Nephrogenic fibrosing dermopathy

Ragunatha, S; Palit, Aparna; Inamadar, Arun C; Madraki, R; Yelikar, B R

doi:10.4103/0378-6323.45224

Cited by 4 publications

(3 citation statements)

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“…[5] It is chiefly seen in patients of chronic kidney disease, those on hemodialysis and those who have had a history of exposure to gadolinium-based contrast media. [7] Many endogenous as well as exogenous factors have been implicated in the causation of NSF.…”

Section: Etiopathogenesis [Figure 1]mentioning

confidence: 99%

“…Endogenous factors include thrombotic events, hypercoaguable states (antiphospholipid antibody syndrome, anticardiolipin antibody), chronic hepatitis C infection, anasarca, and calciphylaxis. [58] Exogenous factors that are hypothesized to initiate the process of this disorder are — erythropoietin, surgical intervention, vascular procedures, peritoneal dialysis, hemodialysis, and the use of gadolinium chelate contrast media during imaging studies. [9] A putative role of endothelial cell damage and toxic substances is proposed, but it is yet to be established.…”

Section: Etiopathogenesis [Figure 1]mentioning

confidence: 99%

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Nephrogenic systemic fibrosis

Madke

Khopkar

2011

Indian Dermatol Online J

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Nephrogenic systemic fibrosis (NSF) is a relatively new fibrosing disorder which has caught the attention of various specialities in the past decade. NSF is an extremely disabling and often painful condition, affecting up to 13% of the individuals with chronic kidney disease. The administration of a gadolinium chelate contrast agent has been reported to induce the development of NSF, particularly in patients who have acute or chronic renal disease with a glomerular filtration rate (GFR) lower than 30-mL/min/1.73 m2 and in those with acute renal insufficiency. Mass spectroscopy studies have demonstrated particles of gadolinium in the lesional tissue. The exact pathogenesis of this curious sclerosing condition is unknown. The role of the aberrant targeting of ‘circulating fibrocytes’ to the peripheral tissues and viscera has been hypothesized. NSF has distinct clinicopathological features in the setting of renal failure and needs to be looked upon as a new entity on the block. The condition is characterized by irregular indurated plaques, with amoeba-like projections and islands of sparing, chiefly on the trunk and extremities. Flexion contractures of fingers, knees, and elbow joints are known to occur in advanced cases of NSF. The course is frequently associated with painful episodes and loss of ambulation. Histopathology shows haphazard arrangement of thickened bundles of collagen, varying amount of mucin, and increased population of fibroblast-like cells in the dermis. Immunohistochemistry shows increased deposition of type-I procollagen and CD 34+ cells having fibroblastic activity. The condition is refractory to treatment with corticosteroids and immunosuppressive agents. Various modalities of therapy such as UVA1 phototherapy, imatinib mesylate, photodynamic therapy, plasmapheresis, extracorporeal photochemotherapy, and high-dose intravenous immunoglobulin have shown a moderate degree of improvement in skin thickness scores. A prudent option is restoration of renal function to normalcy via renal transplantation but to date the outcome of renal transplantation is unknown.

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Section: Etiopathogenesis [Figure 1]mentioning

confidence: 99%

Section: Etiopathogenesis [Figure 1]mentioning

confidence: 99%

Nephrogenic systemic fibrosis

Madke

Khopkar

2011

Indian Dermatol Online J

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“…Subsequently, a couple of cases have been reported from different parts of India, one of which provides a clear-cut evidence of the role of a GBCA (Omniscan),[9] while the other gives a negative history of such exposure. [10]…”

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confidence: 99%

Nephrogenic systemic fibrosis: Time for the requiem?

Panda¹

2011

Indian J Dermatol

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

Farooqi

Mumtaz

Arif

et al. 2023

IJNRD

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Aim 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.

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Nephrogenic fibrosing dermopathy

Cited by 4 publications

References 0 publications

Nephrogenic systemic fibrosis

Nephrogenic systemic fibrosis

Nephrogenic systemic fibrosis: Time for the requiem?

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

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