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Nephrogenic Systemic Fibrosis Clinical Presentation

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Feb 08, 2016
 

History

All patients with nephrogenic systemic fibrosis have a history of renal insufficiency of varying severity and duration and gadolinium exposure (with the exception of one report involving transplant recipients in which the donor exposure to gadolinium was not reported). A few have primarily liver disease. Most patients have had treatments that include hemodialysis, peritoneal dialysis, or renal transplantation. However, neither dialysis nor transplantation is a prerequisite for nephrogenic systemic fibrosis.

Thomsen et al[9] noted that more than 90% of proven nephrogenic systemic fibrosis cases are related to gadodiamide (Omniscan) and some to gadopentetate (Magnevist).[10]

Many patients with nephrogenic systemic fibrosis have calcium pathology. Traumatic calcinosis cutis in a dialysis patient with nephrogenic systemic fibrosis has been reported. Osseous metaplasia in the setting of nephrogenic systemic fibrosis has been noted.[36] A variant of nephrogenic systemic fibrosis with osteoclastlike giant cells, which has been termed a syndrome of dysregulated matrix remodeling, has been noted. Calciphylaxis and metastatic calcification associated with nephrogenic systemic fibrosis has also been reported.[28]

Patients may have a history of a tightening or a thickening of the skin. Stiffening of the hands and flexion contractures can also occur.

Some patients may have a history of preceding episodes of severe anasarca, recent vascular surgery, or hypercoagulability.

Some patients have known concurrent liver disease and/or neoplasia. A history of liver problems may be present. Some patients may have chronic hepatitis C.

The disease can develop days, months, or years after undergoing dialysis or after renal failure starts.

Patients may report that their skin is shiny.

This condition can develop slowly.

Pain or pruritus can be a prominent feature in many patients and can be a major component of their disability.

Some patients may have taken immunosuppressive medications.

Nephrogenic systemic fibrosis has been reported in 2 patients with systemic lupus erythematosus.[37]

Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic systemic fibrosis) has been noted.[38]

Nephrogenic systemic fibrosis has been described in a renal transplant recipient with tubulointerstitial nephritis and uveitis.[39]

Glaich et al[40] noted generalized elastolysis following nephrogenic systemic fibrosis.

Kucher et al[41] noted nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure.

Edward et al[42] noted cutaneous mucinosis associated with dermatomyositis and nephrogenic systemic fibrosis. They reported on fibroblast hyaluronan synthesis and the effect on patients' serum.

Saenz et al[43] noted nephrogenic systemic fibrosis with involvement of the dura mater.

Naylor et al[44] reported on a 65-year-old woman with nephrogenic systemic fibrosis with septal panniculitis whose biopsy specimen demonstrated unique histologic features of septal panniculitis with lymphocytic aggregates and Miescher radial granulomas mimicking erythema nodosum.

Neuromuscular involvement can occur in nephrogenic systemic fibrosis, and it can be documented by electromyography/nerve conduction studies. These findings may be difficult to appreciate clinically because of joint and skin fibrosis.[45]

Nephrogenic systemic fibrosis has been reported in a 14-year-old-girl with new-onset systemic lupus erythematosus and acute lupus nephritis after gadolinium exposure.[46]

Goddard et al[47] noted nephrogenic systemic fibrosis with recurrence after allograft failure.

Metformin use might influence nephrogenic systemic fibrosis, but this is not proven.[19]

Wahba et al[1] noted 2 organ transplant recipients who they stated had had no gadolinium exposure and yet developed nephrogenic systemic fibrosis. This report is of uncertain significance because (1) the nephrogenic systemic fibrosis tissue was not examined for gadolinium in the manner of High et al[48] and (2) the history of the apposite organ donors was not obtained; thus, gadolinium may have been present in the bodies of the patients reported by Wahba et al.

