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

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

Affiliations
  • 1Division of Nephrology, Department of Internal Medicine, Hanyang University Guri Hospital, Guri, Korea
  • 2Department of Internal Medicine, Charm Clinic, Namyangju, Korea
  • 3Department of Dermatology, Hanyang University Guri Hospital, Guri, Korea
  • 4Department of Pathology, Hanyang University Guri Hospital, Guri, Korea

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.

Keyword

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

Figure

  • Fig. 1 Change in renal function and clinical course in our patient. The horizontal axis illustrates the chronological manifestation in the patient following gadolinium exposure. The vertical axis indicates patient renal function in terms of serum creatinine level. The eGFR is presented in brackets.eGFR = estimated glomerular filtration rate.aMeasurement in mg/dL (mL/min/1.73 m2).

  • Fig. 2 Clinical images of the lower extremities before treatment and six months after treatment. (A, B) Symmetrical edema with erythematous papules extending from the dorsum to the thigh. (C, D) Contracture of both ankles and hyperpigmentation of both anterior tibial lesions.The images are published under informed consent of the patient.

  • Fig. 3 Pathologic findings of the skin biopsy. (A) Thickened dermis with collagen deposits (H & E stain, × 40). (B) Collagen bundles in dermis with scant inflammatory cell infiltration and a well preserved adnexal structure (H & E stain, × 200). (C) The septal fibrosis of the subcutaneous fat, not accompanied by active inflammation or fat necrosis (H & E stain, × 200). (D) Infiltration of CD34 positive spindle cells.H & E stain = hematoxylin and eosin stain.


Reference

1. Daram SR, Cortese CM, Bastani B. Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: report of a new case with literature review. Am J Kidney Dis. 2005; 46(4):754–759. PMID: 16183432.
Article
2. Koo TH, Lee DH, Baek HK, Kim DK, Kim BK, Hong SH, et al. A case of nephrogenic systemic fibrosis following gadolinium exposure in a peritoneal dialysis patient. Korean J Med. 2010; 78(4):507–511.
3. Do JG, Kim YB, Lee DG, Hwang JH. A case of delayed onset nephrogenic systemic fibrosis after gadolinium based contrast injection. Ann Rehabil Med. 2012; 36(6):880–886. PMID: 23342325.
Article
4. Hong MH, Koo HM, Choi J, Ahn JR, Chon HJ, Kim C, et al. A case of nephrogenic systemic fibrosis after gadolinium-based contrast agent injection. Korean J Med. 2010; 78(1):127–131.
5. Levey AS, Stevens LA, Schmid CH, Zhang YL, Castro AF 3rd, Feldman HI, et al. A new equation to estimate glomerular filtration rate. Ann Intern Med. 2009; 150(9):604–612. PMID: 19414839.
Article
6. Davenport MS, McDonald CR, Asch D, Mervak B, Cavallo J, Mody R, et al. Chapter 15. Nephrogenic systemic fibrosis (NSF). ACR Manual on Contrast Media. Reston, VA: American College of Radiology;2020. p. 85–89.
7. Elmholdt TR, Jørgensen B, Ramsing M, Pedersen M, Olesen AB. Two cases of nephrogenic systemic fibrosis after exposure to the macrocyclic compound gadobutrol. NDT Plus. 2010; 3(3):285–287. PMID: 28657062.
Article
8. Sadowski EA, Bennett LK, Chan MR, Wentland AL, Garrett AL, Garrett RW, et al. Nephrogenic systemic fibrosis: risk factors and incidence estimation. Radiology. 2007; 243(1):148–157. PMID: 17267695.
Article
9. Rofsky NM, Sherry AD, Lenkinski RE. Nephrogenic systemic fibrosis: a chemical perspective. Radiology. 2008; 247(3):608–612. PMID: 18487530.
Article
10. Hubbard V, Davenport A, Jarmulowicz M, Rustin M. Scleromyxoedema-like changes in four renal dialysis patients. Br J Dermatol. 2003; 148(3):563–568. PMID: 12653751.
Article
11. Läuchli S, Zortea-Caflisch C, Nestle FO, Burg G, Kempf W. Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis. Dermatology. 2004; 208(3):278–280. PMID: 15118390.
Article
12. Tran KT, Prather HB, Cockerell CJ, Jacobe H. UV-A1 therapy for nephrogenic systemic fibrosis. Arch Dermatol. 2009; 145(10):1170–1174. PMID: 19841406.
Article
13. Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017; 76(8):1327–1339. PMID: 27941129.
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