Guideline sources The following summarized guidelines for the evaluation and management of nephrogenic systemic fibrosis are prepared by our editorial team based on guidelines from the European Academy of Dermatology and Venereology (EADV 2017). 1 Guidelines 1. Screening and diagnosis Etiology: recognize that nephrogenic systemic fibrosis is an iatrogenic condition observed in patients with end-stage renal failure and is associated with gadolinium exposure. E 2. Medical management Medical therapy: insufficient evidence to recommend any treatment in patients with nephrogenic systemic fibrosis. I 3. Preventative measures Avoidance of gadolinium-containing contrast agents: https://web.pathway.md/diseases/recJm8m2f5f6PTUJR 1/2 6/30/23, 1:16 AM Nephrogenic systemic fibrosis Pathway Avoid using high-risk gadolinium-based contrast agents, especially in patients with an eGFR of < 30 mL/min, for the prevention of nephrogenic systemic fibrosis. D Use a low-risk gadolinium medium and reduce the dose of the gadolinium-based contrast agents to the minimum effective dosage for imaging if MRI with gadolinium-based contrast agents is indispensable. E Dialysis: perform at least one full 4-hour dialysis session in patients with renal insufficiency after gadolinium-based contrast agent-based MRI, based on the dialysability of gadolinium-based contrast agents. E Clinical findings Symptoms Past medical history Burning sensation CKD Itching Exposure to gadolinium-based contrast agents Integument exam Hyperpigmentation Peau d'orange Skin atrophy Skin edema Skin induration Skin papules Subcutaneous nodules References 1. R Knobler, P Moinzadeh, N Hunzelmann et al. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2 Scleromyxedema, scleredema and nephrogenic systemic fibrosis. J Eur Acad Dermatol Venereol. 2017 Oct;31 10 1581 1594. Open 2. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open https://web.pathway.md/diseases/recJm8m2f5f6PTUJR 2/2