Nephrogenic systemic fibrosis (NSF), also called nephrogenis fibrosing dermopathy, is a rare condition of fibrosis of the skin, joints, eyes and internal organs. It usually affects people that have advanced acute kidney failure or people receiving dialysis. There is some evidence that suggests that the nephrogenic systemic fibrosis is related to exposure to gadolinium (or gadolinium-based MRI contrast agents) frequently used in patients with severe kidney failure. The disease manifests with widely spread patches of thick and hardened skin. The disease can also affect the internal organs, such as heart, kidneys and lungs causing shortening of muscle and tendons in the joints or joint contracture.
When affected by nephrogenic systemic fibrosis people develop large parts of hardened skin with fibrotic nodules and plaques. The disease can also lead to joint contracture that causes joint pain and reduces the mobility of the joint. The most severe form of NSF is severe systemic fibrosis affecting the internal organs.
It is not known what exactly causes nehrogenic systemic fibrosis to develop. However, in almost every case there is some type of underlying kidney dysfunction present. Almost 90% of the patients that experienced NSF had an end-stage kidney disease and were on hemodialysis or peritoneal dialysis. In the rest of the cases, people had chronic kidney disease or acute kidney injury. It seems that an underlying kidney disease is necessary for NSF to appear. However, not every patient with some kind of kidney disease develops nephrogenic systemic fibrosis, so it can be assumed that there must be a trigger for the disease to develop. The exact trigger is not familiar, but the contrast agent gadolinium in the magnetic resonance imaging is considered the main suspect.
Factors that can increase the risk of developing nephrotic systemic fibrosis can also be associated to an underlying kidney disease or MRI scans. Some risk factors include the following:
- Gadolinium exposure
- Proinflammatory state
- Infection (pneumonia, osteomyelitis, sepsis)
- Ischemic event
- Limb injury
- Major tissue injury
- Recent surgery
- Renal impairment
- Acute kidney failure
- Chronic kidney failure
- Peritoneal dialysis
There is no specific treatment for nephrotic systemic fibrosis. However, the best option seems to be a therapy that has been designed to improve renal function. It slows and reverses the effects of the NSF.
Physical therapy is also recommended in order to improve mobility of the joints in case of joint contracture.
There are several approaches to treat nephrogenic systemic fibrosis that show different success results. Some of these approaches are:
· Renal transplantation
· Extracorporeal photophoresis
· Sodium thiosulfate therapy
· Imatinib mesylate therapy
· Pentoxifylline therapy
· Intravenous immunoglobulin therapy
· Steroid therapy
· Cyclophosphamide therapy