Nephrogenic systemic fibrosis with a spectrum of clinical and histopathological presentation: a disorder of aberrant dermal remodeling

Deng, April; Martin, Donna Bilu; Spillane, Anne P.; Chwalek, Jennifer; Surin-Lord, S St; Brooks, Sara; Petrali, John P.; Sina, Bahram; Gaspari, Anthony A.; Kao, Grace F.

doi:10.1111/j.1600-0560.2009.01301.x

Cited by 29 publications

(15 citation statements)

References 16 publications

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“…Taken together, our findings suggest that a process of aberrant reparative process is occurring in the skin lesions of patients with NSF 11 . This reparative process can be seen in the findings of accumulation of collagen and elastin (‘elastocollagenous balls'), sclerotic collagen bodies as well as areas of osseous metaplasia in skin biopsies of patients with late‐stage NSF.…”

Section: Discussionsupporting

confidence: 53%

Findings of osseous sclerotic bodies: a unique sequence of cutaneous bone formation in nephrogenic systemic fibrosis

Kartono

Basile

Roshdieh³

et al. 2010

Journal of Cutaneous Pathology

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Nephrogenic systemic fibrosis (NSF) is a progressive and potentially fatal fibrosing skin disorder found mainly in patients with renal insufficiency. NSF is characterized by thickened and hyperpigmented skin lesions with or without systemic involvement. In essentially all patients, this disease entity has been associated with the administration of gadolinium contrast agents for imaging purposes. Microscopic recognition of this entity remains challenging, as the diagnosis is based on various clinical and histopathologic features that overlap with other fibrosing disorders. No single feature is absolutely specific for NSF. We report a finding of osseous sclerotic bodies with elastin trapping appearing on histopathology in the clinical setting of NSF with hemodialysis-dependent renal failure. Our report of an additional attribute indicative of NSF may aid in making the diagnosis.

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Section: Discussionsupporting

confidence: 53%

Findings of osseous sclerotic bodies: a unique sequence of cutaneous bone formation in nephrogenic systemic fibrosis

Kartono

Basile

Roshdieh³

et al. 2010

Journal of Cutaneous Pathology

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“…NSF is undeniably a rare but devastating disease, presenting with a spectrum of clinical and histopathologic manifestations (2,3). It is associated with increased tissue deposition of collagen in the skin, subcutaneous fascia, striated muscles, joints, diaphragm, lungs, liver, and myocardium (4).…”

Section: Discussionmentioning

confidence: 99%

Low Risk for Nephrogenic Systemic Fibrosis in Nondialysis Patients Who Have Chronic Kidney Disease and Are Investigated with Gadolinium-Enhanced Magnetic Resonance Imaging

Chrysochou

Power²,

Shurrab³

et al. 2010

Clinical Journal of the American Society of Nephrology

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Background and objectives: During the past decade, nephrogenic systemic fibrosis (NSF) has been reported in patients who have severe renal impairment and have been exposed to a gadolinium (Gd)-based contrast agent during magnetic resonance imaging (MRI). As a result of positive reporting bias, many suitable patients with chronic kidney disease (CKD) are being denied a highly important form of investigation that can be safely undertaken. We analyzed the safety of Gd-MRI in patients with CKD and varying levels of estimated GFR (eGFR).Design Conclusions: In this study, no patients developed NSF during extended follow-up, even after multiple Gd doses in some. Gd-MRI can be safely undertaken in the majority of patients with CKD, but caution is merited for dialysis patients and those with acute kidney injury, with relative caution for predialysis patients with stage 5 CKD.

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“…There have also been a few case reports in which exposure to GBCA could not be confirmed from patients' medical records [43]. Recently, Deng et al [44] reported seven NSF patients; although six patients had no known exposure history to GBCA, tissue gadolinium levels tested for three of these patients were significantly increased compared with normal tissue, supporting the association of GBCA and NSF. Without examining affected tissue for the presence of gadolinium, a history of exposure to GBCA cannot be excluded with certainty, since documentation of contrast media use is often inadequate [45].…”

Section: Discussionmentioning

confidence: 94%

Nephrogenic systemic fibrosis: risk factors suggested from Japanese published cases

Tsushima

Kanal²,

Thomsen³

2010

BJR

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Nephrogenic systemic fibrosis with a spectrum of clinical and histopathological presentation: a disorder of aberrant dermal remodeling

Cited by 29 publications

References 16 publications

Findings of osseous sclerotic bodies: a unique sequence of cutaneous bone formation in nephrogenic systemic fibrosis

Findings of osseous sclerotic bodies: a unique sequence of cutaneous bone formation in nephrogenic systemic fibrosis

Low Risk for Nephrogenic Systemic Fibrosis in Nondialysis Patients Who Have Chronic Kidney Disease and Are Investigated with Gadolinium-Enhanced Magnetic Resonance Imaging

Nephrogenic systemic fibrosis: risk factors suggested from Japanese published cases

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