Nephrogenic Fibrosing Dermopathy/Nephrogenic Systemic Fibrosis in 2 Patients With End-Stage Renal Disease on Hemodialysis

Kim, Richard H.; Ma, Lin; Hayat, Samina; Ahmed, Mariam

doi:10.1097/01.rhu.0000222053.47708.dd

Cited by 13 publications

(5 citation statements)

References 14 publications

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“…This was demonstrable in both renal insufficiency and normal renal function. Multinucleated giant cells, similar to those observed in humans (Kim et al, 2006), were found in the dermis using this model. The morphology of the electron-dense deposits was similar to what has been described for gadolinium oxide in simulated phagolysosomal fluid in vitro (Li et al, 2014).…”

Section: Discussionsupporting

confidence: 85%

Nephrogenic Systemic Fibrosis Is Mediated by Myeloid C-C Chemokine Receptor 2

Drel

Tan

et al. 2019

Journal of Investigative Dermatology

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Gadolinium-based contrast agents are implicated in several pathologic abnormalities (long-term retention in vital organs such as the skin and the brain) and are the cause of a sometimes fatal condition in patients, nephrogenic systemic fibrosis. Bone marrowederived fibrocytes and the monocyte chemoattractant protein-1 inflammatory pathway have been implicated as mediators of the adverse effects induced by gadolinium-based contrast agents. Mechanistic studies are scant; therefore, a mouse model of nephrogenic systemic fibrosis was established. Dermal cellularity was increased in contrast-treated green fluorescent protein (GFP) chimeric mice. GFP in the skin and fibrosis were increased in the contrast-treated chimeric animals. Monocyte chemoattractant protein-1 and CC chemokine receptor 2 were increased in the tissues from contrast-treated mice. CC chemokine receptor 2edeficient recipients of GFP-expressing marrow had an abrogation of gadolinium-induced pathology and displayed less GFP-positive cells in the skin. Wild-type animals that received CC chemokine receptor 2edeficient bone marrow had a complete abrogation of dermal pathology. That GFP levels and expression increase in the skin, in tandem with a fibrocyte marker, supports the blood-borne circulating fibrocyte hypothesis of the disease. As of now, fibrocyte trafficking has yet to be demonstrated. Importantly, our data demonstrate that the monocyte chemoattractant protein-1/C-C chemokine receptor 2 axis plays a critical role in the pathogenesis of nephrogenic systemic fibrosis.

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Section: Discussionsupporting

confidence: 85%

Nephrogenic Systemic Fibrosis Is Mediated by Myeloid C-C Chemokine Receptor 2

Drel

Tan

et al. 2019

Journal of Investigative Dermatology

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“… 29 described a case report of four patients with features similar, but not identical to scleromyxoedema whereas a later follow‐up study in 2008 confirmed the diagnosis of NSF 30 . Thus, we believe that the participation of a skilled dermatologist or rheumatologist with specialist knowledge of scleroderma diseases is required in the determination of this disease to avoid diagnostic misclassification 31–33 …”

Section: Discussionmentioning

confidence: 97%

Nephrogenic systemic fibrosis is found only among gadolinium‐exposed patients with renal insufficiency: a case–control study from Denmark

Elmholdt

Pedersen

Jørgensen³

et al. 2011

British Journal of Dermatology

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Our study supports the view that GBCA is a major risk factor for NSF. Importantly, we found that patients with CKD stage 3 and 4 can be at risk of NSF. NSF may also be triggered by macrocyclic GBCA. Further, we observed a trend for higher phosphate levels in NSF cases compared with controls. The important findings drawn from this case-control study indicate that NSF is not an overlooked condition among patients with renal insufficiency not exposed to GBCA.

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“…Often the cells are described as being spindle shaped (61,77), fibroblasts (6), fibroblast like (56), fibrohistiocytic (68,75,82), or "polygonal epitheliod fibroblasts" (6). With the use of light microscopy, the dermal collagen is notably increased (27), with bundles haphazardly arranged (56) or "disorganized" (48). Mucin may be present (29,55,61,105), but is not required for the diagnosis (59).…”

Section: Histology and Immunohistologymentioning

confidence: 99%