Nfd is marked by areas of tight, rigid skin and may progress to nephrogenic systemic. Gadoliniumbased contrast exposure, nephrogenic systemic. Nephrogenic systemic fibrosis is characterized by skin induration preferentially affecting the extremities. A case of nephrogenic fibrosing dermopathy nephrogenic. It is typically characterised by hard, erythematous and indurated skin plaques with surrounding subcutaneous oedema. Jul 24, 2017 nephrogenic systemic fibrosis nsf is a complication of the gadoliniumbased contrast agent used in imaging studies. The skin findings are similar to those seen in patients with scleroderma. Pdf nephrogenic fibrosing dermopathy is a recently described fibrosing disorder that emerges in different settings of renal insufficiency. Type of mri contrast, tissue gadolinium, and fibrosis american. The age of patients with nfd ranges from 8 to 87 years at the time of onset, with a mean age of 46. The skin nodules or plaques mostly occurs on the legs and sometimes on the hands in addition to skin fibrosis, there may be fibrosis of other major organs of the body such as the heart. Pdf gadolinium deposition in nephrogenic fibrosing.
Nephrogenic fibrosing dermopathy mostly affects adults young, middleaged, and old. Nephrogenic systemic fibrosis what you need to know. Nephrogenic fibrosing dermopathy associated with exposure. Later this entity was renamed nephrogenic systemic fibrosis nsf when involvement of. Feb 03, 2020 what is nephrogenic systemic fibrosis. Nephrogenic fibrosing dermopathy nfd is an acquired, idiopathic, chronic, progressive eruption of the skin sometimes accompanied by systemic fibrosis that occurs in the context of renal failure. Symptoms of the condition may include progressive swelling and tightening of the skin, sometimes resulting in contractures, and pruritis itching. Recently described systemic fibrosing disorder primarily affecting patients with chronic renal insufficiency exposed to gadolinium based contrast agents clin j am soc nephrol 2010. Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. It is characterized by firm, erythematous, and indurated plaques of the skin associated with. May 22, 2018 nephrogenic systemic fibrosis nsf, also known as nephrogenic fibrosing dermopathy nfd, is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. However, subsequent studies showed that some patients had fibrosis of deeper structures, including muscle, fascia, lungs, and heart. Nephrogenic systemic fibrosis nsf, also known as nephrogenic fibrosing dermopathy nfd, is a disease of fibrosis of the skin and internal.
The age of patients with nfd ranges from 8 to 87 years at. The underlying pathogenetic mechanism of fibrosis remains to be elucidated. Nephrogenic systemic fibrosisnephrogenic fibrosing. Renal transplantation is associated with improved clinical. A 70yearold man was admitted to the hospital for evaluation of thickening of the skin. Nephrogenic fibrosing dermopathy mainly affects patients with renal insufficiency kidney disease. Cutaneous changes of nephrogenic systemic fibrosis predict early mortality and. It was first described in patients with kidney disease in 1997. Erythema nodosum masking nephrogenic systemic fibrosis as. Gadoliniumbased mr contrast agents and nephrogenic systemic fibrosis1.
The condition was first described as scleromyxedemalike cutaneous disease, and it had been observed exclusively in hemodialysis patients. Pdf nephrogenic systemic fibrosis nsf is a relative new disease affecting. It has been attributed largely to the usage of gadoliniumbased contrast agents. Nephrogenic fibrosing dermopathy nephrogenic systemic fibrosis setting the record straight. A case of nephrogenic fibrosing dermopathynephrogenic. Lesions are typically symmetrical and usually develop on the limbs and trunk. Exposure to gadoliniumbased magnetic resonance mr contrast media has been associated with subsequent development of nsf. No better example of this exists than the progressive fibrosis that accompanies all chronic renal disease. Nephrogenic systemic fibrosis nsf is an uncommon fibrotic disorder occurring after administration of linear gadolinium contrast agents in patients with severely decreased kidney function.
