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.

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.