Nephrogenic Systemic Fibrosis / Nephrogenic Fibrosing Dermopathy
Nephrogenic systemic fibrosis (NSF) is a systemic disorder affecting the skin, as well as the body's connective tissues. NSF is also known as nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy (NSF/NFD).
NSF has been linked to gadolinium-based contrast agents (GBCAs) used in magnetic resonance imaging (MRI) procedures, including a form of MRI called magnetic resonance angiography (MRA) an imaging scan used by doctors to distinguish blood vessels from surrounding tissues. Thus far NSF has only been reported in patients with kidney disorders.
Attorneys representing patients affected by nephrogenic systemic fibrosis claim that the manufacturers of gadolinium contrast dyes should have done more to warn doctors and patients of potential risks associated with their products. If you or someone you know has been harmed as a result of side effects for gadolinium, contact a personal injury attorney as soon as possible.
NSF Symptoms, Treatment and Prognosis
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. NSF is a form of fibrosis, or scarring, that in extreme cases can affect an organ's ability to function properly. This can have severe consequences specifically, NSF's appearance on the diaphragm and lungs can make it difficult for patients to breathe, which has contributed to the deaths of several patients. In addition, the hardening of skin associated with NSF has led to restricted mobility in some patients, which in turn has caused some patients to fall and harm themselves.
NSF is closely associated with a number of conditions and events, including:
- Coagulation issues
- Recent surgery (particularly vascular surgery, e.g., AV fistula, angioplasty)
- Failed kidney transplant
- Sudden onset of kidney disease characterized by swelling of the extremities
Symptoms. Patients suffering from nephrogenic systemic fibrosis can experience a number of symptoms, including:
- Rapid, unforeseen hypertension (high blood pressure) prior to the appearance of skin lesions
- Muscle weakness
- Elevated yellow spots on the whites of the eyes
- Hip and/or rib pain
- Swelling, hardening and tightening of the skin
Skin-related symptoms usually occur in the extremities but sometimes occur in the trunk as well. Skin lesions may appear in the form of papules, plaques or patches. Over time, these lesions may take on a wood-like appearance, with the texture of an orange peel.
Treatment. While there are currently no effective treatments for nephrogenic systemic fibrosis, improvement of kidney function has been known to slow or gradually reverse NSF. Medications and treatments currently being studied include the following:
- Oral steroids
- Extracorporeal photopheresis (ECP)
- Ultraviolet therapy
- Physical therapy
- Pentoxifylline (PXF)
- Renal transplantation
- High-dose intravenous Ig therapy
Those interested in learning more about possible treatment options for NSF should speak with their doctors.
Prognosis. Because nephrogenic systemic fibrosis is a relatively recent phenomenon, there is still much to learn about this disease, including factors pertaining to prognosis. Some patients with kidney problems later experience improvement in kidney function for reasons unrelated to NSF. A limited number of these patients have later reported softening of skin and therefore improvement in mobility.
In other cases, however, NSF can be a contributing factor in a patient's negligent cause of death. Fibrosis surrounding major organs can adversely affect their ability to function. The presence of fibrosis around the lungs in patients with NSF has led to breathing problems, which in turn contributed to the deaths of some of these patients.
Nephrogenic systemic fibrosis is caused by exposure to gadolinium contrast dyes used in magnetic resonance angiographies. Lawsuits filed against the manufacturers of these potentially defective drugs claim that the manufacturers failed to adequately warn doctors and the general public of the risks associated with their products.
Since 1988, five gadolinium contrast agents have been approved by the FDA:
- Omniscan (gadodiamide), manufactured by GE Healthcare
- ProHance (gadoteridol), manufactured by Bracco
- MultiHance (gadobenate dimeglumine), manufactured by Bracco
- Magnevist (gadopentetate dimeglumine), manufactured by Bayer Healthcare
- OptiMARK (gadoversetamide), manufactured by Mallinckrodt
If you or someone you know was exposed to one of these agents prior to developing NSF, you should contact a gadolinium attorney. Laws vary depending on where you live, so be sure to speak with a lawyer who is familiar with the laws in your state.
At least two helpful NSF/NSD support groups have been established online: NFD_Support.com and Yahoo's NFD Support Group.