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      Hepatic Sarcoidosis

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      Canadian Journal of General Internal Medicine
      Dougmar Publishing Group, Inc.

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          Abstract

          Sarcoidosis is an idiopathic multisystem disease, characterized by non-caseating granulomas in affected organs. Pulmonary involvement is the most common site of disease activity, although the disease can also affect the liver, bone marrow, and heart. We report a case of symptomatic hepatic sarcoidosis in a 31-year-old African-Canadian woman which responded to treatment with doxycycline. The patient was referred to internal medicine from a peripheral emergency department after presenting with fever, hepatitis, and thrombocytopenia with a computed tomography scan concerning for hepatic abscesses. The patient remained febrile with worsening liver enzymes despite broad spectrum antibiotics. An extensive infectious workup was negative. Biopsies of bone marrow and liver revealed non-caseating granuloma, and an elevated angiotensin-converting enzyme level. Doxycycline, initially started for presumptive infective etiology, lead to rapid resolution of symptoms and normalization of biochemical markers. To our knowledge, this is the first case report of doxycycline used to treat hepatic sarcoidosis in Canada. Résume La sarcoïdose est une maladie multisystémique idiopathique, caractérisée par la présence de granulomes non caséeux dans les organes affectés. Les poumons sont le plus souvent le site de la maladie, quoique celle-ci se manifeste aussi dans le foie, la moelle osseuse et le cœur. Nous décrivons ici un cas de sarcoïdose hépatique symptomatique chez une afro-canadienne de 31 ans qui a répondu à un traitement à la doxycycline. La patiente a été aiguillée en médecine interne par un service des urgences en périphérie après s’y être présentée avec de la fièvre, une hépatite et une thrombopénie et y avoir subi une tomodensitométrie relative à des abcès au foie. Malgré l’administration d’antibiotiques à large spectre, la patiente est demeurée fébrile et ses enzymes hépatiques ont continué de se dégrader. Les nombreux tests infectieux effectués se sont révélés négatifs. Des biopsies de la moelle osseuse et du foie ont révélé la présence de granulomes non caséeux et un taux élevé de l’enzyme de conversion de l'angiotensine. La doxycycline, initialement administrée pour une étiologie infectieuse présumée, a rapidement entrainé la résorption des symptômes et la normalisation des marqueurs biochimiques. À notre connaissance, il s’agit du premier cas rapporté au Canada de traitement d’une sarcoïdose hépatique par la doxycycline.    

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          A concise review of pulmonary sarcoidosis.

          This is an update on sarcoidosis, focusing on etiology, diagnosis, and treatment. In the area of etiopathogenesis, we now have a better understanding of the immune response that leads to the disease as well as genetic factors that modify both the risk for the disease and its clinical outcome. Several groups have also identified possible agents as a cause for sarcoidosis. Although none of these potential causes has been definitely confirmed, there is increasing evidence to support that one or more infectious agents may cause sarcoidosis, although this organism may no longer be viable in the patient. The diagnosis of sarcoidosis has been significantly aided by new technology. This includes the endobronchial ultrasound, which has been shown to increase the yield of needle aspiration of mediastinal and hilar lymph nodes. The positive emission tomography scan has proven useful for selecting possible biopsy sites by identifying organ involvement not appreciated by routine methodology. It has also helped in assessing cardiac involvement. The biologic agents, such as the anti-tumor necrosis factor antibodies, have changed the approach to refractory sarcoidosis. There is increasing evidence that the clinician can identify which patient is most likely to benefit from such therapy. As new and more potent antiinflammatory agents have been developed, it is clear that there are other factors that burden the patient with sarcoidosis, including fatigue and sarcoidosis-associated pulmonary hypertension. There have been several recent studies demonstrating treatment options for these problems.
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            Gastrointestinal and hepatic manifestations of sarcoidosis.

            Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the affected organs, including skin, heart, nervous system, and joints. Diagnosis of sarcoidosis is generally based upon a compatible history, demonstration of granulomas in at least two different organs, negative staining and culture for acid fast bacilli, absence of occupational or domestic exposure to toxins, and lack of drug-induced disease. Involvement of the hollow organs is rare. Rather than being due to sarcoidosis, some reported mucosal lesions may simply have incidental granulomas. Extrinsic compression from lymphadenopathy can occur throughout the gastrointestinal tract. The stomach, particularly the antrum, is the most common extrahepatic organ to be involved, while the small bowel is the least common. Liver involvement frequently occurs and ranges from asymptomatic incidental granulomas to portal hypertension from granulomas in the portal triad, usually with relatively preserved liver function. CT scans show hepatosplenomegaly and adenopathy, followed in frequency by focal low-attenuation lesions of the liver and spleen. Ascites is usually a transudate from right heart failure (because of pulmonary hypertension) or portal hypertension (because of biliary cirrhosis). Rarely, an exudative ascites may occur from studding of the peritoneum with nodules. Pancreatic involvement presents as a mass, usually in the head or a diffusely firm, nodular organ. Corticosteroids should be instituted when organ function is threatened, usually lungs, eyes, and central nervous system. Their role in the treatment of hepatic sarcoidosis is unclear. The overall prognosis is good although most patients will have some permanent organ impairment. Cardiac and pulmonary diseases are the main causes of death.
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              The Use of Tetracyclines for the Treatment of Sarcoidosis

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                Author and article information

                Journal
                Canadian Journal of General Internal Medicine
                Can Journ Gen Int Med
                Dougmar Publishing Group, Inc.
                2369-1778
                1911-1606
                January 02 2018
                January 02 2018
                : 12
                : 4
                Article
                10.22374/cjgim.v12i4.179
                3a88299a-006d-4e25-8a6c-09f60c6cc5ab
                © 2018

                Copyright of articles published in all DPG titles is retained by the author. The author grants DPG the rights to publish the article and identify itself as the original publisher. The author grants DPG exclusive commercial rights to the article. The author grants any non-commercial third party the rights to use the article freely provided original author(s) and citation details are cited. To view a copy of this license, visit https://creativecommons.org/licenses/by-nc/4.0/


                General medicine,Geriatric medicine,Neurology,Internal medicine
                General medicine, Geriatric medicine, Neurology, Internal medicine

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