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| | __NOTOC__ |
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = Sarcoidosis | | | Name = Sarcoidosis | |
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| OMIM = 181000 | | | OMIM = 181000 | |
| MedlinePlus = 000076 | | | MedlinePlus = 000076 | |
| eMedicineSubj = med |
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| eMedicineTopic = 2063 |
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| MeshID = D012507 | | | MeshID = D012507 | |
| }} | | }} |
| {{SI}} | | {{Sarcoidosis}} |
| '''Editor-in-Chief:''' Philip Marcus, M.D., M.P.H. [mailto:pmarcus192@aol.com], Division of Pulmonary Medicine
| | {{CMG}}; Philip Marcus, M.D., M.P.H. [mailto:pmarcus192@aol.com], Division of Pulmonary Medicine |
| St. Francis Hospital-The Heart Center, Roslyn, NY | | St. Francis Hospital-The Heart Center, Roslyn, NY |
| __NOTOC__
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| '''Associate Editor-In-Chief:''' {{CZ}} and [[user:Hilary Womble|Hilary Womble, M. D.]][[mailto:hwomble@bidmc.harvard.edu]]
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| {{Editor Join}} | | '''Associate Editor-In-Chief:''' {{CZ}}; [[user:Hilary Womble|Hilary Womble, M. D., Roshan Dinparasti Saleh M. D.]] |
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| | {{SK}} Besnier-Boeck disease; Besnier-Boeck-Schaumann disease; Sarcoid; Uveoparotid fever; Loefgren's syndrome |
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| ===[[The Heart in Sarcoidosis|Click Here For The Chapter On The Heart In Sarcoidosis]]=== | | ===[[The Heart in Sarcoidosis|Click Here For The Chapter On The Heart In Sarcoidosis]]=== |
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| ==Overview== | | ==[[Sarcoidosis overview|Overview]]== |
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| '''Sarcoidosis''', also called '''sarcoid''' (from the Greek 'sark' and 'oid' meaning "flesh-like") or '''Besnier-Boeck disease''', is an immune system disorder characterised by non-[[caseating]] [[granuloma]]s (small inflammatory nodules) that most commonly arises in young adults. The cause of the disease is still unknown. Virtually any organ can be affected; however, granulomas most often appear in the [[lung]]s ({{ICD10|D|86|0|d|80}}) or the [[lymph nodes]] ({{ICD10|D|86|1|d|80}}). Symptoms can occasionally appear suddenly but usually appear gradually. The clinical course varies and ranges from asymptomatic disease that resolves spontaneously to a debilitating chronic condition that may lead to death.<ref name="pmid">{{cite journal |author= |title= |journal= |volume= |issue= |pages= |year= |pmid= |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0954-6111(07)00366-6}}</ref>
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| ==Epidemiology==
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| Sarcoidosis most commonly affects young adults of both sexes, with a slight preponderance for women having been reported by most studies. Incidence is highest for individuals younger than 40 and peaks in the age-group from 20 to 29 years.<ref name="Baughman"/>
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| Sarcoidosis occurs throughout the world in all races with a prevalence ranging from 1 to 40 per 100,000.
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| The disease is most prevalent in Northern European countries, and the highest annual incidence of 60 per 100,000 is found in Sweden and Iceland. In the United States, sarcoidosis is more common in people of African descent than Caucasians, with annual incidence reported as 35.5 and 10.9 per 100,000, respectively.<ref name="Henke">Henke, C. E., G. Henke, L. R. Elveback, C. M. Beard, D. J. Ballard and L. T. Kurland. 1986. The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival. Am. J. Epidemiol. 123:840–845.</ref> Sarcoidosis is less commonly reported in South America, Spain and India.
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| The differing incidence across the world may be at least partially attributable to the lack of screening programs in certain regions of the world and the overshadowing presence of other granulolomatous diseases such as [[tuberculosis]], that may interfere with the diagnosis of sarcoidosis where they are prevalent.<ref name="Baughman">Baughman RP, Lower EE, du Bois RM. Sarcoidosis. The Lancet 2003/3/29;361(9363):1111-8.</ref>
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| There may also be racial differences in the severity of the disease. Several studies suggest that the presentation in people of African origin may be more severe than for Caucasians, who are more likely to suffer from asymptomatic disease.<ref name="Am Thor Soc"> "American Thoracic Society: Statement on sarcoidosis. Am J Respir Crit Care Med 1999;160:736-755.</ref>
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| ==Signs and symptoms==
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| Sarcoidosis is a Systemic Disease that can affect any organ. Common symptoms are vague, such as [[fatigue (physical)|fatigue]] unchanged by sleep, [[asthenia|lack of energy]], [[weight loss]], aches and pains, [[arthralgia]], [[xerophthalmia|dry eyes]], blurry vision, [[dyspnea|shortness of breath]], a dry hacking [[cough]] or skin lesions. The cutaneous symptoms vary, and range from [[rash]]es and noduli (small bumps) to [[erythema nodosum]] or [[lupus pernio]]. It is often [[asymptomatic]].
