Illness & Symptoms: NSF & Gadolinium
Overview
Patients with NSF develop thickening
of the skin and connective tissues that inhibits
their ability to move and may result in broken
bones. Other organs are at risk of thickening
as well. The cause of NSF is not known and
there is no consistently effective treatment
of this condition.
Patients with NSF describe swelling and
tightening of the skin, usually limited to
the extremities (images),
but sometimes involving the trunk. The condition
may develop over a period of days to several
weeks. In many cases, the skin thickening
inhibits the flexion and extension of joints,
resulting in contractures. Severely affected
patients may be unable to walk, or fully
extend the joints of their arms, hands, legs,
and feet. Complaints of muscle weakness are
common. Approximately 5% of patients have
a rapidly progressive (fulminant) course.
The skin changes may start as reddened or darkened patches, papules, or plaques.
In time, the skin may feel “woody” and the surface may resemble the texture of
the peel of an orange. Patients may experience burning, itching, or severe sharp
pains in areas of involvement. Radiography may reveal calcifications of the soft
tissue. Deep "bone pain" has been described in the hips and in the ribs.
The skin lesions are commonly symmetrical, with zones between the ankles and
thighs most commonly involved, followed by involvement between the wrist and
upper arms. Hand and foot swelling with blister-like lesions has also been reported.
Some patients have reported yellow papules or plaques on or near the eyes. Rapid,
new onset fluctuating hypertension of unknown cause has been described prior
to the onset of the skin lesions.
NSF
Nephrogenic Systemic Fibrosis
(NSF), also known as nephrogenic fibrosing
dermopathy (NFD), is a condition that, so far,
has occurred only in people with kidney disease.
There is no convincing evidence that NSF is
caused by a medication, a microorganism, or
by dialysis. There have been no cases identified
prior to early 1997. At this point it appears
NSF is a systemic disorder with its most
prominent and visible effects in the skin.
For this reason, Nephrogenic Systemic Fibrosis
has been suggested as an equivalent terminology
in those previously diagnosed with NFD,
and is preferred in that it more accurately
reflects our current understanding of the
disorder.
Neither the duration of kidney disease nor its underlying cause are related to
the development of NSF. Some patients with NSF develop skin tightening in the
earliest stages of kidney disease, and others may have had kidney disease for
years. Specific triggers for the development of NSF are still being investigated.
Recent reports have strongly correlated the development of NSF with exposure
to gadolinium-containing MRI contrast agents. Further information from the US
Food and Drug Administration regarding this observation can be found here.
The initial FDA advisory has been recently modified to the statement present
at this site.
GE healthcare, one manufacturer of gadolinium-containing MRI contrast,
has issued an informational announcement here.
This announcement has been updated to include new information as of 12/2006 and
is present at this site.
NSF appears to affect males and females in
approximately equal numbers. NSF has been
confirmed in children and the elderly,
but tends to affect the middle-aged most
commonly. It has been identified in patients
from a variety of ethnic backgrounds and
from North America, Europe, and Asia.
Besides kidney disease, conditions that may
be associated with NSF include coagulation
abnormalities and deep venous thrombosis,
recent surgery (particularly vascular surgery),
recent failure of a transplanted kidney,
and sudden onset kidney disease with severe
swelling of the extremities. It is very common
for the NSF patient to have undergone a vascular
surgical procedure (such as revision of an
AV fistula, or angioplasty of a blood vessel)
or to have experienced a thrombotic episode
(thrombotic loss of a transplant or deep
venous thrombosis) approximately two weeks
before the onset of the skin changes.
Reports
Reports have identified the development
of NSF following single and multiple administrations
of the gadolinium-based contrast agents.
The reports have not always identified a
specific agent. Omniscan was the most commonly
reported agent, when a specific agent was
identified, followed by Magnevist and OptiMARK.
NSF also has developed after the sequential administration of Omniscan and MultiHance and Omniscan and ProHance. Because reports incompletely describe exposure to gadolinium-based contrast agents, it is not possible to know if the extent of risks for developing NSF is the same for all agents.
Patients should be screened for kidney problems prior to receiving one of these imaging agents. The recommended dose should not be exceeded and enough time should elapse to ensure that a dose has been eliminated from the body before the agent is used again.
There have been no reports of NSF among patients with normal kidney function or those with mild-to-moderate kidney insufficiency.
For More Information on NSF
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