Macrophage activation syndrome resistant to medical therapy in a patient with systemic lupus erythematosus and its remission with splenectomy
Identifieur interne : 001697 ( Main/Exploration ); précédent : 001696; suivant : 001698Macrophage activation syndrome resistant to medical therapy in a patient with systemic lupus erythematosus and its remission with splenectomy
Auteurs : Ji Min Kim [Corée du Sud] ; Seung Ki Kwok [Corée du Sud] ; Ji Hyeon Ju [Corée du Sud] ; Kyung Su Park [Corée du Sud] ; Gyeong Sin Park [Corée du Sud] ; Ho Youn Kim [Corée du Sud] ; Sung Hwan Park [Corée du Sud]Source :
- Rheumatology International [ 0172-8172 ] ; 2013-03-01.
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Abstract
Abstract: Macrophage activation syndrome (MAS) is a rare, but potentially life-threatening complication of systemic lupus erythematosus (SLE). A bone marrow biopsy often provides pathologic evidence of MAS, and MAS usually responds to corticosteroids alone or with the addition of cyclosporine A. We describe a case of MAS developing in a pregnant patient with SLE, who presented with fever and pancytopenia. Extensive investigations could not find the evidence of infection. Although intensive medical treatment was performed with a suspicion of MAS based on clinical grounds, no response was observed and bone marrow biopsy showed no evidence of hemophagocytosis. Positron emission tomography/computed tomography (PET/CT) suggested the possible cause of fever was in the spleen where fluorodeoxyglucose uptake was markedly increased. After splenectomy, the patient was improved and numerous hemophagocytic macrophages were proved in the splenic tissue. With this unique case, we would like to emphasize that bone marrow biopsy cannot always be relied on in making a diagnosis of MAS and PET/CT can provide helpful information in the diagnosis of MAS.
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DOI: 10.1007/s00296-010-1654-4
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syndrome</term><term>Positron emission tomography</term><term>Spleen</term><term>Systemic lupus</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: Macrophage activation syndrome (MAS) is a rare, but potentially life-threatening complication of systemic lupus erythematosus (SLE). A bone marrow biopsy often provides pathologic evidence of MAS, and MAS usually responds to corticosteroids alone or with the addition of cyclosporine A. We describe a case of MAS developing in a pregnant patient with SLE, who presented with fever and pancytopenia. Extensive investigations could not find the evidence of infection. Although intensive medical treatment was performed with a suspicion of MAS based on clinical grounds, no response was observed and bone marrow biopsy showed no evidence of hemophagocytosis. Positron emission tomography/computed tomography (PET/CT) suggested the possible cause of fever was in the spleen where fluorodeoxyglucose uptake was markedly increased. After splenectomy, the patient was improved and numerous hemophagocytic macrophages were proved in the splenic tissue. With this unique case, we would like to emphasize that bone marrow biopsy cannot always be relied on in making a diagnosis of MAS and PET/CT can provide helpful information in the diagnosis of MAS.</div></front></TEI><affiliations><list><country><li>Corée du Sud</li></country><region><li>Région capitale de Séoul</li></region><settlement><li>Séoul</li></settlement></list><tree><country name="Corée du Sud"><region name="Région capitale de Séoul"><name sortKey="Kim, Ji Min" sort="Kim, Ji Min" uniqKey="Kim J" first="Ji Min" last="Kim">Ji Min Kim</name></region><name sortKey="Ju, Ji Hyeon" sort="Ju, Ji Hyeon" uniqKey="Ju J" first="Ji Hyeon" last="Ju">Ji Hyeon Ju</name><name sortKey="Kim, Ho Youn" sort="Kim, Ho Youn" uniqKey="Kim H" first="Ho Youn" last="Kim">Ho Youn Kim</name><name sortKey="Kwok, Seung Ki" sort="Kwok, Seung Ki" uniqKey="Kwok S" first="Seung Ki" last="Kwok">Seung Ki Kwok</name><name sortKey="Park, Gyeong Sin" sort="Park, Gyeong Sin" uniqKey="Park G" first="Gyeong Sin" last="Park">Gyeong Sin Park</name><name sortKey="Park, Kyung Su" sort="Park, Kyung Su" uniqKey="Park K" first="Kyung Su" last="Park">Kyung Su Park</name><name sortKey="Park, Sung Hwan" sort="Park, Sung Hwan" uniqKey="Park S" first="Sung Hwan" last="Park">Sung Hwan Park</name><name sortKey="Park, Sung Hwan" sort="Park, Sung Hwan" uniqKey="Park S" first="Sung Hwan" last="Park">Sung Hwan Park</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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