It seems to affect only those with some degree of renal dysfunction. Nephrogenic fibrosing dermopathy associated with exposure to gadoliniumcontaining contrast agents st. Nephrogenic fibrosing dermopathy how is nephrogenic. In the literature only about 100 cases of this disease have been described. Nephrogenic fibrosing dermopathy is a rare condition that results in tightened, swollen skin. Food and drug administration 2 and the danish medicines agency 3 and an article by grobner 4 have suggested an association between nephrogenic fibrosing dermopathy and gadolinium use in patients with stage 5 or 6 chronic kidney. Nephrogenic systemic fibrosis american journal of pathology. Rheumatologists, along with other specialists, may be the first to encounter these. Nephrogenic fibrosing dermopathy ukidneys nephrology community. However, higher cost of the research and development, stringent government regulations, and risk of serious illness such as nephrogenic systemic fibrosis nsf or nephrogenic fibrosing dermopathy, and gadolinium deposition disease gdd are.
We describe a patient with nfd for whom histopathologic studies indicated that the fibrotic process. Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, was first documented in 1997 as a sclerodermalike fibrosing entity of the skin in association with renal insufficiency. Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to the gadoliniumbased contrast agents. The first case of a new fibrosing dermopathy distinct from scleroderma was reported in 1997. The condition can resemble scleroderma in appearance. Nephrogenic systemic fibrosis in a patient with multiple. We present a case study of a patient who showed manifestations of nfd in the abdomen after a second. Nephrogenic fibrosing dermopathy associated with exposure to. Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, nsfnfd is a rare disorder which is progressive and often results in permanent problems or possibly even death. By routine microscopy, the findings range from a very subtle proliferation of dermal fibroblasts in. The limited form affects areas below, but not above. Nephrogenic systemic fibrosis nephrogenic fibrosing. Nephrogenic systemic fibrosis nsf, also known as nephrogenic fibrosing dermopathy nfd, is a sclerosing disorder found exclusively in patients with impaired renal function. Systemic findings seen in scleromyxedema, which the condition resembles in some respects, were absent.
Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis nsf was first recognized in 1997, is a rare, diffuse, multisystem, fibrosing disease with an incidence of approximately 35%. It is a progressive debilitating disorder with limited therapeutic options. Renal fibrosis is a direct consequence of the kidneys limited capacity to regenerate after injury. Mar 12, 2007 nephrogenic fibrosing dermopathy is a recently described fibrosing disorder that emerges in different settings of renal insufficiency. Nephrogenic systemic fibrosis nsf is a newly recognized disorder occurring exclusively in patients with renal failure. Rheumatologists, along with other specialists, may be the first to encounter these patients.
Nephrogenic fibrosing dermopathy in pediatric patients. Epidemiological studies suggest that the incidence of nsf is unrelated to gender or ethnicity and it is not thought to have a genetic basis. The purpose of this article is to discuss nephrogenic systemic fibrosis nsf in detail regarding its history, possible pathophysiology, clinical and pathologic presentations, diagnosis, and implications for the radiology community. The condition typically consists of swelling and tightening of the skin, which becomes hard and thick. Nfd occurs most frequently in patients with renal insufficiency or after kidney transplantation. Nephrogenic systemic fibrosis nsf, also known as nephrogenic fibrosing dermopathy nfd, is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. Treatment options are limited, but some patients have had an improvement of their clinical symptoms after renal transplantation and the use of immunosuppression. Soft tissue calcification is rare in patients with nfd and systemic involvement has not been reported. In some cases nsf has contributed to the death of the afflicted individual. Nephrogenic fibrosing dermopathy in a patient with acute. Pediatric nephrogenic systemic fibrosis is rarely reported.
Later, it came to be known as nephrogenic fibrosing dermopathy nfd. Nephrogenic fibrosing dermopathy in children springerlink. Nephrogenic systemic fibrosis is a fibrosing disorder associated with exposure to gadoliniumbased contrast agents in people with severely compromised renal function. Nephrogenic fibrosing dermopathy with systemic involvement. In this article, the authors describe a case of a patient who developed nsf in the setting of gadolinium exposure and multiple inflammatory dermatologic conditions. Apr 24, 20 nephrogenic systemic fibrosis is a condition that affects different parts of the body, particularly the skin.
There are two major subgroups of systemic sclerosis based on the extent of skin involvement. Louis, missouri, 20022006 nephrogenic fibrosing dermopathy nfd causes thickening and hardening of the skin, often in the extremities, and occurs in patients with underlying renal disease. Pdf minimizing risk of nephrogenic systemic fibrosis in. Plasmapheresis for nephrogenic fibrosing dermopathy nfd. Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, was first documented in 1997 as a sclerodermalike fibrosing entity of.
