{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T12:29:36Z","timestamp":1791289776800,"version":"4.3.0"},"reference-count":34,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,2,10]],"date-time":"2014-02-10T00:00:00Z","timestamp":1391990400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"funder":[{"DOI":"10.13039\/501100004431","name":"Fondation de France","doi-asserted-by":"crossref","award":["2009002515-16 & 201200029519"],"award-info":[{"award-number":["2009002515-16 & 201200029519"]}],"id":[{"id":"10.13039\/501100004431","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Intern Med"],"published-print":{"date-parts":[[2014,9]]},"abstract":"Abstract<\/jats:title>\n \n Rationale<\/jats:title>\n \n Xanthomatosis associated with monoclonal gammopathy includes hyperlipidaemic xanthoma (\n HX<\/jats:styled-content>\n ), normolipidaemic xanthoma (\n NX<\/jats:styled-content>\n ) and necrobiotic xanthogranuloma (\n NXG<\/jats:styled-content>\n ). All three pathologies are characterized by skin or visceral lesions related to cholesterol accumulation, monoclonal immunoglobulin (MIg) and hypocomplementemia. The pathophysiology underlying\n NXG<\/jats:styled-content>\n remains unknown although the involvement of MIg is suspected.\n <\/jats:p>\n <\/jats:sec>\n \n Objective<\/jats:title>\n \n To provide further insights into the pathophysiology of\n NXG<\/jats:styled-content>\n , we evaluated the plasma lipid phenotype, mechanisms involved in cellular cholesterol accumulation and role of MIg in an analysis of blood and plasma markers of inflammation in 16 patients with xanthomatosis [\n NXG<\/jats:styled-content>\n (\n n<\/jats:italic>\n \u00a0=\u00a08) and NX (\n n<\/jats:italic>\n \u00a0=\u00a08)] associated with monoclonal IgG relative to the relevant controls.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n The lipid profile of patients with\n NXG<\/jats:styled-content>\n was characterized by a low\n HDL<\/jats:styled-content>\n \u2010C phenotype and an abnormal distribution of\n HDL<\/jats:styled-content>\n particles. Sera from patients with\n NXG<\/jats:styled-content>\n induced cholesterol accumulation in human macrophages. This accumulation was due in part to a significant reduction in the\n HDL<\/jats:styled-content>\n capacity to promote cholesterol efflux from macrophages, which was not found in the case of\n NX<\/jats:styled-content>\n . The MIg of NXG and NX patients was tested positively by ELISA to recognize a large spectrum of lipoproteins. High plasma levels of pro\u2010inflammatory cytokines (\n TNF<\/jats:styled-content>\n \u03b1 and IL\u20106), soluble cytokine receptors (sIL\u20106R, s\n TNFRI<\/jats:styled-content>\n and s\n TNFRII<\/jats:styled-content>\n ), adhesion molecules (\n VCAM<\/jats:styled-content>\n \u20101 and\n ICAM<\/jats:styled-content>\n \u20101) and chemokines (\n MCP<\/jats:styled-content>\n \u20101, IL\u20108 and\n MIP<\/jats:styled-content>\n \u20101\u03b1) were observed in both patients with\n NXG<\/jats:styled-content>\n and\n NX<\/jats:styled-content>\n , revealing a specific xanthoma inflammatory signature which was inversely correlated with plasma levels of anti\u2010inflammatory\n HDL<\/jats:styled-content>\n . However, patients with\n NXG<\/jats:styled-content>\n were distinguished by elevated levels of IL\u201015 and a marked increase in the rate of intermediate CD14++CD16+ monocytes.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n \n This study revealed that\n NXG<\/jats:styled-content>\n is characterized by impaired macrophage lipid homeostasis associated with a systemic inflammatory profile that may result from the interaction of MIg and lipoproteins.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/joim.12195","type":"journal-article","created":{"date-parts":[[2014,1,16]],"date-time":"2014-01-16T01:18:47Z","timestamp":1389835127000},"page":"269-284","source":"Crossref","is-referenced-by-count":43,"title":["Physiopathology of necrobiotic xanthogranuloma with monoclonal gammopathy"],"prefix":"10.1111","volume":"276","author":[{"given":"R.","family":"Szalat","sequence":"first","affiliation":[{"name":"D\u00e9partement d'immunologie Clinique H\u00f4pital Saint Louis Paris France"},{"name":"EA3963 Universit\u00e9 Paris 7 Denis Diderot INSERM IFR105 Institut Universitaire d'H\u00e9matologie Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Pirault","sequence":"additional","affiliation":[{"name":"INSERM UMR_S939 Dyslipidemia, Inflammation and Atherosclerosis in Metabolic Diseases Paris France"},{"name":"UPMC Univ Paris 06 UMR_S939 Paris France"},{"name":"Institute of Cardiometabolism and Nutrition (ICAN) Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.\u2010P.","family":"Fermand","sequence":"additional","affiliation":[{"name":"D\u00e9partement d'immunologie Clinique H\u00f4pital Saint Louis Paris France"},{"name":"EA3963 Universit\u00e9 Paris 7 Denis Diderot INSERM IFR105 Institut Universitaire d'H\u00e9matologie Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Carri\u00e9","sequence":"additional","affiliation":[{"name":"INSERM UMR_S939 Dyslipidemia, Inflammation and Atherosclerosis in Metabolic Diseases Paris France"},{"name":"UPMC Univ Paris 06 UMR_S939 Paris France"},{"name":"Institute of Cardiometabolism and Nutrition (ICAN) Paris France"},{"name":"Assistance\u2010Publique H\u00f4pitaux de Paris H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Saint\u2010Charles","sequence":"additional","affiliation":[{"name":"INSERM UMR_S939 Dyslipidemia, Inflammation and Atherosclerosis in Metabolic Diseases Paris France"},{"name":"UPMC Univ Paris 06 UMR_S939 Paris France"},{"name":"Institute of Cardiometabolism and Nutrition (ICAN) Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Olivier","sequence":"additional","affiliation":[{"name":"INSERM UMR_S939 Dyslipidemia, Inflammation and Atherosclerosis in Metabolic Diseases Paris France"},{"name":"UPMC Univ Paris 06 UMR_S939 Paris France"},{"name":"Institute of Cardiometabolism and Nutrition (ICAN) Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Robillard","sequence":"additional","affiliation":[{"name":"INSERM UMR_S939 Dyslipidemia, Inflammation and Atherosclerosis in Metabolic Diseases Paris France"},{"name":"UPMC Univ Paris 06 UMR_S939 Paris France"},{"name":"Institute of Cardiometabolism and Nutrition (ICAN) Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Frisdal","sequence":"additional","affiliation":[{"name":"INSERM UMR_S939 Dyslipidemia, Inflammation and Atherosclerosis in Metabolic Diseases Paris France"},{"name":"UPMC Univ Paris 06 UMR_S939 Paris France"},{"name":"Institute of Cardiometabolism and Nutrition (ICAN) Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. 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