged. Examination of the eyes using a special light called a slit-lamp may reveal changes indicative of sarcoidosis.

The chest x ray will show some pattern of abnormalities, which may include enlargement of the lymph nodes which drain the lung, scarring and abnormalities to the tissue of the lungs, and fluid accumulation around the lungs.

Lung function tests measure such things as the amount of air an individual can breathe in and breathe out, the speed at which the air flows in and out, and the amount of air left in the lung after blowing out as much as possible in one second. A variety of lung function tests may show abnormal results in sarcoidosis.

Other types of tests may be abnormal in sarcoidosis. The abnormal test results may also indicate other diseases. They include an elevation of a substance called angiotensin-converting enzyme in the blood, and an increased amount of calcium present in 24 hours worth of urine.

Bronchoscopy is a very helpful diagnostic test. This involves passing a tiny tube (bronchoscope) through the nose or mouth, down the trachea, and into the airways (bronchial tubes). The bronchial tubes can be inspected through the bronchoscope. The bronchoscope is also designed in such a way as to allow biopsies to be obtained. Bronchoalveolar lavage involves washing the surfaces with a sterile saltwater (saline) solution. The saline is then retrieved and examined in a laboratory. Cells and debris from within the bronchial tubes and the tiny sacs of the lung (the alveoli) will be obtained in this way, and can be studied for the presence of an abnormally large number of white blood cells. A tiny piece of the lung tissue can also be obtained through the bronchosocope. This can be studied under a microscope to look for the characteristic granulomas and inflammation of sarcoidosis.

A gallium 67 scan involves the injection of a radioactive material called gallium 67. In sarcoidosis, areas of the body which are inflamed will retain the gallium 67. These areas will then show up on the scan.

Treatment

Many cases of sarcoidosis resolve without treatment. If treatment is needed, the most effective one for sarcoidosis is the administration of steroid medications. These medications work to decrease inflammation throughout the body. The long-term use of steroid medications has serious potential side-effects. Patients are only treated with steroids when the problems caused by sarcoidosis are particularly serious. Many cases of sarcoidosis resolve without treatment.

Prognosis

The prognosis for sarcoidosis is quite good. About 60-70% of the time, sarcoidosis cures itself within a year or two. In about 20-30% of patients, permanent damage occurs to the lungs. About 15-20% of all patients go on to develop a chronic, relapsing form of sarcoidosis. Death can be blamed on sarcoidosis in about 10% of all sarcoidosis cases.

Prevention

Until researchers are able to pinpoint the cause of sarcoidosis, there will be no available recommendations for how to prevent it.

Key terms

Granuloma — Masses made up of a variety of immune cells, as well as fibroblasts (cells which make up connective tissue).

Immune system — The system of specialized organs, lymph nodes, and blood cells throughout the body which work together to prevent foreign invaders (bacteria, viruses, fungi, etc.) from taking hold and growing.

Resources

Periodicals

Zitkus, Bruce S. "Sarcoidosis: Varied Symptoms Often Impede Diagnosis of this Multisystem Disorder." American Journal of Nursing 97, no. 10 (October 1997): 40+.

sarcoidosis

 [sahr″koi-do´sis]

a chronic, progressive, generalized granulomatous reticulosis that may affect any part of the body but most frequently involves the lymph nodes, liver, spleen, lungs, skin, eyes, and small bones of the hands and feet, characterized by the presence in all affected organs or tissues of epithelioid cell tubercles, which become converted, in the older lesions, into a rather hyaline featureless fibrous tissue.



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.

Patient Care. Sarcoidosis is a multisystem disease that can cause complex problems for those who have an active case. However, some patients are asymptomatic and have pulmonary function within normal limits, so that no treatment is required. For patients who do have impaired lung function with pulmonary infiltrates, adrenal corticosteroids are prescribed, often for an extended period of time.



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.

