Scleromyxoedema-like cutaneous diseases in renal-dialysis patients

Cowper, Shawn E.; Robin, H; Steinberg, Steven M.; Su, Lyndon D.; Gupta, Samardeep; LeBoit, Philip E.

doi:10.1016/s0140-6736(00)02694-5

Cited by 830 publications

(604 citation statements)

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Section: Fibrocytes and Fibroblasts In Nsfmentioning

confidence: 99%

“…Fibrocytes or ''spindle'' cells are abundant and very closely associated with the developing fibrosis (1). Fibrocytes are a class of bone marrow-derived leukocyte subpopulation that display a distinctive phenotype with surface expression of procollagen, vimentin, and CD34 and are capable of specifically entering and localizing to tissue injury sites (1,16). Although fibrocytes are thought to account for as many as 10% of cells that are recruited to sites of acute tissue injury (16), it is not known whether these cells initiate or support the development of the fibrotic lesions associated with NSF.…”

Section: Fibrocytes and Fibroblasts In Nsfmentioning

confidence: 99%

“…NEPHROGENIC SYSTEMIC FIBROSIS (NSF) is a rare but potentially serious acquired systemic disease initially described as a form of scleromyxedema (1). To date, it has only been reported in patients with severely impaired renal function (SIRF), endstage renal disease (ESRD), and those in acute renal failure (ARF) (2)(3)(4)(5).…”

mentioning

confidence: 99%

“…The many other risk factors thought to be associated with NSF include: edema (6), metabolic acidosis, thrombotic events, high-dose erythropoietin (EPO) (7,8), systemic inflammation (9), recent surgery, kidney disease (10), and gadolinium (Gd 3þ )-based contrast agent (GBCA) exposure (11,12), especially when used at relatively high dose GBCA (5,13). NSF, which was first recognized by Cowper et al (1) and linked to GBCA in 2006 (11), is characterized by induration, thickening, and tightening of the skin. The typical description is of brawny erythematous skin with a peau d'orange appearance.…”

mentioning

confidence: 99%

“…The typical description is of brawny erythematous skin with a peau d'orange appearance. Distal parts of the body are usually most affected (1,14) but the trunk as well as internal organs such as the lungs, heart, liver, kidneys, skeletal muscle, and diaphragm can also develop lesions. Differential diagnosis includes scleromyxedema, eosinophilic fasciitis, calciphylaxis, cellulitis, and panniculitis (1).…”

mentioning

confidence: 99%

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Mechanism of NSF: New evidence challenging the prevailing theory

Newton¹,

Jimenez

2009

Magnetic Resonance Imaging

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Nephrogenic systemic fibrosis (NSF) has been associated with the administration of gadolinium‐based contrast agents in patients with severely impaired renal function (SIRF), endstage renal disease (ESRD), or acute renal failure (ARF). Since the vast majority of these patients do not get NSF, it is highly likely that patient factors play a role in its development. Although “free” or dechelated gadolinium is thought by some to be the only trigger of NSF, recent evidence suggests that chelated gadolinium may be important. Chelated gadolinium such as Omniscan (gadodiamide) and Magnevist (gadopentetate) can directly stimulate macrophages and monocytes in vitro to release profibrotic cytokines and growth factors capable of initiating and supporting the tissue fibrosis that is characteristic of NSF. In addition, an effect of chelated gadolinium on fibroblasts has also been demonstrated. Chelated gadolinium in the form of Omniscan, Magnevist, MultiHance, and ProHance increased proliferation of human dermal fibroblasts. Indeed, increased numbers of macrophages, together with activated fibroblasts and fibrocytes, are essential cells in the fibrotic process and are present in NSF skin. Accordingly, it is important that chelated gadolinium, in combination with patient cofactors, is considered in the etiology of NSF associated with enhanced scans. J. Magn. Reson. Imaging 2009;30:1277–1283. © 2009 Wiley‐Liss, Inc.

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Section: Fibrocytes and Fibroblasts In Nsfmentioning

confidence: 99%

Section: Fibrocytes and Fibroblasts In Nsfmentioning

confidence: 99%