Late Onset Nephrogenic Systemic Fibrosis in a Patient with Stage 3 Chronic Kidney Disease: a Case Report

J Korean Med Sci. 2020 Sep 7;35(35):e293. doi: 10.3346/jkms.2020.35.e293.

Abstract

Nephrogenic systemic fibrosis (NSF) is a progressive systemic fibrosing disease that may occur after gadolinium contrast exposure. It can lead to severe complications and even death. NSF is highly prevalent among patients with advanced chronic kidney disease (CKD). In this report, however, we describe the case of a patient with NSF that occurred during early CKD. A 65-year-old man with stage 3a CKD was transferred to our hospital because of lower extremity edema. The medical history revealed that he was exposed to gadolinium 185 days earlier, and the result of his tibial skin biopsy was consistent with NSF. The patient underwent a combined therapy with ultraviolet-A1 phototherapy and methotrexate and steroid therapy for 6 months. The combined therapy stopped the systemic progression of NSF.

Keywords: Chronic Kidney Disease; Gadolinium-based Contrast Agents; Nephrogenic Systemic Fibrosis.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Contrast Media / adverse effects
  • Contrast Media / chemistry
  • Dermatologic Agents / therapeutic use
  • Disease Progression
  • Gadolinium / chemistry
  • Glomerular Filtration Rate
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Methotrexate / therapeutic use
  • Nephrogenic Fibrosing Dermopathy / diagnosis*
  • Nephrogenic Fibrosing Dermopathy / etiology
  • Nephrogenic Fibrosing Dermopathy / therapy
  • Renal Insufficiency, Chronic / pathology*
  • Severity of Illness Index
  • Skin / pathology
  • Ultraviolet Therapy

Substances

  • Contrast Media
  • Dermatologic Agents
  • Gadolinium
  • Methotrexate