Sarcoidosis. The most common sites of granulomas are the lungs and thoracic lymph nodes. Other extrathoracic sites are less commonly involved. Also shown is a granuloma composed primarily of epithelioid cells, macrophages, and lymphocytes. From Damjanov, 2000.

cardiac sarcoidosis involvement of the heart in sarcoidosis, with lesions ranging from a few asymptomatic granulomas to widespread infiltration of the myocardium by large masses of sarcoid tissue; it is characterized by a high incidence of atrioventricular block as well as right bundle branch block and ventricular arrhythmias.

muscular sarcoidosis sarcoidosis involving the skeletal muscles, with sarcoid tubercles, interstitial inflammation with fibrosis, and disruption and atrophy of the muscle fibers.

sar·coi·do·sis

(sar'koy-dō'sis), [MIM*181000]

A systemic granulomatous disease of unknown cause, especially involving the lungs with resulting interstitial fibrosis, but also involving lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands; granulomas are composed of epithelioid and multinucleated giant cells with little or no necrosis.

Synonym(s): Besnier-Boeck-Schaumann disease, Besnier-Boeck-Schaumann syndrome, Boeck disease, Boeck sarcoid, sarcoid, Schaumann syndrome

[sarcoid + G. -osis, condition]

sarcoidosis

(sär′koi-dō′sĭs)

n. pl. sarcoido·ses (-sēz)

A disease of unknown origin characterized by the formation of granulomatous lesions that appear especially in the liver, lungs, skin, and lymph nodes. Also called sarcoid.

sarcoidosis

Immunology 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.

sar·coi·do·sis

(sahr'koy-dō'sis)

A systemic granulomatous disease of unknown cause, especially involving the lungs with resulting fibrosis, but also involving lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands; granulomas are composed of epithelioid and multinucleated giant cells with little or no necrosis.
Synonym(s): Besnier-Boeck-Schaumann disease, Boeck disease, sarcoid.

[sarcoid + G. -osis, condition]

sarcoidosis

(săr″koyd-ō′sĭs) [″ + ″ + osis, condition]

SARCOIDOSIS

A chronic multisystem disease of unknown etiology, characterized by noncaseating (hard) granulomas and lymphocytic alveolitis. Sarcoidosis occurs most often in the southeastern U.S., is 10 times more common in blacks than whites, and is more common in women than men. See: illustration

Symptoms

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

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.

Treatment

Sarcoidosis may progress insidiously or rapidly or may remit as the result of treatment with corticosteroids.

illustration

sarcoidosis

A 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.

Besnier-Boeck disease - systemic granulomatous disease of unknown cause which involves lungs with resulting fibrosis; also involving lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands. Synonym(s): Besnier-Boeck-Schaumann disease; Besnier-Boeck-Schaumann syndrome; Boeck disease; Boeck sarcoid; sarcoidosis; Schaumann syndrome

Besnier-Boeck-Schaumann disease - Synonym(s): Besnier-Boeck disease

Besnier-Boeck-Schaumann syndrome - Synonym(s): Besnier-Boeck disease

Besnier prurigo - an atopic form which may be associated with asthma, hay fever, or other allergic conditions.

sarcoidosis 

An idiopathic, multisystem gra-nulomatous disorder in which the eye is affected in about 20% of patients. Ocular manifestations include anterior uveitis with aqueous flare, keratoconjunctivis sicca, lacrimal gland lesion, 'mutton-fat' keratic precipitates, posterior synechia, iris granulomas and nodules, intermediate uveitis, posterior uveitis or optic neuropathy. See dacryoadenitis; iridocyclitis; band keratopathy; exposure keratopathy; Mikulicz's syndrome.

sar·coi·do·sis

(sahr'koy-dō'sis) [MIM*181000]

Systemic granulomatous disease of unknown cause, especially involving lungs with resulting interstitial fibrosis, but also involving lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands.

[sarcoid + G. -osis, condition]

Patient discussion about sarcoidosis

Q. SARCOIDOSIS is it a lung disease?

A. It's an autoimmune disease that may affect almost every organ in the body, although involvement of the lung is the most common (90%), therefore it may be perceived as a lung disease. It may damage the lungs, nut not only them.

You may read more here:
www.mayoclinic.com/health/sarcoidosis/DS00251