Sarcoidosis: Difference between revisions

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==Causes and pathophysiology==
==Causes and pathophysiology==
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>
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]].
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.
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.''
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.


==Image Examples==
==Image Examples==

Revision as of 14:24, 26 September 2012

For patient information click here

Sarcoidosis
Sarcoidosis in a Lymph Node.
ICD-10 D86
ICD-9 135
OMIM 181000
DiseasesDB 11797
MedlinePlus 000076
MeSH D012507

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Editor-in-Chief: Philip Marcus, M.D., M.P.H. [1], Division of Pulmonary Medicine St. Francis Hospital-The Heart Center, Roslyn, NY

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2] and Hilary Womble, M. D.[[3]]

Click Here For The Chapter On The Heart In Sarcoidosis

Causes and pathophysiology

Image Examples






Treatment

Corticosteroids, 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 [1]), and their use is generally limited to severe, progressive, or organ-threatening disease. The influence of corticosteroids or other immunosuppressants on the natural history is unclear.

Severe symptoms are generally treated with steroids, and steroid-sparing agents 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 cells, 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.

Avoidance of sunlight and Vitamin D foods may be helpful in patients who are susceptible to developing hypercalcemia.

Case Examples

Case #1

Clinical Summary

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.

Histopathological Findings

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

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.


This photomicrograph of lymph node tissue illustrates a paucity of lymphocytes as well as numerous small, pale-staining nodules (arrows) throughout the tissue.


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.


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.


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.


This is a higher-power photomicrograph of an asteroid body (arrow) inside of a multinucleated giant cell.


See also

References

External links




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