Fibrosis sistémica nefrogénica: la pandemia que no fue / Causal relationship between the use of gadolinium based contrast media and nephrogenic systemic fibrosis

Nephrogenic systemic fibrosis (NSF) is a severe iatrogenic disease that affect patients with impaired renal function exposed to gadolinium-based contrast agents. Clinically, symptoms develop within days or weeks after the exposure and mimic a scleromyxedema. The causal relationship between use of gadolinium-based contrast agents and NSF led to develop clinical guidelines aiming to limit the use of this contrast medium in high risk patients. These guidelines decreased the incidence of NSF in the last years. Unfortunately there is no specific treatment for NSF yet. Thus, strict adherence to current guidelines is key to prevent new cases. Renal dysfunction is increasingly common in our population. Therefore, practicing physicians should be aware of this potential complication of the use of gadolinium based contrast media.

Nephrogenic systemic fibrosis: concepts and perspectives / Fibrose nefrogênica sistêmica: conceitos e perspectivas

Nephrogenic systemic fibrosis is a chronic, progressive condition that develops in some patients with renal impairment after exposure to gadolinium-based contrast agents used in magnetic resonance imaging. Thickening of the skin is typical, usually affecting the extremities. Visceral organs can also be affected. The diagnosis of the disease requires careful clinicopathological correlation. Treatment aims at restoring renal function, which is associated with delayed progression and, eventually, remission of skin changes. Reduction and prevention of nephrogenic systemic fibrosis cases are based on limiting the use of gadolinium-based contrast agents in patients with kidney disorders (especially in patients with advanced renal failure at stages 4 and 5), and restricting their use to situations in which they are essential to diagnosis/follow-up. Other than limiting exposure to gadolinium based contrast agents, no effective preventive methods have been reported. Due to increased awareness about the disease among radiologists and nephrologists, the incidence of nephrogenic systemic fibrosis is declining.

Fibrose nefrogênica sistêmica é condição crônica, progressiva, desenvolvida caracteristicamente em pacientes nefropatas após exposição a contrastes radiológicos que contenham gadolínio. O espessamento cutâneo é aspecto típico, envolvendo predominantemente as extremidades. Envolvimento visceral pode ocorrer. O diagnóstico da doença requer cuidadosa correlação clínico-patológica. O tratamento visa à restauração da função renal, que se associa ao retardo da progressão e, eventualmente, remissão das alterações cutâneas. A prevenção da ocorrência e redução da incidência baseiam-se na limitação do uso de contrastes à base de gadolínio em nefropatas (especialmente na insuficiência renal avançada em estágios 4 e 5), restringindo-os às condições nas quais seja imprescindível ao diagnóstico/acompanhamento. À exceção da restrição de exposição aos agentes de contraste a base de gadolínio, não há métodos preventivos efetivos relatados. Devido à ampla divulgação da doença entre radiologistas e nefrologistas, a incidência da fibrose nefrogênica sistêmica está em declínio.

Gadolinio y fibrosis sistémica nefrogénica / Gadolinium and nephrogenic systemic fibrosis

Inicialmente llamada dermopatía fibrosante nefrogénica, la fibrosis sistémica nefrogénica (FSN) está fuertemente ligada a la inyección endovenosa de medios de contraste -basados en gadolinio- en pacientes con insuficiencia renal. En esta revisión corta se analiza la fisiopatología y la clínica, la responsabilidad del radiólogo en la prevención a través de la puesta en práctica de las reglamentaciones vigentes y, por último, se mencionan algunos conceptos sobre el tratamiento de los pacientes con insuficiencia renal crónica que deben exponerse a la inyección de gadolinio...

Cinecoronariografia com gadolínio em pacientes com alergia grave ao contraste iodado / Coronary angiography with gadolinium in patients with severe allergy to iodinated contrast media

Em alguns pacientes a utilização dos meios de contraste iodados é contraindicada pela possibilidade de desencadearem efeitos colaterais graves ameaçadores da vida. Nesses casos, existem relatos em que o gadolínio (gadobutrol), comumente empregado na ressonância nuclear magnética, pode ser usado como meio de contraste para coronariografia. Relatamos dois casos com antecedente de alergia grave ao contraste iodado, que realizaram coronariografia com gadolínio. Os procedimentos foram bem tolerados, a quantidade de contraste não excedeu 0,3-0,4 ml/kg e as imagens obtidas foram de qualidade aceitável para efeitos de diagnóstico.

The use of iodinated contrast media is contraindicated in some patients due to serious life-threatening adverse events. In such cases, there are reports that gadolinium (gadobutrol), which is commonly employed in magnetic resonance imaging, may be used as contrast media for coronary angiography. We report two cases with a history of severe allergy to iodinated contrast agent who underwent coronary angiography with gadolinium. The procedures were well tolerated, the amount of contrast media did not exceed 0.3-0.4 ml/kg and the images obtained were of acceptable quality for diagnostic purposes.

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.

Nephrogenic systemic fibrosis: A review of the new conundrum.

Nephrogenic systemic fibrosis is an idiopathic, sclerosing condition that occurs only in patients who have impaired renal function. Although its most conspicuous manifestation is in the skin, the condition involves multiple organ systems and is potentially fatal. Its postulated association with gadolinium-based magnetic resonance contrast agents has attracted attention. The distinctive clinical features of this entity include a prodromal systemic inflammatory syndrome followed by a chronic course of fibrosis that has a predilection for the skin and subcutaneous tissues of the extremities. The progressive systemic fibrosing process involves multiple organs and contributes to the morbidity and the increased mortality. Appropriate preventive action, prompt recognition and timely reporting of cases may enable better management of this condition.

