A markedly elevated serum ferritin level has been associated with inflammatory conditions such as adult-onset Still’s disease, systemic juvenile idiopathic arthritis, and hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Hyperferritinemia, however, can also be caused by a wide variety of disparate conditions, often with impressively high serum levels. The objective of this analysis was to investigate the underlying etiology of markedly elevated ferritin levels in a large group of patients treated as outpatients and inpatients in a tertiary-care medical center.
Data of all adult patients from 2008 through 2010 with at least 1 serum ferritin level greater than 1000 μg/L were reviewed. If a patient had multiple qualifying levels, the highest one was used. For each case, the most likely cause of the elevated ferritin was assessed based on the available clinical data using a simple algorithmic approach.
Six hundred twenty-seven patients were found. The average serum ferritin level was 2647 μg/L. The most frequent condition was malignancy (153/627), with iron-overload syndromes the second most common (136/627). There were 6 cases of adult-onset Still’s disease, systemic juvenile idiopathic arthritis, or hemophagocytic lymphohistiocytosis/macrophage activation syndrome. The average ferritin level in these syndromes was 14242 μg/L. Seven patients appeared to have anemia of chronic inflammation, and in 5 patients, there was no clearly definable cause for hyperferritinemia.
Although extremely elevated ferritin levels may be associated with rheumatologic diseases, more often they are found in patients with other conditions such as malignancy or infection. In addition, extremely high ferritin levels can be found in patients with seemingly indolent disease or levels of chronic inflammation.
Only 6 of 627 patients with elevated ferritin levels greater than 1000 uG/L had adult onset Still disease or macrophage activation syndrome.
From the Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN.
This study has no sources of support.
The authors declare no conflict of interest.
Reprints: Howard Fuchs, MD, Division of Rheumatology and Immunology, Vanderbilt University Medical Center, S-3113 MCN, Nashville, TN 37232.
Correspondence: Charles Moore, Jr, MD, Division of Rheumatology and Immunology, Vanderbilt University Medical Center, S-3113 MCN, Nashville, TN 37232. E-mail: Charles.email@example.com.