Imágenes del depósito de gadolinio en el sistema nervioso central / Images of gadolinio deposit in the central nervous system Abstract

Magnetic resonance imaging has been shown to be very useful in the diagnosis and monitoring of multiple diseases affecting the central nervous system. Gadolinium has been used extensively worldwide. It has been estimated that since its introduction more than two hundred million doses of GBCA have been administered globally. Gadolinium-based contrast agents (GBCAs) were long considered medicines with a high safety profile, the literature reports incidences of immediate adverse effects such as headache, dizziness, and altered sense of taste. Studies performed a few years after the introduction of GBCA showed that there were gadolinium deposits in the tissues but their clinical significance was not known. It wasn’t until 2006 when the first reports were published that associated the gadolinium ion tissue deposits from GBCA with a systemic inflammatory entity of the connective tissue similar to scleroderma known as Nephrogenic Systemic Fibrosis (NSF), in patients with chronic renal failure. In 2013, the association between the use of GBCAs and the progressive increase in the signal intensity of the dentate nucleus and the globus pallidus in T1-weighted MRI images without contrast medium, was described for the first time. This review describes the most relevant aspects of the pathophysiology of these findings taking into account their differential diagnosis.

Las imágenes por resonancia magnética han mostrado ser de gran utilidad en el diagnóstico y seguimiento de múltiples enfermedades que afectan el sistema nervioso central. El gadolinio se ha utilizado ampliamente a nivel mundial. Se estima que desde su introducción se han administrado más de doscientos millones de dosis de MCBG en el mundo. Los medios de contraste basados en Gadolinio (MCBG) fueron considerados por mucho tiempo medicamentos con un alto perfil de seguridad, la literatura reporta incidencia de efectos adversos inmediatos como cefalea, mareo y alteración del sentido del gusto. Estudios realizados pocos años después de la introducción de los MCBG mostraron que había depósitos de gadolinio en los tejidos, pero no se conocía su significancia clínica Fue hasta 2006 cuando se publicaron los primeros reportes que asociaban el depósito tisular del ion gadolinio proveniente de los MCBG con una entidad inflamatoria sistémica del tejido conectivo similar a la escleroderma conocida como Fibrosis Sistémica Nefrogénica (FSN) en pacientes con falla renal crónica. En 2013 se describió por primera vez la asociación entre el uso de los MCBG y el aumento progresivo de la intensidad de señal del núcleo dentado y los globos pálidos en las imágenes de RM ponderadas en T1 sin medio de contraste. En esta revisión se describen los aspectos más relevantes de la fisiopatología de estos hallazgos considerando su diagnóstico diferencial.

Prevention of Nephrogenic Systemic Fibrosis / 대한내과학회지

Gadolinium is widely used as a contrast agent for magnetic resonance imaging (MRI). In patients with radiocontrast media-induced nephrotoxicity, MRI has generally been considered to be a safe substitute for computed tomography (CT). However, recent studies have described the potential emergence of gadolinium-related nephrogenic systemic fibrosis (NSF) in patients with advanced kidney disease. NSF is a fibrosing disorder seen only in patients with moderate to severe kidney failure; it is particularly common in patients on dialysis. Skin involvement occurs in all patients and is characterized by plaques, papules, nodules, thickening, and hardening of the skin overlying the extremities and trunk. Histopathologic examination of an incisional or punch biopsy of affected skin reveals marked expansion and fibrosis of the dermis with proliferation of CD34+ fibrocytes and long dendritic processes. There is no proven medical treatment for NSF other than recovery of renal function. Therefore, the currently recommended preventive measure for NSF in patients with advanced kidney failure is avoidance of gadolinium. Gadolinium-containing contrast agents, especially at high doses, should be used only if clearly necessary. Prompt commencement of hemodialysis may be necessary after gadolinium administration.

Fibrosis sistémica nefrogénica: Presentación de un caso y revisión de la literatura / Nephrogenic systemic fibrosis: A case report and review of the literature

La fibrosis sistémica nefrogénica (FSN) es una enfermedad fibrosante de reciente identificación, que afecta exclusivamente a pacientes con insuficiencia renal. Se caracteriza por una fibrosis extensa de los tejidos, especialmente de la piel. Aunque su etiología no es del todo conocida, se ha asociado fuertemente al uso de contrastes de gadolinio en pacientes con insuficiencia renal avanzada. Se describe a continuación un caso de FSN diagnosticado en España.

Nephrogenic systemic fibrosis (NSF) is a rare condition appearing only in patients with severe renal impairment and characterized by widespread tissue fibrosis. Although the pathogenesis of this disorder remains unclear, there is evidence of a strong association between gadolinium-based contrast agents (Ga-CA) exposure and the triggering of this disease. Cellular elements involved in pathogenesis of NSF include bone-marrow-derived collagen producing fibrocytes, myofibroblasts and activated macrophages. We present the case of a caucasian female with advanced renal failure who underwent several times magnetic resonance imaging (MRI) procedures using Ga-CA. The patient developed a progressive fibrosing disorder with bilateral indurated papules on the lower legs, joint contractures and hairlessness, and was diagnosed of NSF based on the histologic findings of skin biopsies. Later in the disease she presented with systemic involvement and fatal evolution.