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| The combination of [[erythema nodosum]], bilateral [[hilar]] [[lymphadenopathy]] and [[arthralgia]] is called [[Lofgren syndrome|''Lofgren syndrome'']]. This syndrome has a relatively good prognosis.
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| Renal, liver (including [[portal hypertension]]), heart or brain involvement may cause further symptoms and altered functioning. Manifestations in the eye include [[uveitis]] and retinal inflammation, which may result in loss of visual acuity or blindness. Sarcoidosis affecting the brain or nerves is known as [[neurosarcoidosis]].
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| The combination of [[anterior uveitis]], [[parotitis]] and fever is called [[uveoparotitis]], and is associated with ''Heerfordt-Waldenstrom syndrome''. ({{ICD10|D|86|8|d|80}})
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| ==Investigations==
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| [[Hypercalcemia]] (high [[calcium in biology|calcium]] levels) and its symptoms may be the result of excessive [[vitamin D]] activation.
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| Sarcoidosis most often manifests as a restrictive disease of the lungs, causing a decrease in [[lung volume]] and decreased [[compliance]] (the ability to stretch). The disease typically limits the amount of air drawn into the lungs, but produces higher than normal expiratory flow ratios. The [[vital capacity]] (full breath in, to full breath out) is decreased, and most of this air can be blown out in the first second. This means the [[spirometry|FEV<sub>1</sub>/FVC ratio]] is increased from the normal of about 80%, to 90%. Obstructive lung changes, causing a decrease in the amount of air that can be exhaled, may occur when enlarged lymph nodes in the chest compress airways or when internal inflammation or nodules impede airflow.
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| Chest X-ray changes are divided into five stages
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| * Stage 0 Negative chest radiograph
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| * Stage 1 [[bihilar lymphadenopathy]]
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| * Stage 2 Hilar lymphadenopathy and reticulonodular pulmonary infiltrates
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| * Stage 3 bilateral infiltrates
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| * Stage 4 fibrocystic sarcoidosis typically with upward hilar retraction, cystic & bullous changes
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| Because sarcoidosis can affect multiple organ systems, follow-up on a patient with sarcoidosis should always include an [[electrocardiogram]], [[ophthalmologic]] exam, [[liver function tests]], serum calcium and 24-hour urine calcium.
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| ==Causes and pathophysiology==
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| No direct cause of sarcoidosis has been identified, although there have been reports of cell wall deficient [[bacteria]] that may be possible pathogens.<ref>Almenoff PL, Johnson A, Lesser M, Mattman LH. ''Growth of acid fast L forms from the blood of patients with sarcoidosis.'' Thorax 1996;51:530-3. PMID 8711683.</ref> These bacteria are not identified in standard laboratory analysis. It has been thought that there may be a hereditary factor because some families have multiple members with sarcoidosis. To date, no reliable genetic markers have been identified, and an alternate hypothesis is that family members share similar exposures to environmental pathogens. There have also been reports of transmission of sarcoidosis via [[organ transplant]]s.<ref>Padilla ML, Schilero GJ, Teirstein AS. ''Donor-acquired sarcoidosis.'' Sarcoidosis Vasc Diffuse Lung Dis 2002;19:18-24. PMID 12002380.</ref>
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| Sarcoidosis frequently causes a dysregulation of [[vitamin D]] production with an increase in extrarenal (outside the kidney) production.<ref>Barbour GL, Coburn JW, Slatopolsky E, Norman AW, Horst RL. ''Hypercalcemia in an anephric patient with sarcoidosis: evidence for extrarenal generation of 1,25-dihydroxyvitamin D.'' N Engl J Med 1981;305:440-3. PMID 6894783.</ref> Specifically, [[macrophages]] inside the granulomas convert vitamin D to its active form, resulting in elevated levels of the hormone 1,25-dihydroxyvitamin D and symptoms of [[hypervitaminosis D]] that may include [[fatigue (physical)|fatigue]], [[lack of strength]] or energy, [[irritability]], [[metallic taste]], temporary [[memory loss]] or cognitive problems. Physiological compensatory responses (e.g. suppression of the [[parathyroid hormone]] levels) may mean the patient does not develop frank [[hypercalcemia]].
