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Systemic Fibrosis (systemic + fibrosis)
Kinds of Systemic Fibrosis Selected AbstractsHypercalcemia and Overexpression of CYP27B1 in a Patient With Nephrogenic Systemic Fibrosis: Clinical Vignette and Literature Review,,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 6 2009Vivian Y Pao Abstract Nephrogenic systemic fibrosis (NSF) is a disease of thickened, hard, hyperpigmented skin lesions with or without systemic fibrosis occurring in patients with renal insufficiency and associated with the administration of gadolinium-containing contrast. The pathogenesis of this disease is unclear, and there is no definitive treatment. We describe a 71-yr-old patient with stable chronic lymphocytic leukemia (CLL), end-stage renal disease (ESRD), and NSF who presented with hypercalcemia in 2006. Before onset of renal insufficiency in 2002, serum calcium, phosphorus, and PTH levels were normal. In 2004, the patient began hemodialysis, and he was diagnosed with NSF in 2005, shortly after undergoing an MRI with gadolinium contrast administration. Over the next 6 mo, albumin-corrected serum total calcium levels rose from 9.9 to 13.1 mg/dl (normal range, 8.5,10.5 mg/dl) with normal serum phosphorus levels. On admission in September 2006, 1,25-dihydroxyvitamin D [1,25(OH)2D] levels were elevated at 130.7 pg/ml (normal range, 25.1,66.1 pg/ml). Biopsy of an NSF lesion showed increased 25-hydroxyvitamin D3,1-, hydroxylase (CYP27B1) immunostaining compared with the biopsy from a normal control. This is the first reported association of NSF with hypercalcemia caused by elevated 1,25(OH)2D levels. This metabolic disturbance should be sought in future cases to determine a connection between NSF, 1,25(OH)2D metabolism, and CYP27B1 activation in the skin, which may shed light on the pathogenesis of this unusual local and systemic fibrosing disorder. [source] Gadolinium-Induced Nephrogenic Systemic Fibrosis Is Associated with Insoluble Gd Deposits in Tissues: In Vivo Transmetallation Confirmed by MicroanalysisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 12 2009Charu Thakral Background: Nephrogenic systemic fibrosis (NSF) is an extremely debilitating systemic fibrosing disorder affecting renal failure patients. The association of NSF with gadolinium (Gd) containing magnetic resonance contrast agents was noted in 2006. Gd deposition in skin biopsies was demonstrated shortly thereafter. Methods: We used automated scanning electron microscopy (SEM)/energy dispersive x-ray spectroscopy for in situ quantitative analysis of insoluble Gd-containing deposits, recording multi-elemental composition and spatial distribution of detected features. Results: Gd was detected in all 29 patients (53 of 57 skin biopsies) with NSF, biopsied from 2 weeks to 3 years after Gd exposure. Gd concentration ranged from 1 to 2270 cps/mm2 and was detected predominantly in the deep dermis and subcutaneous fibrous septa. Gd was found associated with Ca, P and sometimes Fe or Zn. Patients with sequential biopsies showed persistence or increase of Gd in tissues (6 of 11). Transmission electron microscopy (TEM) identified the intracellular deposits in fibrocytes and macrophages. Conclusions: The demonstration of insoluble tissue deposits of Gd with co-associated elements clearly confirms in vivo transmetallation and dissociation of soluble Gd-chelates. Toxic Gd3+ may trigger fibrosis under permissive conditions, e.g., in renal insufficiency. Pathologists and clinicians need to be aware of this serious but preventable disease. [source] The Outcome of Patients with Nephrogenic Systemic Fibrosis after Successful Kidney TransplantationAMERICAN JOURNAL OF TRANSPLANTATION, Issue 3 2010N. Leung Nephrogenic systemic fibrosis (NSF) is a debilitating disease in patients with severely diminished kidney function. Currently, no standard treatment exists but improvement has been reported after restoration of kidney function. We retrospectively studied 17 NSF patients with and without successful kidney transplantation (KTx) to evaluate the effects of KTx on NSF. Nine of the 11 KTx developed NSF pretransplant whereas two developed NSF immediately after KTx with delayed graft function. Two of the six dialysis