A Case of Delayed Onset Nephrogenic Systemic Fibrosis After Gadolinium Based Contrast Injection

Nephrogenic systemic fibrosis (NSF) is a rare fibrosing disorder of the skin and joints that occurs in patients with advanced renal insufficiency. This condition is progressive and can be seriously disabling. Gadolinium based contrast agent (GBCA) has been identified as a potential cause of this condition. A 56-years-old man in hemodialysis developed stiffness and contracture of the whole limbs eight years after frequent GBCA exposure for cervical magnetic resonance imaging. For the first time in Korea, we report late-onset nephrogenic systemic fibrosis after GBCA exposure and performed an electrophysiologic study of this condition.

Nephrogenic systemic fibrosis following hair-dye ingestion induced acute renal failure.

We report a patient who developed acute renal failure following the ingestion of a hair-dye with a suicidal intent. He was managed by hemodialysis and other symptomatic measures. He developed generalized seizures and underwent MRI scan of the brain using gadolinium containing contrast material followed by development of bilateral and symmetrical thickening and induration of the skin over the extremities and verrucous papules and plaques over the lower back. Skin biopsy and immunohistochemistry showed typical features of nephrogenic fibrosing dermopathy as well as deposits of calcium. Successful management of renal failure resulted in clearing of all skin lesions except a small bony hard plate like area overt the left leg, the biopsy of which showed features of osseous metaplasia.

Nephrogenic systemic fibrosis following acute kidney injury and exposure to gadolinium.

Nephrogenic systemic fibrosis (NSF) is a scleroderma-like systemic fibrosing condition of unknown etiology described in patients with renal insufficiency. Gadolinium exposure has been strongly associated with the development of NSF though the mechanism of such injury is not known. There are only few reported cases of NSF in the setting of acute renal failure and fewer reported cases where skin lesions developed after kidney function had returned to normal. We report a case of NSF in a young Hispanic woman with lupus nephritis but normal creatinine, who received gadolinium during a brief episode of prerenal acute kidney injury not requiring dialysis, secondary to sepsis.

A case of nephrogenic systemic fibrosis after gadolinium-based contrast agent injection / 대한내과학회지

Nephrogenic systemic fibrosis is a rare multisystemic disorder mainly affecting the skin and joints in patients with underlying renal insufficiency exposed to gadolinium-based contrast. We report a patient who had renal insufficiency caused by multiple myeloma and developed nephrogenic systemic fibrosis after exposure to gadolinium-based contrast for the first time in Korea.

A case of nephrogenic systemic fibrosis following gadolinium exposure in a peritoneal dialysis patient / 대한내과학회지

Nephrogenic systemic fibrosis is a systemic illness that only affects patients with kidney failure. Exposure to gadolinium-based contrast agents has been associated with the subsequent development of nephrogenic systemic fibrosis in patients with decreased renal function. Nephrogenic systemic fibrosis is characterized by skin induration after swelling and limited joint movement through a loss in flexibility that preferentially affects the extremities. A 58-year-old man in peritoneal dialysis developed swelling and stiffness of the lower limbs following gadolinium exposure for brain magnetic resonance imaging. This patient was diagnosed with nephrogenic systemic fibrosis by CD34 immunoreactivity of subcutaneous fibroblasts in a skin biopsy. We report, for the first time in Korea, that nephrogenic systemic fibrosis developed after gadolinium exposure in a peritoneal dialysis patient.

Fibrose sistêmica nefrogênica: uma complicação grave do uso do gadolínio em pacientes com insuficiência renal / Nephrogenic systemic fibrosis: a severe complication of use to gadolinium in patients with kidney failure

Fibrose sistêmica nefrogênica (FSN), também conhecida como dermopatia fibrosante nefrogênica (DFN), é uma condição que ocorre apenas em pacientes com disfunção renal. Além das lesões cutâneas, esta síndrome inclui fibrose de músculo esquelético, articulações, fígado, pulmão e coração e pode ser fatal. Esta doença foi primeiramente descrita em 1997 e vários estudos descrevem a relação etiológica da FSN com a exposição a agentes de contraste contendo gadolínio, usado em exames de ressonância magnética. Esta revisão tem como objetivo alertar médicos clínicos e nefrologistas sobre essa nova patologia que acomete pacientes com alteração da função renal, demonstrando aspectos demográficos e epidemiológicos, apresentação clínica, diagnóstico e prognóstico além das opções de prevenção e terapêuticas atuais. Concluímos que todo paciente apresentando creatinina sérica elevada deve ter sua função renal (clearance de creatinina) estimada, visando a segurança na realização da ressonância magnética.

Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy (NFD), is a condition that has occurred only in patients with renal insufficiency. Besides lesions of the skin, this syndrome include fibrosis of skeletal muscle, joints, liver, lung, and heart, with possible fatal outcomes. This disease was first described in 1997 and several reports described the development of NSF after the exposure to gadolinium-based magnetic resonance imaging contrast agents. This review aims to alert physicians and nephrologists about this new pathology that affects patients with renal dysfunction, describing its demographic and epidemiologics aspects, clinic presentation, diagnosis and prognosis, beyond options to prevent and current treatment. We concluded that in all patient with elevated serum creatinine physicians should estimade his kidney function (creatinine clearence) in order to safety of magnetic resonance.