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| Sarcoidosis has been associated with [[celiac disease]]. Celiac disease is a condition in which there is a chronic reaction to certain protein chains, commonly referred to as glutens, found in some cereal grains. This reaction causes destruction of the villi in the small intestine, with resulting malabsorption of nutrients.
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| While disputed, some cases have been determined to be caused by inhalation of the dust from the collapse of the World Trade Center after the September 11, 2001 attacks.<ref>[http://www.nytimes.com/2007/05/24/nyregion/24dust.html ''New York Times'' article, May 24, 2007]</ref> ''See [[Health effects arising from the September 11, 2001 attacks]] for more information.''
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| Gallium-67 citrate is useful for diagnosing suspected sarcoidosis and evaluation of treatment response. It is more sensitive than radiographic images on diagnosis of Sarcoidosis.
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| ==Image Examples==
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| <div align="left">
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| <gallery heights="175" widths="125">
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| Image:Sarcoid-001.jpg|Sarcoidosis
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| Image:Sarcoid-002.jpg|Sarcoidosis
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| Image:Sarcoid-003.jpg|Sarcoidosis
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| </gallery>
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| </div>
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| <div align="left">
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| <gallery heights="175" widths="125">
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| Image:Sarcoid-004.jpg|Sarcoidosis
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| Image:Sarcoid-005.jpg|Sarcoidosis
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| Image:Sarcoid-006.jpg|Sarcoidosis
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| </gallery>
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| </div>
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| <div align="left">
| | ==[[Sarcoidosis historical perspective|Historical Perspective]]== |
| <gallery heights="175" widths="125">
| | ==[[Sarcoidosis classification|Classification]]== |
| Image:Osseous-sarcoid-001.jpg|Osseous sarcoidosis
| | ==[[Sarcoidosis pathophysiology|Pathophysiology]]== |
| Image:Osseous-sarcoid-002.jpg|Osseous sarcoidosis
| | ==[[Sarcoidosis causes|Causes]]== |
| Image:Osseous-sarcoid-003.jpg|Osseous sarcoidosis
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| </gallery>
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| </div>
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| | ==[[Sarcoidosis differential diagnosis|Differentiating Sarcoidosis from other Disorders]]== |
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| <div align="left">
| | ==[[Sarcoidosis epidemiology and demographics|Epidemiology and Demographics]]== |
| <gallery heights="175" widths="125">
| | ==[[Sarcoidosis risk factors|Risk Factors]]== |
| Image:Complicated-sarcoid-001.jpg|Complicated sarcoidosis
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| Image:Complicated-sarcoid-002.jpg|Complicated sarcoidosis
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| Image:Complicated-sarcoid-003.jpg|Complicated sarcoidosis
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| </gallery>
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| </div>
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| | ==[[Sarcoidosis screening|Screening]]== |
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| <div align="left">
| | ==[[Sarcoidosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| <gallery heights="175" widths="125">
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| Image:Complicated-sarcoid-004.jpg|Complicated sarcoidosis
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| Image:Complicated-sarcoid-005.jpg|Complicated sarcoidosis
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| </gallery>
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| </div>
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| | | ==Diagnosis== |
| <div align="left">
| | [[Sarcoidosis history and symptoms|History and Symptoms]] | [[Sarcoidosis physical examination|Physical Examination]] | [[Sarcoidosis laboratory findings|Laboratory Findings]] | [[Sarcoidosis electrocardiogram | Electrocardiogram]] | [[Sarcoidosis chest x ray|Chest X Ray]] | [[Sarcoidosis CT|CT]] | [[Sarcoidosis MRI|MRI]] | [[Sarcoidosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Sarcoidosis other imaging findings|Other Imaging Findings]] | [[Sarcoidosis other diagnostic studies|Other Diagnostic Studies]] |
| <gallery heights="175" widths="125">
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| Image:Sarcoid-101.jpg|Sarcoidosis
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| Image:Sarcoid-102.jpg|Sarcoidosis
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| Image:Sarcoid-103.jpg|Sarcoidosis
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| </gallery>
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| </div>
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| ==Treatment== | | ==Treatment== |