Nsf was originally named nephrogenic fibrosing dermopathy nfd because of the characteristic skin findings. Nephrogenic fibrosing dermopathynephrogenic systemic. Nephrogenic systemic fibrosis is a debilitating disorder seen in chronic kidney disease patients and is characterized by stiffening of the joints and thickening of the skin. Gadolinium deposition and the development of nephrogenic systemic fibrosis pose risks to patients undergoing imaging enhanced with gadolinium. Mar 02, 2010 plasmapheresis for nephrogenic fibrosing dermopathy nfdnephrogenic systemic fibrosis nsf nfd the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to. Nephrogenic fibrosing dermopathy nfd is a rare disease which has only recently been described histopathologically. Who is affected by nephrogenic fibrosing dermopathy. Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of skin, joints, eyes, and internal organs.
If fibrosis is a primary change or is present with little or no inflammation, then it. Its etiopathology is unknown, and no standard therapy currently exists. It is a condition normally found in people with kidney disease. Aug 21, 2012 nephrogenic fibrosing dermopathy nfd is an acquired, idiopathic, chronic, progressive eruption of the skin sometimes accompanied by systemic fibrosis that occurs in the context of renal failure.
Nephrogenic systemic fibrosis radiology reference article. Balance of profibrotic and antifibroting signaling in. Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, is associated with the formation of excessive scar tissue in the skin and connective tissue of other internal organs. Nephrogenic fibrosing dermopathy is a relatively new entity, first described in 1997. Nephrogenic fibrosing dermopathy, also known as nephrogenic systemic fibrosis, is a recently described acquired, idiopathic disorder that manifests in patients with renal disease. Nephrogenic fibrosing dermopathy is a newly described fibrosing disorder in patients with renal failure. Fibrosis appears as a slightly fibrillar, eosinophilic material in the interstitium figure 1 and figure 2. It is characterized by firm, erythematous, and indurated plaques of the skin associated with subcutaneous edema 1. Renal scarring results in a progressive loss of renal function, ultimately leading to endstage renal. Nephrogenic fibrosing dermopathy nfd is a rare condition in which there is a sudden onset of nodular or plaquelike skin lesions. Nephrogenic systemic fibrosis nsf is a disorder seen only in patients with advanced kidney disease and is characterized by two primary features. Request pdf nephrogenic fibrosing dermopathy this report details the histopathologic findings in a unique fibrosing disorder that recently emerged among patients with renal disease. Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis is a devastating disease that causes fibrosis of the skin and connective tissues. Fibrosis may result in progressive atrophy of the nephron in affected areas andor dilation of renal tubules and bowmans space.
It is necessary to combine both the clinical features and the histopathologic. Nephrogenic systemic fibrosis nsf, also known as nephrogenic fibrosing dermopathy, is a complication of gadoliniumbased contrast agents used in mri. Clinically, these patients present with sclerodermalike plaques often involving the extremities. The degree and cause of the underlying renal impairment seem. Nephrogenic fibrosing dermopathy is a rare condition which generally affects the skin, but may involve internal organs in some cases, and is known as nephrogenic systemic fibrosis. Nephrogenic fibrosing dermopathy predominantly affects the skin, but abnormal fibrosis of the internal organs may also occur, which is then often called nephrogenic systemic fibrosis. Fibrotic disorders are commonplace, take many forms and can be lifethreatening. Nephrogenic systemic fibrosis nephrogenic fibrosing dermopathy is most visible in the form of skin lesions, but its most dangerous manifestations occur in the connective tissues surrounding major organs and body parts, including the lungs, abdomen and diaphragm. Minimizing risk of nephrogenic systemic fibrosis in cardiovascular. There is growing evidence that the fibrosis may not be restricted to the s.
