Nephrogenic Systemic Fibrosis Is Mediated by Myeloid C-C Chemokine Receptor 2

J Invest Dermatol. 2019 Oct;139(10):2134-2143.e2. doi: 10.1016/j.jid.2019.03.1145. Epub 2019 Apr 9.

Abstract

Gadolinium-based contrast agents are implicated in several pathologic abnormalities (long-term retention in vital organs such as the skin and the brain) and are the cause of a sometimes fatal condition in patients, nephrogenic systemic fibrosis. Bone marrow-derived fibrocytes and the monocyte chemoattractant protein-1 inflammatory pathway have been implicated as mediators of the adverse effects induced by gadolinium-based contrast agents. Mechanistic studies are scant; therefore, a mouse model of nephrogenic systemic fibrosis was established. Dermal cellularity was increased in contrast-treated green fluorescent protein (GFP) chimeric mice. GFP in the skin and fibrosis were increased in the contrast-treated chimeric animals. Monocyte chemoattractant protein-1 and C-C chemokine receptor 2 were increased in the tissues from contrast-treated mice. C-C chemokine receptor 2-deficient recipients of GFP-expressing marrow had an abrogation of gadolinium-induced pathology and displayed less GFP-positive cells in the skin. Wild-type animals that received C-C chemokine receptor 2-deficient bone marrow had a complete abrogation of dermal pathology. That GFP levels and expression increase in the skin, in tandem with a fibrocyte marker, supports the blood-borne circulating fibrocyte hypothesis of the disease. As of now, fibrocyte trafficking has yet to be demonstrated. Importantly, our data demonstrate that the monocyte chemoattractant protein-1/C-C chemokine receptor 2 axis plays a critical role in the pathogenesis of nephrogenic systemic fibrosis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Animals
  • Biopsy, Needle
  • Cell Movement
  • Chemokines, CC / metabolism*
  • Contrast Media / adverse effects
  • Contrast Media / pharmacology
  • Disease Models, Animal
  • Female
  • Fibroblasts / cytology
  • Fluorescent Antibody Technique / methods
  • Gadolinium / adverse effects*
  • Gadolinium / pharmacology
  • Humans
  • Immunoblotting / methods
  • Immunohistochemistry
  • Mice
  • Mice, Inbred C57BL
  • Nephrogenic Fibrosing Dermopathy / chemically induced*
  • Nephrogenic Fibrosing Dermopathy / pathology*
  • Nephrogenic Fibrosing Dermopathy / physiopathology
  • Random Allocation
  • Receptors, CCR2 / metabolism*
  • Reference Values
  • Sensitivity and Specificity

Substances

  • Chemokines, CC
  • Contrast Media
  • Receptors, CCR2
  • Gadolinium