Presentation
We present a case of NSF occurred after gadolinium exposure in which the initial presentation mimics an erythema nodosum (EN)-like picture. An initial skin biopsy showed EN. Subsequently the patient developed progressive skin and joints contracture. [ncbi.nlm.nih.gov]
Entire Body System
- Pain
The patients reported significant improvements in subjective pain levels after TPE. Patient 1 reported decreased skin and contracture pain after the 3rd treatment, with similar results for a second series 6 months later. [ncbi.nlm.nih.gov]
Go to the pain clinic. Healthcare providers at a pain clinic can help you learn new ways to control your pain. When should I contact my healthcare provider? Your arms or legs are swollen, or you have trouble moving them. [drugs.com]
- Anemia
[…] may lead to or promote the disease when severe kidney disease and exposure to gadolinium-containing contrast are present include: Use of high-dose erythropoietin ( EPO ), a hormone that promotes the production of red blood cells, often used to treat anemia [mayoclinic.org]
Changes in bone marrow are likely secondary to anemia. Because of the uncommon nature of the meningeal abnormalities, the patient had a right frontal craniotomy and dural biopsy in May 2006. [ajnr.org]
The fact that these patients had significantly worse renal function as compared with most of the control subjects puts them at higher risk for requiring recombinant epoetin therapy for management of anemia. [emedicine.com]
- Leg Swelling
The remaining six patients were more mildly affected (leg swellings and skin induration with/without local pain). [dx.doi.org]
A 46-year-old woman with chronic renal failure, leg swelling, and skin changes. N Engl J Med 2009;361:2166-76. [Pubmed] [40] Gibson SE, Farver CF, Prayson RA. [revistanefrologia.com]
- Fatigue
Physical examination revealed a frail, fatigued white man who was not in acute distress. [archderm.ama-assn.org]
[…] having recognized antifibrotic properties, is a drug of particular interest that I have found most effective in my own practice, but it is a difficult drug to use on those with many comorbidities, as it leads to side effects, including fluid-shifts and fatigue [dermatologyadvisor.com]
Musculoskeletal
- Flexion Contracture
NSF is characterised by skin thickening, tethering and hyperpigmentation; flexion contractures of joints; and extracutaneous fibrosis. [ncbi.nlm.nih.gov]
Her fingers became fixed in flexion. Her hands became claw-like, causing her difficulty buttoning her blouse, and she developed fixed flexion contractures of her knees. [the-rheumatologist.org]
- Joint Stiffness
NSF presents with pain, pruritis, joint stiffness, tightness, swelling of the hands and feet, paraesthesias and burning. 2 Dry skin, weakness and warmth can also occur. [medsafe.govt.nz]
Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain [emedicine.com]
Within weeks, the second patient was unable to walk due to extreme muscle and joint stiffness. In 2003, this patient died from a pulmonary embolism. [ckj.oxfordjournals.org]
Skin
- Skin Thickening
Skin thickening and tethering were assessed using the modified Rodnan skin thickness score (MRSS), and knee joint flexion contractures were measured with a goniometer. [ncbi.nlm.nih.gov]
- Hyperpigmentation
NSF is characterised by skin thickening, tethering and hyperpigmentation; flexion contractures of joints; and extracutaneous fibrosis. [ncbi.nlm.nih.gov]
NSF presents with cutaneous hyperpigmentation and induration and joint contractures, but fibrosis may also develop in other organs. [ard.bmj.com]
- Pruritus
Eventually, flexure contractures develop, with restriction to movement, pain and pruritus. [radiopaedia.org]
Southern California medical center were confronted with a mysterious clinical entity. 1,2 Several patients in their hemodialysis center began experiencing cutaneous induration and erythema of the limbs unassociated with fever, but frequently accompanied by pruritus [appliedradiology.com]
Additionally, many patients report maddening pruritus and/or causalgia. Finally, some patients experience flexion contractures if the disorder occurs over a joint. [emedicine.com]
- Skin Plaque
It is typically characterised by hard, erythematous and indurated skin plaques with surrounding subcutaneous oedema. Distinct papules and subcutaneous nodules can also be seen. Fibrocytes in NSF are immunohistochemically positive for CD34. [ncbi.nlm.nih.gov]
The cutaneous changes include firm and thickened, indurate skin plaques and papules on the extremities and trunk. Histopathology typically shows an increase in dermal fibroblast-like cells associated with mucin deposition. [reumatologiaclinica.org]
- Dry Skin
NSF presents with pain, pruritis, joint stiffness, tightness, swelling of the hands and feet, paraesthesias and burning. 2 Dry skin, weakness and warmth can also occur. [medsafe.govt.nz]
Neurologic
Urogenital
- Kidney Failure
Nephrogenic systemic fibrosis (NSF) is a fibrosing skin disorder that develops in patients with kidney failure and has been linked to exposure to gadolinium-containing contrast agents. [ncbi.nlm.nih.gov]
Based on reported cases currently available, it appears that about 5% of those with kidney failure or kidney problems experience the systemic disorder after receiving a gadolinium agent. [bernllp.com]
Workup
We propose that skin biopsies performed as part of a diagnostic workup for suspected NSF should preferably include the deep dermis and subcutis, in order to assess possible vascular calcification. [ncbi.nlm.nih.gov]
Cowper, Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations, Journal of the American Academy of Dermatology, 10.1016/j.jaad.2010.08.041, 65, 6, (1095-1106.e7), (2011). [doi.org]
Nephrogenic systemic fibrosis: clinicopathological definition and workup recommendations. J Am Acad Dermatol 2011;65(6):1095-106. [29] Kaewlai R, Abujudeh H. Nephrogenic systemic fibrosis. AJR Am J Roentgenol 2012;199(1):W17-23. [revistanefrologia.com]
Nephrogenic systemic fibrosis: clinicopathological definition and workup recommendations, J Am Acad Dermatol, 2011, vol. 65 (pg. 1095 - 1106 ) 37. [ckj.oxfordjournals.org]
Treatment
Because skin changes recurred after discontinuation of imatinib mesylate, the duration for which treatment may be required is undetermined. [ncbi.nlm.nih.gov]
Prognosis
Overall, the prognosis is poor and there is no established therapy that shows a consistent benefit. The purpose of this review is to discuss the clinical spectrum of the disease. [ncbi.nlm.nih.gov]
(Outcomes/Resolutions) The prognosis of Nephrogenic Systemic Fibrosis is generally poor despite suitable treatment The prognosis also depends on the severity of the underlying condition and the involvement of various internal organs Additional and Relevant [dovemed.com]
Etiology
While its histologic characteristics have been well described, the etiology and pathogenesis have not been fully characterized. [ncbi.nlm.nih.gov]
Epidemiology
This article reviews the history, clinical manifestations, epidemiology, histopathology and pathophysiology of this disabling disease. [ncbi.nlm.nih.gov]
In addition, epidemiologic data have yielded several distinct clinical patterns of disease onset. [doi.org]
Pathophysiology
OBJECTIVE: The purpose of this article is to discuss nephrogenic systemic fibrosis (NSF) in detail regarding its history, possible pathophysiology, clinical and pathologic presentations, diagnosis, and implications for the radiology community. [ncbi.nlm.nih.gov]
Prevention
Other than limiting exposure to gadolinium based contrast agents, no effective preventive methods have been reported. [ncbi.nlm.nih.gov]
The only known measure for prevention of NSF is the non-use or cautious use of GBCAs in patients with renal impairment, including preferential use of safer, macrocyclic GBCAs. [en.wikipedia.org]
Currently, it is not possible to prevent Nephrogenic Systemic Fibrosis. [dovemed.com]