Nephrogenic systemic fibrosis: a cause of skin thickening
Updated April 08, 2015.
Written or reviewed by a board-certified physician. See About.com's Medical Review Board.
Nephrogenic systemic fibrosis (NSF) which is also known previously as nephrogenic fibrosing dermopathy (NFD) was first observed in the mid 1990s in patients with renal insufficiency and those patients specifically on hemodialysis. Initially, the cause of nephrogenic systemic fibrosis was unclear. However, researchers first noted that there was association between nephrogenic systemic fibrosis and gadolinium-based magnetic resonance imaging (MRI) contrast agents.
Since then, there has been a significant number of patients with end-stage renal disease and hemodialysis who received gadolinium and ultimately developed nephrogenic systemic fibrosis. Another common skin condition that mimic skin fibrosis seen in nephrogenic systemic fibross includes scleroderma.
The symptoms of nephrogenic systemic fibrosis are characterized by rapidly advancing skin thickening, skin hyper pigmentation, tethering of skin, and skin stiffening. Patients suffer from contractures in late stages of the disease with extensive debilitation and limitations in quality of life. The deep fibrotic process causing flexion contractures usually affect the hands, wrists, knees, and ankles of patients resulting in major loss of range of motion and disability in patients. Itching and burning sensations have been reported by patients. Peripheral neuropathy with loss of sensation has also been cited by patients who have been affected. The condition is painful and associated with significantly increased mortality.
The course of the disease manifests initially most commonly in the lower extremities. The distribution of skin involvement is symmetric. The time course can range between days to weeks in the development of symptoms and skin manifestations. The pathophysiology of the disease are characterized by increased number of fibroblasts in the skin and abnormal architecture of collagen bundles. Research has shown that the skin is not the only organ in the body to be affected. Fibrosis occurs in other organs in nephrogenic systemic fibrosis as well. The organs that can be affected include but not limited to the heart, skeletal muscle, genitourinary tract, diaphragm, and lungs.
As a result of this condition, it is important to minimize risk of exposure to intravenous administration of gadolinium based MRI contrast if one were to receive this imaging study. All radiology departments now follow precautionary procedures to limit use of gadolinium in patients with impaired renal function and those patients on hemodialysis. Although very rare, physicians should be aware of this possible complication before considering MRI for patients with kidney disease.
To prove the diagnosis of nephrogenic systemic fibrosis, it is often required to obtain a skin biopsy. The skin sample is then examined by a trained dermatopathologist to evaluate and to exclude other causes of other fibrosing skin conditions.
There is currently no proven treatment for nephrogenic systemic fibrosis. Rarely does the disease remit or improve, but many cases do stabilize in terms of symptomatology. Research is being conducted evaluating the use of oral steroids, chemotherapeutic agents, ultraviolet (UV) therapy, high dose immunoglobulin infusion, renal transplantation, and physical therapy. There are case reports of spontaneous recovery after improvement in renal function and renal transplantation. Prevention is the key to approaching this condition. Coordination between healthcare practitioners such as nephrologists, radiologists, dermatologists, rheumatologists, and dermatopathologists are critical to the management of this condition in patients who are affected.
The prognosis of nephrogenic systemic fibrosis varies widely among patients affected. In milder forms, patients experience low level chronic symptoms. In more severe forms, patients have significant mobility issues and those with fibrosis affecting critical organs and muscular function such as the diaphragm, thereby affecting respiratory capacity, have died from this condition.
Written or reviewed by a board-certified physician. See About.com's Medical Review Board.
Nephrogenic systemic fibrosis (NSF) which is also known previously as nephrogenic fibrosing dermopathy (NFD) was first observed in the mid 1990s in patients with renal insufficiency and those patients specifically on hemodialysis. Initially, the cause of nephrogenic systemic fibrosis was unclear. However, researchers first noted that there was association between nephrogenic systemic fibrosis and gadolinium-based magnetic resonance imaging (MRI) contrast agents.
Since then, there has been a significant number of patients with end-stage renal disease and hemodialysis who received gadolinium and ultimately developed nephrogenic systemic fibrosis. Another common skin condition that mimic skin fibrosis seen in nephrogenic systemic fibross includes scleroderma.
The symptoms of nephrogenic systemic fibrosis are characterized by rapidly advancing skin thickening, skin hyper pigmentation, tethering of skin, and skin stiffening. Patients suffer from contractures in late stages of the disease with extensive debilitation and limitations in quality of life. The deep fibrotic process causing flexion contractures usually affect the hands, wrists, knees, and ankles of patients resulting in major loss of range of motion and disability in patients. Itching and burning sensations have been reported by patients. Peripheral neuropathy with loss of sensation has also been cited by patients who have been affected. The condition is painful and associated with significantly increased mortality.
The course of the disease manifests initially most commonly in the lower extremities. The distribution of skin involvement is symmetric. The time course can range between days to weeks in the development of symptoms and skin manifestations. The pathophysiology of the disease are characterized by increased number of fibroblasts in the skin and abnormal architecture of collagen bundles. Research has shown that the skin is not the only organ in the body to be affected. Fibrosis occurs in other organs in nephrogenic systemic fibrosis as well. The organs that can be affected include but not limited to the heart, skeletal muscle, genitourinary tract, diaphragm, and lungs.
As a result of this condition, it is important to minimize risk of exposure to intravenous administration of gadolinium based MRI contrast if one were to receive this imaging study. All radiology departments now follow precautionary procedures to limit use of gadolinium in patients with impaired renal function and those patients on hemodialysis. Although very rare, physicians should be aware of this possible complication before considering MRI for patients with kidney disease.
To prove the diagnosis of nephrogenic systemic fibrosis, it is often required to obtain a skin biopsy. The skin sample is then examined by a trained dermatopathologist to evaluate and to exclude other causes of other fibrosing skin conditions.
There is currently no proven treatment for nephrogenic systemic fibrosis. Rarely does the disease remit or improve, but many cases do stabilize in terms of symptomatology. Research is being conducted evaluating the use of oral steroids, chemotherapeutic agents, ultraviolet (UV) therapy, high dose immunoglobulin infusion, renal transplantation, and physical therapy. There are case reports of spontaneous recovery after improvement in renal function and renal transplantation. Prevention is the key to approaching this condition. Coordination between healthcare practitioners such as nephrologists, radiologists, dermatologists, rheumatologists, and dermatopathologists are critical to the management of this condition in patients who are affected.
The prognosis of nephrogenic systemic fibrosis varies widely among patients affected. In milder forms, patients experience low level chronic symptoms. In more severe forms, patients have significant mobility issues and those with fibrosis affecting critical organs and muscular function such as the diaphragm, thereby affecting respiratory capacity, have died from this condition.
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