by Brian H. Tew, M.D., J.D.
What is Nephrogenic Systemic Fibrosis?
NSF has changed the skin texture and thickness on this leg.
Nephrogenic systemic fibrosis, NSF, is a painful, often progressive disease involving the skin, underlying fascia, muscle and in some cases internal organs as well. NSF is caused by increased deposition of collagen, or fibrous tissue, in the dermal layer of the skin as well as in other organs.
NSF usually begins with thickening and hardening of the skin on the hands and feet that progresses up toward the trunk or body. The skin of victims of NSF has a remarkable appearance and is often described as having a wooden feel, swollen or indurated and appearing brawny or dark colored. Sometimes the skin can have an orange peel appearance.
The skin can also have areas of plaque, or thickening, that may appear to have irregular geographic appearing borders. [1] The joints of the fingers, elbows, ankles and knees often are contracted, or drawn up, and frequently there is significant tethering of the skin making it difficult to extend the elbows, knees and fingers. In addition, it may be difficult to place the palms flatly together because of the inability to straighten the fingers. We now know that NSF can affect the internal organs with almost every major organ having been found to be affected by the fibrosing processes of NSF. [2]
NSF can become so severely disabling that some patients are completely confined to bed only able to move around in a wheel chair. The skin of NSF victims can become so tight that it is described as unpinchable and the tightening can lead to compromise of the blood supply leading to amputation. Patients with NSF also manifest a phenomenon that Dr. Girardi at Yale calls banding where the fibrosis involves the deeper areas of the skin causing linear bands to pull down on the arm or the thigh. Contractures of the fingers can cause significant deformity and disability and may be a poor prognostic sign. These same findings can be found in the ankles and feet with severe limitation in the range of motion as the skin becomes increasingly fibrotic. Eventually the extremities may become severely contracted with complete loss of range of motion. [3]
In the vast majority of cases, NSF occurs in patients with severe kidney disease or with profound kidney failure at the time that the gadolinium based contrast agent was administered. Most of the patients with NSF had stage 4 or 5 kidney disease at the time the MRI dye was administered to them.
The first cases of what is now known to be NSF cases appeared between 1997 and 2000 in patients who were undergoing hemodialysis for failed renal transplants. These patients developed severe skin thickening, or induration, that was thought to be Scleromyedema, or scleroderma. [4], [5], [6]
While most of the reported cases of NSF have involved patients on hemodialysis, there are also cases of NSF in patients on peritoneal dialysis, renal transplant recipients, liver transplant recipients, patients with advanced chronic kidney disease and patients with acute renal failure not requiring dialysis. [7]