Gathings et al in 2014 noted that GAP appeared as erythematous plaques, with a diameter of 0.5-2.5 cm. One patient experience pruritus and the other did not. Histopathologically, these plaques demonstrated eosinophilic, collagenous, ovoid or round bodies (sclerotic bodies) in a variety of calcification. Previously, these sclerotic bodies were thought to be pathognomonic for nephrogenic systemic fibrosis, which required chronic renal insufficiency and imaging with gadolinium; this was not the case in these two patients.[49]

Ross in 2014 noted nephrogenic systemic fibrosis in a gadolinium-naïve woman that was treated successfully with oral sirolimus.[50]

It has been found that in patients with chronic liver disease, the incidence of nephrogenic systemic fibrosis after gadolinium exposure is roughly equal to controls.[51]

Perforating osteoma cutis has been noted in a patient with long-standing nephrogenic systemic fibrosis.[52]

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Physical

Nephrogenic systemic fibrosis manifests with induration, thickening, and hardening of the skin with brawny hyperpigmentation. Distinct papules and subcutaneous nodules can also be seen. Cellulitis is commonly suspected. The skin can have a peau d'orange appearance, and plaques may have an amoeboid advancing edge. The skin is often shiny and hard to the touch. A woody consistency is typical.

The extremities are the most common areas of involvement, followed by the trunk. The face is almost never involved. Yellow palmar papules resembling cutaneous calcinosis have been reported. In addition, yellow scleral plaques have been reported in patients with nephrogenic systemic fibrosis. Solomon et al[53] noted nephrogenic systemic fibrosis mimicking inflammatory breast carcinoma.

Note the images below.

Nephrogenic fibrosing dermopathy on the abdomen, d Nephrogenic fibrosing dermopathy on the abdomen, demonstrating a peau d'orange appearance.
Nephrogenic systemic fibrosis on the abdomen, demo Nephrogenic systemic fibrosis on the abdomen, demonstrating a peau d'orange appearance. A keloid from a previous surgery is present.
Nephrogenic systemic fibrosis on the leg, demonstr Nephrogenic systemic fibrosis on the leg, demonstrating a bound-down and sclerotic appearance and texture.
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Causes

The cause of nephrogenic systemic fibrosis is the connexation of renal insufficiency and gadolinium exposure from imaging studies. The exact degree of renal insufficiency that sets up the development of nephrogenic systemic fibrosis is not known. Risk factors include advanced chronic kidney disease (stages 4 and 5) and acute or chronic inflammatory insults. The US Food and Drug Administration (FDA) has updated its public health advisory to include patients with moderate renal insufficiency (chronic kidney disease stage 3).[54] Metformin use might influence nephrogenic systemic fibrosis, but this is not proven.[19]

Gadolinium-based contrast agents (Ablavar, Eovist, Magnevist, MultiHance, Omniscan, OptiMARK, ProHance) have recently been linked to the development of nephrogenic systemic fibrosis. The disease has occurred in patients with moderate- to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or magnetic resonance angiography. As of late December 2006, the FDA had received reports of 90 such cases. Worldwide, the FDA has reported more than 200 cases.

On September 9, 2010, the FDA announced it is requiring that gadolinium-based contrast agents (GBCAs) carry new warnings on their labels about the risk for nephrogenic systemic fibrosis and its association when administered to certain patients with kidney disease. The FDA's review of the safety of the most widely used GBCAs determined that Magnevist, Omniscan, and Optimark are associated with a greater risk for nephrogenic systemic fibrosis than other GBCAs. These 3 agents will be contraindicated for use in certain patients with kidney disease, while other gadolinium contrast agents will carry a warning. Data suggest that nephrogenic systemic fibrosis may follow the administration of any GBCA, and the FDA continues to assess the safety of each GBCA to better estimate its nephrogenic systemic fibrosis risks.[55]

Nephrogenic systemic fibrosis is a debilitating and sometimes fatal disease. 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 deep in the hip bones or ribs; and muscle weakness.

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Coauthor(s)

Shawn Cowper, MD Director, NSF Registry Project, Associate Professor, Departments of Dermatology and Pathology, Yale University School of Medicine

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bracco pharmaceuticals; Ecron-Acunova.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

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Nephrogenic fibrosing dermopathy on the abdomen, demonstrating a peau d'orange appearance.
Nephrogenic systemic fibrosis on the abdomen, demonstrating a peau d'orange appearance. A keloid from a previous surgery is present.
Nephrogenic systemic fibrosis on the leg, demonstrating a bound-down and sclerotic appearance and texture.
 
 
 
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