Hemophagocytic lymphohistiocytosis (HLH) is an aggressive hematologic disorder involving hyperstimulation of immune responses and severe inflammation. HLH has been well documented in lymphoid cancers and leukemias, but more rarely in solid tumors. The non-specific clinical characteristics of HLH can cause a diagnostic dilemma and delay in proper treatment, resulting in poor outcomes.
View Article and Find Full Text PDFObjective: Systemic juvenile idiopathic arthritis (JIA) is characterized by fevers, rash, and arthritis, for which interleukin-1 (IL-1) and IL-6 inhibitors appear to be effective treatments. Pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and alveolar proteinosis (AP) have recently been reported with increased frequency in systemic JIA patients. Our aim was to characterize and compare systemic JIA patients with these complications to a larger cohort of systemic JIA patients.
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