{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T16:14:50Z","timestamp":1785255290449,"version":"3.55.0"},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2007,1,30]],"date-time":"2007-01-30T00:00:00Z","timestamp":1170115200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatism"],"published-print":{"date-parts":[[2007,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up\u2010regulated monokine production, high serum levels of triglycerides and ferritin, clotting abnormalities with hypofibrinogenemia, and rapidly evolving pancytopenia should alert the clinician to the possible diagnosis of macrophage activation syndrome, even in autoinflammatory diseases characterized basically by the periodic recurrence of unprovoked inflammatory attacks. Bone marrow aspiration showing well\u2010differentiated macrophages phagocytosing hematopoietic elements remains the main tool for a final diagnosis, and cyclosporine is the best strategy for treatment.<\/jats:p>","DOI":"10.1002\/art.22409","type":"journal-article","created":{"date-parts":[[2007,2,2]],"date-time":"2007-02-02T23:52:27Z","timestamp":1170460347000},"page":"658-661","source":"Crossref","is-referenced-by-count":62,"title":["First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome"],"prefix":"10.1002","volume":"56","author":[{"given":"Donato","family":"Rigante","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ettore","family":"Capoluongo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Bertoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Ansuini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Chiaretti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Piastra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Pulitan\u00f2","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orazio","family":"Genovese","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adele","family":"Compagnone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Achille","family":"Stabile","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,1,30]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(84)92505-4"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/40.11.1285"},{"key":"e_1_2_6_4_2","first-page":"53","article-title":"The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age","volume":"10","author":"Stabile A","year":"2006","journal-title":"Eur Rev Med Pharmacol Sci"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.286.5446.1957"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1097\/00002281-200209000-00012"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.12.016"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(96)70160-9"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.85.5.421"},{"key":"e_1_2_6_10_2","first-page":"2081","article-title":"Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis","volume":"33","author":"Behrens EM","year":"2006","journal-title":"J Rheumatol"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-05-1980"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1532\/IJH97.04008"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2006.tb02228.x"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1067\/mai.2003.1455"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1007\/s102380200009"},{"key":"e_1_2_6_16_2","first-page":"2513","article-title":"Macrophage activation syndrome\u2014what's in a name","volume":"30","author":"Ramanan AV","year":"2003","journal-title":"J Rheumatol"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(96)70160-9"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V85.12.3586.bloodjournal85123586"},{"key":"e_1_2_6_19_2","first-page":"108","article-title":"Dramatic efficacy of cyclosporine A in macrophage activation syndrome","volume":"19","author":"Ravelli A","year":"2001","journal-title":"Clin Exp Rheumatol"}],"container-title":["Arthritis &amp; 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