In recent years it has become clear that nsf is also associated with fibrotic damage to internal viscera such as the esophagus, lungs, heart, skeletal muscle, and kidneys prompting a change in name from nephrogenic fibrosing dermopathy to nephrogenic systemic fibrosis. Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a novel fibrosing disorder characterized by prominent cutaneous and systemic fibrosis. Diagnosis nephrogenic fibrosing dermopathy nephrogenic systemic fibrosis. Fibrocytes in nsf are immunohistochemically positive for cd34. There is growing recognition of the association between the use of gadoliniumcontaining radiocontrast agents for magnetic resonance imaging and the serious dermal and systemic disease nephrogenic fibrosing dermopathy nephrogenic systemic fibrosis nfdnsf. Eventually, flexure contractures develop, with restriction to movement, pain and pruritus. The disease is characterized clinically by an acute onset of hardening and thickening of the skin of the extremities and trunk, often resulting in flexion contractures, and. Risk factors for developing gadoliniuminduced nephrogenic. Nephrogenic systemic fibrosisnephrogenic fibrosing dermopathy. Nsf is caused by exposure to gadolinium in gadoliniumbased mri contrast agents gbcas in patients with impaired kidney function. Pdf nephrogenic systemic fibrosis is a rare condition appearing only in patients with severe renal impairment or failure and presents with. Gadoliniumbased contrast agent use, their safety, and. Management methylprednisolone, thalidomide and physical therapy. Background nephrogenic fibrosing dermopathynephrogenic systemic fibrosis nfdnsf is a fibrosing cutaneous disorder recently recognized to have systemic manifestations.
Nephrogenic fibrosing dermopathy is a newly recognized cutaneous fibrosing disorder marked by the acute onset of induration involving the upper and lower limbs in patients with acute or chronic. Nephrogenic systemic fibrosis is an uncommon fibrosing systemic disorder with predominant cutaneous manifestation which occurs exclusively in patients with renal insufficiency. Gadoliniumassociated nephrogenic systemic fibrosis. It has characteristic diagnostic findings by skin biopsy and due to its systemic involvement in some reported cases, the term nephrogenic systemic fibrosis has been recently suggested as more appropriate for the nomenclature of this entity. Nephrogenic systemic fibrosis is a rare condition appearing only in patients with severe renal. Patients present with woody, indurated plaques usually involving their trunk and extremities. Gadolinium deposition and nephrogenic systemic fibrosis. Nephrogenic fibrosing dermopathy nfd is a fibrosing condition of the skin that is being described increasingly in patients with renal diseases, many of whom are on dialysis therapy or have undergone renal transplantation.
Objective the purpose of this study was to determine the reported number of cases of nephrogenic systemic fibrosis in children using three distinct publicly available data sources. Drugscardiovascularandrenaldrugsadvisorycommittee ucm196218. Nfd nephrogenic fibrosing dermopathy acronymfinder. Nephrogenic fibrosing dermopathy nfd is an entity of unknown etiology that has only been reported in patients with impaired kidney function. Nephrogenic fibrosing dermopathy is a recently described fibrosing disorder that emerges in different settings of renal insufficiency. Nephrogenic fibrosing dermopathy nfd is a recent designation to describe cutaneous findings in patients with endstage renal disease who developed sclerotic plaques with scleromyxedemalike histologic features. This condition was named nephrogenic systemic fibrosis nsf and presents with cutaneous thickening or indurated and discolored skin affecting the limbs and trunk but sparing the face 11, 12. Nephrogenic systemic fibrosis is a devastating and rapidly progressive condition, making its prevention in atrisk populations of utmost importance.
Nephrogenic systemic fibrosis is a condition that affects different parts of the body, particularly the skin. This condition was originally termed nephrogenic fibrosing dermopathy as initially only. Distinct papules and subcutaneous nodules can also be seen. No etiologic factor besides kidney disease has been linked to this disorder, although cd34 is a marker of hematopoietic progenitor cells. Nephrogenic systemic fibrosis nsf is the thickening and hardening of your skin, mostly on your arms and legs. Symptoms nephrogenic systemic fibrosis diagnosis nsf. Nephrogenic systemic fibrosis genetic and rare diseases. The degree and cause of the underlying renal impairment seem not to be related to the severity of the nephrogenic fibrosing dermopathy. Nephrogenic systemic fibrosis nsf is a relatively new fibrosing disorder. Nsf is caused by exposure to gadolinium in gadoliniumbased mri contrast agents in patients with impaired kidney function. Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of skin, joints, eyes, and.