Background/Purpose: Clinical associations of hyperferritinemia include: hepatocellular injury, hemophagocytic lymphohistiocytosis (HLH), hematologic malignancy, adult-onset Still’s disease (AOSD), and iron overload. Fever of undetermined origin (FUO) may or may not co-exist with hyperferritinemia in adults. The aim of this study was to describe the associations of with hyperferritinemia and FUO in adults.

Methods: We retrospectively identified patients >16 years old with serum ferritin levels > 5,000 μg/L at a tertiary medical center in Japan between 2002-2014. FUO was defined as temperature>38.3℃ with etiology undetermined after 3 days of in-hospital evaluation. Data were collected on clinical features, medications and mortality.

Results: We identified 156 Japanese patients with hyperferritinemia. Median age was 66 years (range, 16-95); 47% were female. Median serum ferritin level was 9,369μg/L (range, 5,000-170,202); Mortality during the admission was 42%. FUO was absent in 118/156 and present in 38/156 patients (Table). The most common diagnosis was hepatocellular injury, but this was rarely seen with FUO. Among those with FUO and elevated ferritin, hematologic malignancies, HLH, AOSD, and lupus (SLE) were diagnosed. HLH and AOSD remains most prevalent for patients with serum ferritin>10,000μg/L and FUO.

Conclusion: Although hyperferritinemia commonly indicates liver disease and hemochromatosis, clinicians should be aware that hyperferritinemia in conjunction with FUO is a reliable marker for more complex diagnoses such as HLH, AOSD or SLE, especially when ferritin levels are extremely high.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/hyperferritinemia-and-fever-in-adults/