Nephrogenic fibrosing dermopathy: response to high-dose intravenous immunoglobulin

Chung, Hee-Chul; Chung, Kee Yang

doi:10.1111/j.1365-2133.2003.05795.x

Cited by 60 publications

(36 citation statements)

References 11 publications

(18 reference statements)

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“…Another three patients were treated with extracorporeal phototherapy also with varying results from improved mobility to complete lesion resolution (18). Other treatment modalities attempted to treat this condition include corticosteroids, calcipotriene ointment, cyclosporine, cyclophosphamide, thalidomide, isotretinoin, alpha interferon, photodynamic therapy and IVIG (5,(17)(18)(19). Due to the small number of patients with this condition, meaningful data relating to their treatment efficacy remains lacking.…”

Section: Nephrogenic Systemic Fibrosis In Liver Transplant Recipientsmentioning

confidence: 99%

Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic Update

Maloo

Abt

Kashyap

et al. 2006

American Journal of Transplantation

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Nephrogenic systemic fibrosis (NSF) is a recently characterized systemic fibrosing disorder developing in the setting of renal insufficiency. NSF's rapidly progressive nature resulting in disability within weeks of onset makes early diagnosis important. Two reports of NSF after liver transplantation are known of. We present three cases of NSF developing within a few months after liver transplantation and review the current literature. Loss of regulatory control of the circulating fibrocyte, its aberrant recruitment, in a milieu of renal failure and a recent vascular procedure appear important in its development. Known current therapies lack consistent efficacy. Only an improvement in renal function has the greatest likelihood of NSF's resolution. Delayed recognition may pose a significant barrier to functional recovery in the ubiquitously deconditioned liver transplant patient. Early recognition and implementation of aggressive physical therapy appear to have the greatest impact on halting its progression.

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Section: Nephrogenic Systemic Fibrosis In Liver Transplant Recipientsmentioning

confidence: 99%

Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic Update

Maloo

Abt

Kashyap

et al. 2006

American Journal of Transplantation

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“…Other therapies with reported benefit include sodium thiosulphate [74], rapamycin [75], imatinib mesylate [76], and pentoxifylline [2]. Limited success has been seen with immune modulators, such as glucocorticoids [77], thalidomide, immunoglobulins [78], and plasmapheresis [79]. A common limitation of these studies is confounding by concomitantly improving renal function.…”

Section: Treatment and Preventionmentioning

confidence: 99%

Gadolinium and nephrogenic systemic fibrosis: an update

2013

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Nephrogenic systemic fibrosis (NSF) is a multisystem disease seen exclusively in patients with renal impairment. It can be severely debilitating and sometimes fatal. There is a strong association with gadolinium-based contrast agents used in magnetic resonance imaging (MRI). Risk factors include renal impairment and proinflammatory conditions, e.g. major surgery and vascular events. Although there is no single effective treatment for NSF, the most successful outcomes are seen following restoration of renal function, either following recovery from acute kidney injury or following renal transplantation. There have been ten biopsy-proved pediatric cases of NSF, with no convincing evidence that children have a significantly altered risk compared with the adult population. After implementation of guidelines restricting the use of gadolinium-based contrast agents in at-risk patients, there has been a sharp reduction in new cases and no new reports in children. Continued vigilance is recommended: screening for renal impairment, use of more stable gadolinium chelates, consideration of non-contrast-enhanced MRI or alternative imaging modalities where appropriate.

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“…Plasmapheresis, intravenous immunoglobulins (IVIG) and ultraviolet (UV-A1) phototherapy have also been proposed (20)(21)(22). In our experience, these treatment strategies have been overall disappointing.…”

Section: Treatment and Prognosismentioning

confidence: 99%

Scleroderma-like Fibrosing Disorders

Boin¹,

Hummers²

2008

Rheumatic Disease Clinics of North America

111

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SynopsisMany conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology and disease associations may allow to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is important to spare the patients from ineffective treatments and inadequate management. In this review, we highlighted nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy), eosinophilic fasciitis (Shulman's syndrome), scleromyxedema and scleredema. These are often detected in the primary care setting and referred to rheumatologists for further evaluation. Rheumatologists must be able to promptly recognize them to provide valuable prognostic information and appropriate treatment options for affected patients.

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Nephrogenic fibrosing dermopathy: response to high-dose intravenous immunoglobulin

Cited by 60 publications

References 11 publications

Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic Update

Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic Update

Gadolinium and nephrogenic systemic fibrosis: an update

Scleroderma-like Fibrosing Disorders

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