Nephrogenic Systemic Fibrosis (NSF)
No post or summary on skin disorders in renal failure can
omit NSF. This has been mentioned in multiple previous posts on RFN – although never summarized:
omit NSF. This has been mentioned in multiple previous posts on RFN – although never summarized:
The first
cases of this disorder were noted between 1997 and 2000. Nearly all have been
in patients with gadolinium exposure. Most of these patients had severe CKD or
were on dialysis. The onset of NSF post gadolinium exposure ranges from 2
months to 15 years. The risk of NSF in dialysis patients has been reported as
between 2.5 and 5%. Most centers have stopped using gadolinium if the GFR is less than
30ml/min/1.73m2. Starting or increasing EPO dose has also been associated
with NSF. This disorder is a systemic disease. Skin disease typically presents
with symmetrical, bilateral fibrotic, indurated papules, plaques or subcutaneous
nodules. Lesions start in the hands, feet and ankles and move proximally.
Rarely are lesions found on the abdomen and the head is spared. Lesions are
edematous and may look like cellulitits initially. Sharp pain, pruritis and
burning are also a feature. Joints lose flexibility due to fibrosis. Histological
findings are variable: Increased dermal cellularity, CD34+ cells with tram
tracking, collagen bundles, septal involvement and osseous metaplasia are
typical findings. A comprehensive screen for other causes of severe skin
disease is important. NSF has a poor prognosis and one review of the literature
suggests a mortality of 28%. There is no treatment for NSF other than recovery
of renal function. The preventative measure of avoiding gadolinium exposure in
those at risk has significantly reduced the incidence of NSF. A good website by
The International Center for Nephrogenic Systemic Fibrosis
Research (ICNSFR) can be found at http://www.icnfdr.org/.
cases of this disorder were noted between 1997 and 2000. Nearly all have been
in patients with gadolinium exposure. Most of these patients had severe CKD or
were on dialysis. The onset of NSF post gadolinium exposure ranges from 2
months to 15 years. The risk of NSF in dialysis patients has been reported as
between 2.5 and 5%. Most centers have stopped using gadolinium if the GFR is less than
30ml/min/1.73m2. Starting or increasing EPO dose has also been associated
with NSF. This disorder is a systemic disease. Skin disease typically presents
with symmetrical, bilateral fibrotic, indurated papules, plaques or subcutaneous
nodules. Lesions start in the hands, feet and ankles and move proximally.
Rarely are lesions found on the abdomen and the head is spared. Lesions are
edematous and may look like cellulitits initially. Sharp pain, pruritis and
burning are also a feature. Joints lose flexibility due to fibrosis. Histological
findings are variable: Increased dermal cellularity, CD34+ cells with tram
tracking, collagen bundles, septal involvement and osseous metaplasia are
typical findings. A comprehensive screen for other causes of severe skin
disease is important. NSF has a poor prognosis and one review of the literature
suggests a mortality of 28%. There is no treatment for NSF other than recovery
of renal function. The preventative measure of avoiding gadolinium exposure in
those at risk has significantly reduced the incidence of NSF. A good website by
The International Center for Nephrogenic Systemic Fibrosis
Research (ICNSFR) can be found at http://www.icnfdr.org/.
Posted by Andrew Malone
