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

• Lim YJ
• Bang J
• Ko Y
• Seo HM
• Jung WY
• Yi JH
• Han SW
• Yu My

• KMID: 2506087
• DOI: http://doi.org/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.

Keyword

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

Figure

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  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).

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  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.

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  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.

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