patients had previous failed kidney transplants. Age and sex were well matched. All but one patient was dialysis dependent at the time of NSF. Median follow-up was 35 months for KTx patients and 9 months for dialysis patients. Kidney transplants achieved adequate renal function with median serum creatinine of 1.4 (0.9,2.8) mg/dL and a glomerular filtration rate of 42 (19,60) mL/min/1.73 m2. NSF improved in 54.6% of the transplanted patients and 50% of the nontransplanted patients (p = 0.86). Two KTx patients had complete resolution of their symptoms whereas four had partial improvement. Improvement in the dialysis patients was all partial. Successful KTx did not insure improvement in NSF and in fact appeared to have no significant benefit over dialysis. [source] Gadolinium Is Not the Only Trigger for Nephrogenic Systemic Fibrosis: Insights From Two Cases and Review of the Recent LiteratureAMERICAN JOURNAL OF TRANSPLANTATION, Issue 10 2007I. M. Wahba Nephrogenic systemic fibrosis (NSF) is an emerging fibrosing disease with serious consequences in patients with acute and chronic kidney disease including solid organ and renal transplant recipients. It has recently been linked to gadolinium exposure. Almost all recently reported cases of NSF were found to be preceded by gadolinium administration, which led the FDA to issue a warning against the use of gadolinium in patients with moderate-to-severe reduction in the glomerular filtration rate. We report two organ transplant recipients who developed NSF and in whom extensive record review failed to document any prior gadolinium exposure. We then critically review the recently published literature linking NSF and gadolinium and we propose other possible triggers. We conclude that gadolinium is not the only trigger for NSF, and that the search for other triggers should be sought. We believe that this information is an important addition to the NSF literature, such that the definitive etiology and pathogenesis of NSF can be researched. [source] Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic UpdateAMERICAN JOURNAL OF TRANSPLANTATION, Issue 9 2006M. Maloo 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. [source] Spectrum of ALMS1 variants and evaluation of genotype-phenotype correlations in Alström syndrome,HUMAN MUTATION, Issue 11 2007Jan D. Marshall Abstract Alström syndrome is a monogenic recessive disorder featuring an array of clinical manifestations, with systemic fibrosis and multiple organ involvement, including retinal degeneration, hearing loss, childhood obesity, diabetes mellitus, dilated cardiomyopathy (DCM), urological dysfunction, and pulmonary, hepatic, and renal failure. We evaluated a large cohort of patients with Alström syndrome for mutations in the ALMS1 gene. In total, 79 disease-causing variants were identified, of which 55 are novel mutations. The variants are primarily clustered in exons 8, 10, and 16, although we also identified novel mutations in exons 12 and 18. Most alleles were identified only once (45/79), but several were found recurrently. Founder effects are likely in families of English and Turkish descent. We also identified 66 SNPs and assessed the functional significance of these variants based on the conserved identity of the protein and the severity of the resulting amino acid substitution. A genotype,phenotype association study examining 18 phenotypic parameters in a subset of 58 patients found suggestive associations between disease-causing variants in exon 16 and the onset of retinal degeneration before the age of 1 year (P = 0.02), the occurrence of urological dysfunction (P = 0.02), of DCM (P = 0.03), and of diabetes (P = 0.03). A significant association was found between alterations in exon 8 and absent, mild, or delayed renal disease (P = 0.0007). This data may have implications for the understanding of the molecular mechanisms of ALMS1 and provides the basis for further investigation of how alternative splicing of ALMS1 contributes to the severity of the disease. Hum Mutat 28(11),1114,1123, 2007. Published 2007 Wiley-Liss, Inc. [source] Malignant fibrous histiocytoma complicating nephrogenic systemic fibrosis post liver transplantationINTERNAL MEDICINE JOURNAL, Issue 9 2009K. So Abstract A 