Chronic inflammation and accelerated atherosclerosis as important cofactors in nephrogenic systemic fibrosis following intravenous gadolinium exposure.
Nephrogenic systemic fibrosis (NSF) is a rare disorder in patients with chronic kidney disease characterized by an increased tissue deposition of collagen. Its pathogenesis remains unclear. Prior studies indirectly suggested a possible impact of chronic inflammation and accelerated atherosclerosis--a common feature in kidney diseased patients--whereas recent data focused almost exclusively on gadolinium (Gd)-based MR contrast agents. Usually NSF develops a maximum of 2-3 months after Gd. Longer intervals have not yet been described. Therefore, we present the first case with an extraordinary long time course in terms of chronic inflammation. A 52-year-old Caucasian woman with end-stage renal disease was admitted to our hospital with progressive muscle weakness and skin induration resulting in growing immobility. Her past medical history revealed a secondary HPT, multiple vascular complications, a seronegative rheumatoid arthritis, and a pituitary gland adenoma. The latter conditions led to multiple MR examinations with Gd-based contrast agents, the last one more than 4 years ago. Numerous laboratory tests were performed including ESR, CRP, intact parathyroid hormone (iPTH), serum ferritin, cyclic-citrullinated peptide antibodies (CCP), ANA, ANCA, immunoelectrophoresis, and serology for hepatitis as well as human immunodeficiency virus. Eventually a skin biopsy of her left thigh was obtained. The laboratory investigation showed persistently elevated levels of CRP, ESR, serum ferritin, and iPTH, whereas all other parameters were inconspicuous. The hisology displayed typical signs of nephrogenic systemic fibrosis. NSF can occur at any time after Gd exposure in the long term. Gd is a necessary, but not the sole cause of NSF. Certain other cofactors such as chronic inflammation and accelerated atherosclerosis seem to be involved.
Grebe
Scott O
SO
Clinic of Internal Medicine, Section of Nephrology, HELIOS-Klinikum Wuppertal, University of Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Germany. sogrebe@t-online.de.
Borrmann
Marc
M
Clinic of Internal Medicine, Section of Nephrology, HELIOS-Klinikum Wuppertal, University of Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Germany.
Altenburg
Alexander
A
Department of Diagnostic and Interventional Radiology, HELIOS-Klinikum Wuppertal, University of Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Germany.
Wesselman
Ulrich
U
Clinic of Dermatology, HELIOS-Klinikum Wuppertal, University of Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Germany.
Hein
Dietmar
D
Clinic of Internal Medicine, Section of Nephrology, HELIOS-Klinikum Wuppertal, University of Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Germany.
Haage
Patrick
P
Department of Diagnostic and Interventional Radiology, HELIOS-Klinikum Wuppertal, University of Witten/Herdecke, Heusnerstr. 40, 42283, Wuppertal, Germany.
D050197:Atherosclerosis / Q000150:complications*; D002908:Chronic Disease; D003287:Contrast Media / Q000009:adverse effects*; D005260:Female; D005355:Fibrosis; D005682:Gadolinium / Q000009:adverse effects*; D006801:Humans; D007249:Inflammation / Q000150:complications*; D007674:Kidney Diseases / Q000139:chemically induced* / Q000175:diagnosis / Q000209:etiology*; D008875:Middle Aged; D013997:Time Factors
D003287:Contrast Media; D005682:Gadolinium