Nephrogenic Fibrosing Dermopathy: Are ACE Inhibitors the Missing Link?

Fazeli, Amin; Lio, Peter A.; Liu, Vincent

doi:10.1001/archderm.140.11.1401

Cited by 37 publications

(22 citation statements)

References 3 publications

(5 reference statements)

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“…Angiotensin-Converting Enzyme inhibitors have anti-fibrogenic properties and their absence may promote the development of NSF [26]. However, this has not been a consistent finding in patients with NSF [8].…”

Section: What Is the Pathophysiology Of Nsf?mentioning

confidence: 99%

Nephrogenic Systemic Fibrosis: More Questions and Some Answers

Morcos

Thomsen

2008

Nephron Clin Pract

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Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder that may develop in patients who have advanced reduction in renal function. A causal relation between gadolinium (Gd³⁺)-based contrast agents (Gd-CA) and NSF is probable and is supported by the accumulating data in the literature. From those data, the prevalence of NSF is seen to be significantly higher after exposure to gadodiamide than any other gadolinium-based agent. Gd-CA are either linear or macrocyclic chelates and are available as ionic or non-ionic preparations. The molecular structure, whether cyclic or linear, and the ionicity determine the stability of Gd-CA. Linear chelates are flexible open chains which do not offer a strong binding to Gd³⁺. In contrast, the macrocyclic chelates offer a strong binding to Gd³⁺ by the virtue of being pre-organised rigid rings of almost optimal size to cage the Gd³⁺ atom. Non-ionic preparations are also less stable in comparison to the ionic ones, as the binding between Gd³⁺ and the negatively charged carboxyl groups is stronger than that with amides or alcohol in the non-ionic preparations. According to stability constants and kinetic measurements, the most stable Gd-CA is the ionic-macrocyclic chelate Gd-DOTA and the least stable agents are the non-ionic linear chelates gadodiamide and gadoversetamide. The stability of Gd-CA seems to be an important factor in the pathogenesis of NSF. Gd-CA of low stability are likely to undergo transmetallation and release free Gd ions that may deposit in tissues and attract circulating fibrocytes to initiate the process of fibrosis. There have been no cases of NSF reported in the peer-reviewed literature after the exclusive use of the stable macrocyclic Gd-CA. This minireview covers the clinical and pathological features of NSF and updates the current understanding of the pathophysiology of this condition.

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Section: What Is the Pathophysiology Of Nsf?mentioning

confidence: 99%

Nephrogenic Systemic Fibrosis: More Questions and Some Answers

Morcos

Thomsen

2008

Nephron Clin Pract

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“…Its use dramatically increased after the publication of the Dialysis Outcomes Quality Initiative guidelines for anemia management in 1997 (http://www.kidney.org/professionals/KDOQI) (10). Alternatively, a report correlating the decreasing plasma levels of transforming growth factor (TGF)-␤ after plasmapheresis with the amelioration of clinical signs of NSF in a few patients (11) is consistent with the hypothesis that a lack of treatment with an angiotensin converting enzyme (ACE) inhibitor may at least partially be linked to the development of this disease (12).…”

Section: Etiology and Pathogenesis Of Nsfmentioning

confidence: 79%

Safety update on the possible causal relationship between gadolinium‐containing MRI agents and nephrogenic systemic fibrosis

Pedersen

2007

Magnetic Resonance Imaging

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“…Los inhibidores de la calcineurina han sido implicados en la iniciación de la fibrosis de los tejidos, dado su capacidad de inducir altos niveles de una citoquina profibrótica, el TGFß 1 (transforming growth factor ß 1 ) 21,22 .…”

Section: Etiologíaunclassified

“…Algunos autores relacionan la falta de uso de inhibidores de enzima de conversión de la angiotensina (IECA) con la aparición de la enfermedad 22 . Estas observaciones se basan en el hecho de que los IECA han demostrado tener un efecto protector en contra de la fibrosis en distintos modelos y a que muchos de los pacientes que presentaban DFN no utilizaban IECA, a pesar de que la mayoría de los pacientes en insuficiencia renal lo hacía.…”

Section: Etiologíaunclassified

Dermopatía fibrosante nefrogénica: Reporte de dos casos

et al. 2007

Rev. méd. Chile

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Nephrogenic fibrosing dermopathy is a recent designation to describe cutaneous findings in patients with (Rev Méd Chile 2007; 135: 640-6es la designación recientemente incorporada, que se utiliza para describir los hallazgos cutáneos en pacientes con insuficiencia renal que presentan lesiones similares a escleromixedema con engrosamiento e induración de la piel no asociadas a paraproteinemia.Reportada por primera vez en 1997 por Cowper et al, quienes en un comienzo la denominaron «Enfer-medad escleromixedematosa-símil de la hemodiáli-sis» 1 . Describieron 14 pacientes con insuficiencia renal de diferentes etiologías, con engrosamiento e induración cutánea de las extremidades, asociado a hiperpigmentación y, en algunos casos pápulas y nódulos subcutáneos sin compromiso facial. Todos estos pacientes habían recibido o estaban recibiendo hemodiálisis y 9 de los 14 pacientes llegaron al trasplante renal. En esta primera serie de pacientes, los rangos de edad variaban desde los 31 a los 74 años, siendo más frecuente en el sexo masculino y presentándose en todas las razas.Los pacientes desarrollan la enfermedad en forma progresiva, en pocos días a semanas. Las lesiones típicas se describen como edema, eritema, seguidas de placas eritematosas de color café, pápulas y nódulos en las extremidades. En forma excepcional se describen bulas y contractura de las articulaciones. Una de las características clíni-cas relevantes son prurito y dolor urente, asociado a veces a un aumento de la temperatura local 2 .Actualmente se han descrito más de 170 casos de esta patología en Norteamérica, Europa y

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Nephrogenic Fibrosing Dermopathy: Are ACE Inhibitors the Missing Link?

Cited by 37 publications

References 3 publications

Nephrogenic Systemic Fibrosis: More Questions and Some Answers

Nephrogenic Systemic Fibrosis: More Questions and Some Answers

Safety update on the possible causal relationship between gadolinium‐containing MRI agents and nephrogenic systemic fibrosis

Dermopatía fibrosante nefrogénica: Reporte de dos casos

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