| [[Corticosteroid]]s, most commonly [[prednisone]], have been the standard treatment for many years. In some patients, this treatment can slow or reverse the course of the disease, but other patients unfortunately do not respond to steroid therapy. The use of corticosteroids in mild disease is controversial because in many cases the disease remits spontaneously. Additionally, corticosteroids have many recognized dose- and duration-related side effects (which can be reduced through the use of alternate-day dosing for those on chronic prednisone therapy <ref>[http://www.uspharmacist.com/NewLook/CE/glucocort/lesson.htm "Dosing Considerations"]</ref>), and their use is generally limited to severe, progressive, or organ-threatening disease. The influence of corticosteroids or other [[immunosuppressant]]s on the natural history is unclear. | | [[Sarcoidosis medical therapy|Medical Therapy]] | [[Sarcoidosis primary prevention|Primary Prevention]] | [[Sarcoidosis secondary prevention|Secondary Prevention]] | [[Sarcoidosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Sarcoidosis future or investigational therapies|Future or Investigational Therapies]] |
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| Severe symptoms are generally treated with steroids, and [[steroid-sparing agent]]s such as [[azathioprine]] and [[methotrexate]] are often used. Rarely, [[cyclophosphamide]] has also been used. As the granulomas are caused by collections of immune system cells, particularly [[T cell]]s, there has been some early indications of success using immunosuppressants, [[interleukin-2]] inhibitors or anti-[[tumor necrosis factor-alpha]] treatment (such as [[infliximab]]). Unfortunately, none of these have provided reliable treatment and there can be significant side effects such as an increased risk of reactivating latent [[tuberculosis]].
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| Avoidance of sunlight and Vitamin D foods may be helpful in patients who are susceptible to developing [[hypercalcemia]].
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| ==Case Examples== | | ==Case Studies== |
| | [[Sarcoidosis case study one|Case study #1]] |
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| ===Case #1=== | | ==Related Chapters== |
| | | * [[The Heart in Sarcoidosis]] |
| ====Clinical Summary====
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| This 33-year-old white female was admitted for evaluation of abnormal findings on a chest x-ray. She was asymptomatic and a physical examination revealed no significant abnormalities. Laboratory results indicated hypercalcemia and elevated gamma globulin. Radiographic examination showed enlarged subcarinal, hilar, and right paratracheal lymph nodes. A right paratracheal lymph node was biopsied. Special stains for acid-fast bacilli and fungi were negative and a diagnosis of sarcoidosis was made.
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| ====Histopathological Findings====
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| [http://www.peir.net Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] | |
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| [[Image:Sarcoidosis lymph node case 001.jpg|left|thumb|400px|This is a low-power photomicrograph of a lymph node. Note the rather pale-pink color of the tissue with dark-staining cells found in only a few scattered areas. These darker cells represent the original lymphocytes of this lymphoid organ.]] | |
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| [[Image:Sarcoidosis lymph node case 002.jpg|left|thumb|400px|This photomicrograph of lymph node tissue illustrates a paucity of lymphocytes as well as numerous small, pale-staining nodules (arrows) throughout the tissue.]]
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| [[Image:Sarcoidosis lymph node case 003.jpg|left|thumb|400px|This is a photomicrograph of the small nodules (arrows) seen in the previous image. Close examination reveals that they are composed of large macrophages (epithelioid macrophages). These small granulomas form multiple series of reaction centers throughout the lymph node. Note the remaining lymphocytes surrounding the granulomas.]]
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| [[Image:Sarcoidosis lymph node case 004.jpg|left|thumb|400px|This photomicrograph of a single granuloma illustrates the individual macrophages (arrows) which make up the bulk of this tissue. There is an absence of necrosis in the center of the lesions in this case.]]
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| [[Image:Sarcoidosis lymph node case 005.jpg|left|thumb|400px|This is a photomicrograph of a multinucleated giant cell (1). In the center of this foreign body-containing giant cell there is a small asteroid body (2). There is no functional significance to this asteroid body.]]
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| [[Image:Sarcoidosis lymph node case 006.jpg|left|thumb|400px|This is a higher-power photomicrograph of an asteroid body (arrow) inside of a multinucleated giant cell.]]
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| ==See also==
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| * [[Garland's triad]] | | * [[Garland's triad]] |
| * [[Kveim test]] | | * [[Kveim test]] |
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| ==References==
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| {{Reflist|2}}
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| ==External links== | | ==External links== |
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| {{Immune disorders}} | | {{Immune disorders}} |
| {{SIB}}
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