{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T17:56:29Z","timestamp":1793642189060,"version":"4.5.1"},"reference-count":50,"publisher":"The Journal of Rheumatology","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2020,10,1]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>To clarify the prevalence, risk factors, outcome, and outcome-related factors of hemophagocytic lymphohistiocytosis (HLH) in patients with dermatomyositis (DM), polymyositis (PM), or clinically amyopathic dermatomyositis (CADM).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>Data of patients with DM, PM, or CADM who were admitted to the First Affiliated Hospital of Zhejiang University from February 2011 to February 2019 were retrospectively collected. Patients diagnosed with HLH constituted the case group. A 1:4 case-control study was performed to identify risk factors for HLH in patients with DM, PM, or CADM through comparison, univariate, and multivariate logistic regression analysis. Intragroup comparison was made among patients with HLH to identify factors influencing unfavorable short-term outcome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>HLH was a rare (4.2%) but fatal (77.8%) complication in patients with DM, PM, or CADM. The retrospective case-control study revealed that higher on-admission disease activity (p = 0.008), acute exacerbation of interstitial lung disease (AE-ILD, p = 0.002), and infection (p = 0.002) were risk factors for complication of HLH in patients with DM, PM, or CADM. The following intragroup comparison showed that higher on-admission disease activity (p = 0.035) and diagnosis of CADM (p = 0.039) might influence the short-term outcome of patients with HLH. However, no risk factor was identified after false discovery rate correction.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>In this study, secondary HLH was a fatal complication, with higher on-admission disease activity, AE-ILD, and infection working as risk factors. The underlying role of infection and autoimmune abnormality in HLH in connective tissue disease was subsequently noted. Clinical factors influencing the short-term outcome of patients with secondary HLH require further study.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.190542","type":"journal-article","created":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T16:19:13Z","timestamp":1569946753000},"page":"1532-1540","source":"Crossref","is-referenced-by-count":14,"title":["Hemophagocytic Lymphohistiocytosis: Prevalence, Risk Factors, Outcome, and Outcome-related Factors in Adult Idiopathic Inflammatory Myopathies"],"prefix":"10.3899","volume":"47","author":[{"given":"Junyu","family":"Liang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danyi","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chuanyin","family":"Sun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiqian","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heng","family":"Cao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin","family":"Lin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2019,10,1]]},"reference":[{"key":"2022062017050726000_47.10.1532.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2009.11.006"},{"key":"2022062017050726000_47.10.1532.2","doi-asserted-by":"publisher","DOI":"10.1038\/nrneurol.2011.63"},{"key":"2022062017050726000_47.10.1532.3","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1080\/14397595.2018.1467257","article-title":"Current diagnosis and treatment of polymyositis and dermatomyositis","volume":"28","author":"Sasaki","year":"2018","journal-title":"Mod Rheumatol"},{"key":"2022062017050726000_47.10.1532.4","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1002\/acr.22501","article-title":"A population-based study of infection-related hospital mortality in patients with dermatomyositis\/polymyositis","volume":"67","author":"Murray","year":"2015","journal-title":"Arthritis Care Res"},{"key":"2022062017050726000_47.10.1532.5","doi-asserted-by":"crossref","first-page":"e0192491","DOI":"10.1371\/journal.pone.0192491","article-title":"Hospitalization mortality and associated risk factors in patients with polymyositis and dermatomyositis: a retrospective case-control study","volume":"13","author":"Wu","year":"2018","journal-title":"PLoS One"},{"key":"2022062017050726000_47.10.1532.6","first-page":"e2472","article-title":"Infection-associated hemophagocytic lymphohistiocytosis: an unusual clinical masquerader","volume":"10","author":"Abbas","year":"2018","journal-title":"Cureus"},{"key":"2022062017050726000_47.10.1532.7","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.121233"},{"key":"2022062017050726000_47.10.1532.8","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1111\/bjh.14147","article-title":"Advances in the pathogenesis of primary and secondary haemophagocytic lymphohistiocytosis: differences and similarities","volume":"174","author":"Brisse","year":"2016","journal-title":"Br