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.

No association between gadolinium-based contrast agents and development of nephrogenic systemic fibrosis: a case study

Nephrogenic systemic fibrosis [NSF], previously known as nephrogenic fibrosing dermopathy, is an emerging systemic fibrosing disorder that develops in the setting of renal insufficiency. Nephrogenic fibrosing dermopathy [NFD] is a fibrosing condition of the skin which shows an increased number of dendritic cells, fibroblasts and thickened collagen fibers resembling scleromyxedema. It is characterized by indurated plaques mainly on the extremities and the absence of paraproteinemia. Although the exact causes of NSF have not been established, evidence suggests an association between gadoliniumbased contrast agents and development of nephrogenic systemic fibrosis. We report a patient who was under dialysis and developed NSF but she never did MRI

Complicações do uso intravenoso de agentes de contraste à base de gadolínio para ressonância magnética / Complications from the use of intravenous gadolinium-based contrast agents for magnetic resonance imaging: [review]

Os agentes de contraste à base de gadolínio são muito mais seguros que o contraste iodado, no entanto, existem complicações que devem ser reconhecidas, para orientação e tratamento adequados. A incidência total de reações adversas aos meios de contraste em ressonância magnética varia entre 2 por cento e 4 por cento. Casos de reações adversas agudas maiores ao gadolínio, como laringoespasmo e choque anafilático, são raros. As complicações crônicas com o uso do gadolínio também existem e, recentemente, foi descrita associação entre seu uso e uma doença dermatológica rara que ocorre em pacientes com insuficiência renal. A fibrose nefrogênica sistêmica foi tema de anúncio público oficial pela agência americana de regulação de drogas, a Food and Drug Administration. Esta doença progressiva caracteriza-se pelo espessamento e endurecimento da pele e fibrose, que podem acometer outras partes do corpo. Os pacientes que desenvolveram esta complicação apresentavam insuficiência renal crônica, estavam em acidose metabólica e foram submetidos a angiografia por ressonância magnética, provavelmente com injeção de grande volume de contraste paramagnético. Esta revisão tem o objetivo de apresentar uma descrição sucinta dos tipos de meios de contraste à base de gadolínio, possíveis complicações e medidas para prevenção e tratamento destas.

Gadolinium-based contrast agents are much safer than the iodinated ones; however complications may occur and should be recognized for appropriate orientation and management. The total incidence of adverse reactions to contrast agents in magnetic resonance imaging ranges between 2 percent and 4 percent. Cases of severe acute reactions to gadolinium, such as laryngospasm and anaphylactic shock, are rare. Chronic complications secondary to the use of gadolinium also can occur and, recently an association between its use and a rare dermatologic disease occurring in patients with renal failure has been reported. Nephrogenic systemic fibrosis was the subject of an official health notification issued by the American Food and Drug Administration. This progressive disease is characterized by hardened skin with fibrotic nodules and plaques which may involve other parts of the body. Patients who have been affected by this disorder presented chronic renal failure, with metabolic acidosis and had been submitted to magnetic resonance angiography, probably involving exposure to large amounts of intravenous paramagnetic contrast. This review is aimed at presenting a succinct description of the gadolinium-based contrast agent types, possible secondary complications, their preventive measures and management.

Fibrosis sistémica nefrogénica vinculada a gadolinio con evolución fatal / Nephrogenic systemic fibrosis related to gadolinium with fatal outcome

La fibrosis sistémica nefrogénica (FSN), primero conocida como dermopatía fibrosante nefrogénica, es una rara entidad recientemente descripta que se desarrolla en pacientes insuficientes renales. Nueva evidencias relacionan la FSN con medios de contraste basados en gadolinio (MCBG). Diagnosticamos esta enfermedad en una paciente femenina adulta de 33 años con insuficiencia renal crónica terminal en hemodiálisis. Un mes previo al inicio de los síntomas había recibido MCBG. Al examen físco presentaba placas firmes en ambos miembros superiores e inferiores, con endurecimiento y engrosamiento cutáneo marcado, que posteriormente se extendieron a abdomen inferior y mamas. Asociaba dolor, contracturas articulares en flexión y movilidad restringida. Evolucionó con progresión del cuadro, falleciendo al mes de la primera consulta. La FSN puede presentar una evolución rápidamente progresiva que resulta en discapacidad e incluso la muerte. Esto hace necesaria una revisión sobre su posible vinculación con MCBG y sobre las precauciones sugeridas para su indicación en estos pacientes.

Medio de contraste en resonancia magnética. Lesiones cerebrales intraaxiales / MRI contrast medium in intraaxial cerebral lesions

El desarrollo de la resonancia magnética (RM) ha originado un importante avance en el diagnóstico de las enfermedades del sistema nervioso central (SNC). Las ventajas fundamentales incluyen: la ausencia de radiación, la capacidad de obtener imágenes en múltiples planos y una mayor sensibilidad en la detección de procesos patológicos. No obstante, al igual que lo ocurrido en los inicios de la tomografía computada (TC), se ha hecho evidente la necesidad de utilizar medios de contraste. Los primeros estudios se han realizado en torno a sustancias paramagnéticas, de las cuales el Godolinium-DTPA ha sido el primero en obtener una valoración clínica y consiguiente aprobación.