International Journal of Nephrology and Renovascular Disease (Jan 2023)

The Clinical Manifestations and Efficacy of Different Treatments Used for Nephrogenic Systemic Fibrosis: A Systematic Review

  • Farooqi S,
  • Mumtaz A,
  • Arif A,
  • Butt M,
  • Kanor U,
  • Memoh S,
  • Qamar MA,
  • Yosufi A

Journal volume & issue
Vol. Volume 16
pp. 17 – 30

Abstract

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Shaheer Farooqi,1 Afshan Mumtaz,2 Aabiya Arif,1 Mehwish Butt,3 Una Kanor,4 Samuel Memoh,5 Mohammad Aadil Qamar,1 Abubakr Yosufi6 1Department of Medicine, Ziauddin University, Karachi, Pakistan; 2Department of Medicine and Allied, Karachi Medical and Dental College, Karachi, Pakistan; 3Department of Medicine and Allied, Jinnah Medical and Dental college, Karachi, Pakistan; 4Department of Medicine, Bogomolets National Medical University, Kyiv, Ukraine; 5Department of Medicine, Windsor University School of Medicine, Cayon, Saint Kitts and Nevis; 6Medical School, Kabul University of Medical Sciences, Kabul, AfghanistanCorrespondence: Abubakr Yosufi, Kabul University of Medical Sciences, Kabul, Afghanistan, Tel +93 747236767, Email [email protected]: Nephrogenic systemic fibrosis (NSF) is a rare disorder that occurs in association majorly with chronic kidney disease (CKD). The lack of collective quantitative data on its clinical manifestations and the different treatment options’ efficacy, call the need for our investigation.Methods: A systematic review was conducted covering a timeline from inception up to July 2022 without any restrictions. Article screening and data extraction were performed independently on PubMed, Google Scholar, ScienceDirect, and Cochrane Library. The keywords that we used were CKD, NSF, Gadolinium enduced fibrosis, etc; shortlisted articles were assessed for risk of bias. Data were presented as frequencies and percentages, with a confidence interval of 95%. A chi-square test was also done to find significant relationships, with a p-value < 0.05 considered significant.Results: We had 83 patients in this review consisting of 44 (55.7%) females with a mean age of 51.4± 14.6 years. Sixty-nine (83.1%) patients had chronic kidney disease predisposition to NSF. Previous exposure to gadolinium-based contrast dyes was seen in 66 (79.5%) patients). The most common symptom in patients was cutaneous lesions in 69 (83.1%) patients. The most used treatments were ultraviolet therapy, renal transplant, and extracorporeal photopheresis; in 13.3% of the patients each. Condition in most patients either improved (67.1%) or remained stable (11.8%). Chi-square testing found that the treatments offered were also seen to be significantly related to outcome (p=0.015).Conclusion: The findings in this study provide a quantitative measurement of NSF’s presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF.Keywords: chronic kidney disease, fibrosis, scleroderma, renal impairment, CKD

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