J Haematol"},{"key":"2022062017050726000_47.10.1532.9","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(13)61048-X"},{"key":"2022062017050726000_47.10.1532.10","doi-asserted-by":"crossref","first-page":"1753","DOI":"10.1111\/epi.14524","article-title":"Fueling the fires: hemophagocytic lymphohistiocytosis in febrile infection-related epilepsy syndrome","volume":"59","author":"Farias-Moeller","year":"2018","journal-title":"Epilepsia"},{"key":"2022062017050726000_47.10.1532.11","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502132920706"},{"key":"2022062017050726000_47.10.1532.12","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2002.120621"},{"key":"2022062017050726000_47.10.1532.13","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keg427"},{"key":"2022062017050726000_47.10.1532.14","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"2022062017050726000_47.10.1532.15","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201604-0801CI"},{"key":"2022062017050726000_47.10.1532.16","doi-asserted-by":"crossref","first-page":"1621","DOI":"10.21037\/jtd.2019.03.28","article-title":"Acute exacerbation of interstitial lung diseases secondary to systemic rheumatic diseases: a prospective study and review of the literature","volume":"11","author":"Manfredi","year":"2019","journal-title":"J Thorac Dis"},{"key":"2022062017050726000_47.10.1532.17","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/j.ijantimicag.2016.11.014","article-title":"Selective reporting of antibiotic susceptibility test results in European countries: an ESCMID cross-sectional survey","volume":"49","author":"Pulcini","year":"2017","journal-title":"Int J Antimicrob Agents"},{"key":"2022062017050726000_47.10.1532.18","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1111\/myc.12874","article-title":"Can intravenous antifungal therapy be safely used in the outpatient parenteral antimicrobial therapy (OPAT) setting?","volume":"62","author":"Rae","year":"2019","journal-title":"Mycoses"},{"issue":"Suppl","key":"2022062017050726000_47.10.1532.19","doi-asserted-by":"crossref","first-page":"S70","DOI":"10.1016\/j.ypmed.2012.12.003","article-title":"Risk factors for infection\/colonization caused by resistant gram negative bacilli in critically ill patients (an observational study of 1633 critically ill patients)","volume":"57","author":"Vasudevan","year":"2013","journal-title":"Prev Med"},{"key":"2022062017050726000_47.10.1532.20","doi-asserted-by":"crossref","first-page":"e309","DOI":"10.3928\/19382359-20170717-01","article-title":"An overview of hemophagocytic lymphohistiocytosis","volume":"46","author":"Esteban","year":"2017","journal-title":"Pediatr Ann"},{"key":"2022062017050726000_47.10.1532.21","doi-asserted-by":"crossref","first-page":"2297","DOI":"10.1002\/art.38672","article-title":"Clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome in adults","volume":"66","author":"Kumakura","year":"2014","journal-title":"Arthritis Rheumatol"},{"key":"2022062017050726000_47.10.1532.22","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.141261"},{"key":"2022062017050726000_47.10.1532.23","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-pathol-020117-043625"},{"key":"2022062017050726000_47.10.1532.24","doi-asserted-by":"publisher","DOI":"10.1111\/cei.13057"},{"key":"2022062017050726000_47.10.1532.25","doi-asserted-by":"crossref","first-page":"2839","DOI":"10.1007\/s10067-017-3830-3","article-title":"Macrophage activation syndrome in Still\u2019s disease: analysis of clinical characteristics and survival in paediatric and adult patients","volume":"36","author":"Ruscitti","year":"2017","journal-title":"Clin Rheumatol"},{"key":"2022062017050726000_47.10.1532.26","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmii.2011.01.027"},{"key":"2022062017050726000_47.10.1532.27","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1111\/bjh.13837","article-title":"Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years","volume":"172","author":"Schram","year":"2016","journal-title":"Br J Haematol"},{"key":"2022062017050726000_47.10.1532.28","doi-asserted-by":"crossref","first-page":"e373","DOI":"10.1016\/j.clml.2018.06.014","article-title":"Prognostic factors and outcomes in adults with secondary hemophagocytic lymphohistiocytosis: a single-center experience","volume":"18","author":"Apodaca","year":"2018","journal-title":"Clin Lymphoma Myeloma Leuk"},{"key":"2022062017050726000_47.10.1532.29","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/j.semarthrit.2015.07.004","article-title":"Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies","volume":"45","author":"Brito-Zeron","year":"2016","journal-title":"Semin Arthritis