Hemophagocytic lymphohistiocytosis: Prevalence, risk factors, outcome, and outcome-related factors in adult idiopathic inflammatory myopathies
The Journal of Rheumatology Oct 12, 2020
Liang J, Xu D, Sun C, et al. - Researchers conducted the study for clarifying 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). 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 have been retrospectively gathered. In patients with DM, PM, or CADM, HLH was a rare (4.2%) but fatal (77.8%) complication. Secondary HLH was a fatal complication in this study, with higher on-admission disease activity, AE-ILD, and infection working as risk factors. Subsequently, the underlying role of infection and autoimmune abnormality in HLH was noted in connective tissue disease. Further analysis is expected of clinical factors affecting the short-term outcome of patients with secondary HLH.
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