Rheum"},{"key":"2022062017050726000_47.10.1532.30","doi-asserted-by":"publisher","DOI":"10.1155\/2019\/2471215"},{"key":"2022062017050726000_47.10.1532.31","doi-asserted-by":"publisher","DOI":"10.1016\/S1473-3099(07)70290-6"},{"key":"2022062017050726000_47.10.1532.32","doi-asserted-by":"publisher","DOI":"10.1016\/j.micinf.2012.08.004"},{"key":"2022062017050726000_47.10.1532.33","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.170955"},{"key":"2022062017050726000_47.10.1532.34","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1038\/nrrheum.2018.33","article-title":"Update on outcome assessment in myositis","volume":"14","author":"Rider","year":"2018","journal-title":"Nat Rev Rheumatol"},{"key":"2022062017050726000_47.10.1532.35","first-page":"153","article-title":"Disease-specific quality indicators, outcome measures and guidelines in polymyositis and dermatomyositis","volume":"25","author":"Alexanderson","year":"2007","journal-title":"Clin Exp Rheumatol"},{"key":"2022062017050726000_47.10.1532.36","first-page":"222","article-title":"Increased visfatin levels are associated with higher disease activity in anti-Jo-1-positive myositis patients","volume":"34","author":"Hulejova","year":"2016","journal-title":"Clin Exp Rheumatol"},{"key":"2022062017050726000_47.10.1532.37","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.cyto.2018.12.023","article-title":"Pro-inflammatory S100A11 is elevated in inflammatory myopathies and reflects disease activity and extramuscular manifestations in myositis","volume":"116","author":"Andr\u00e9s Cerezo","year":"2019","journal-title":"Cytokine"},{"key":"2022062017050726000_47.10.1532.38","doi-asserted-by":"publisher","DOI":"10.1186\/ar3836"},{"key":"2022062017050726000_47.10.1532.39","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2172-14-9"},{"key":"2022062017050726000_47.10.1532.40","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/j.cyto.2017.08.019","article-title":"High levels of IL-6 and IL-8 characterize early-on idiopathic pulmonary fibrosis acute exacerbations","volume":"102","author":"Papiris","year":"2018","journal-title":"Cytokine"},{"key":"2022062017050726000_47.10.1532.41","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.90637.2008"},{"key":"2022062017050726000_47.10.1532.42","doi-asserted-by":"crossref","first-page":"176","DOI":"10.3389\/fmed.2017.00176","article-title":"Acute exacerbation in interstitial lung disease","volume":"4","author":"Leuschner","year":"2017","journal-title":"Front Med"},{"key":"2022062017050726000_47.10.1532.43","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0116775"},{"key":"2022062017050726000_47.10.1532.44","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.autrev.2016.09.016","article-title":"Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: analysis of 41 cases collected in 2 rheumatologic centers","volume":"16","author":"Ruscitti","year":"2017","journal-title":"Autoimmun Rev"},{"key":"2022062017050726000_47.10.1532.45","doi-asserted-by":"publisher","DOI":"10.1191\/0961203305lu2109oa"},{"key":"2022062017050726000_47.10.1532.46","first-page":"4159727","article-title":"Successful treatment of hemophagocytic lymphohistiocytosis associated with lupus nephritis by using mycophenolate mofetil","volume":"2017","author":"Nawata","year":"2017","journal-title":"Case Rep Rheumatol"},{"key":"2022062017050726000_47.10.1532.47","doi-asserted-by":"crossref","unstructured":"Chokshi B D\u2019Agati V Bizzocchi L Johnson B Mendez B Jim B . Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles. BMJ Case Rep 2019;12.","DOI":"10.1136\/bcr-2018-227860"},{"key":"2022062017050726000_47.10.1532.48","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-06-356261"},{"key":"2022062017050726000_47.10.1532.49","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1007\/s10875-017-0439-x","article-title":"A personalized diagnostic and treatment approach for macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis in adults","volume":"37","author":"Kumar","year":"2017","journal-title":"J Clin Immunol"},{"key":"2022062017050726000_47.10.1532.50","doi-asserted-by":"crossref","first-page":"1041","DOI":"10.1080\/1744666X.2017.1372194","article-title":"Advances in immunopathogenesis of macrophage activation syndrome during rheumatic inflammatory diseases: toward new therapeutic targets?","volume":"13","author":"Ruscitti","year":"2017","journal-title":"Expert Rev Clin Immunol"}],"container-title":["The Journal of Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.3899\/jrheum.190542","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,21]],"date-time":"2022-06-21T00:08:40Z","timestamp":1655770120000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.jrheum.org\/lookup\/doi\/10.3899\/jrheum.190542"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10,1]]},"references-count":50,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2020,10,1]]},"published-print":{"date-parts":[[2020,10,1]]}},"alternative-id":["10.3899\/jrheum.190542"],"URL":"https:\/\/doi.org\/10.3899\/jrheum.190542","relation":{},"ISSN":["0315-162X","1499-2752"],"issn-type":[{"value":"0315-162X","type":"print"},{"value":"1499-2752","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10,1]]}}}