46-year-old man with cirrhosis secondary to hepatitis C virus infection and alcohol underwent orthotopic liver transplantation, which required urgent re-grafting because of biliary sepsis from necrosis of the left liver lobe. Recovery was complicated by renal failure and nephrogenic systemic fibrosis (probably related to intravenous gadolinium exposure). He subsequently developed a malignant fibrous histiocytoma. We present this case highlighting the occurrence of two rare conditions in the same patient following liver transplantation. We believe this is the first case of its kind to be reported. [source] Gadolinium-induced nephrogenic systemic fibrosis: communication breakdown among specialists or intuitive stroke of genius?INTERNAL MEDICINE JOURNAL, Issue 1 2009K. Kraetschmer No abstract is available for this article. [source] Nephrogenic systemic fibrosis: is any contrast safe in renal failure?INTERNAL MEDICINE JOURNAL, Issue 7 2007Z. H. Endre No abstract is available for this article. [source] Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: a case series of nine patients and review of the literatureINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 5 2007Camille E. Introcaso MD Background, Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) is a fibrosing cutaneous disorder recently recognized to have systemic manifestations. The disease is characterized clinically by an acute onset of hardening and thickening of the skin of the extremities and trunk, often resulting in flexion contractures, and histologically by an increase in spindle-shaped cells, collagen, and sometimes mucin deposition in the dermis. The only common exposure amongst patients is acute or chronic renal failure. The pathophysiology of the disease remains to be elucidated, and there is currently no consistently effective treatment for this unremitting disease. Methods, We report a case series of nine patients seen at the University of Pennsylvania between 1998 and mid-2004. The clinical, laboratory, and pathologic data of these patients are reviewed. Results, All patients had renal disease, received peritoneal or hemodialysis, and five had received at least one renal transplant. All patients had characteristic fibrotic cutaneous lesions involving the trunk, extremities, or both, and eight of the nine patients had scleral plaques. There were no other common findings amongst the histories, medications, or laboratory results of the patients. Conclusion, Our report confirms the clinical and histologic characteristics of NFD that have been described previously, and raises new issues regarding the possible subtypes. A review of the current literature stresses that further basic science and translational studies are necessary to understand the disease mechanism and to propose effective therapy, and emphasizes the importance of recognizing the systemic effects of NFD. [source] Hypercalcemia and Overexpression of CYP27B1 in a Patient With Nephrogenic Systemic Fibrosis: Clinical Vignette and Literature Review,,JOURNAL OF BONE AND MINERAL RESEARCH, Issue 6 2009Vivian Y Pao Abstract Nephrogenic systemic fibrosis (NSF) is a disease of thickened, hard, hyperpigmented skin lesions with or without systemic fibrosis occurring in patients with renal insufficiency and associated with the administration of gadolinium-containing contrast. The pathogenesis of this disease is unclear, and there is no definitive treatment. We describe a 71-yr-old patient with stable chronic lymphocytic leukemia (CLL), end-stage renal disease (ESRD), and NSF who presented with hypercalcemia in 2006. Before onset of renal insufficiency in 2002, serum calcium, phosphorus, and PTH levels were normal. In 2004, the patient began hemodialysis, and he was diagnosed with NSF in 2005, shortly after undergoing an MRI with gadolinium contrast administration. Over the next 6 mo, albumin-corrected serum total calcium levels rose from 9.9 to 13.1 mg/dl (normal range, 8.5,10.5 mg/dl) with normal serum phosphorus levels. On admission in September 2006, 1,25-dihydroxyvitamin D [1,25(OH)2D] levels were elevated at 130.7 pg/ml (normal range, 25.1,66.1 pg/ml). Biopsy of an NSF lesion showed increased 25-hydroxyvitamin D3,1-, hydroxylase (CYP27B1) immunostaining compared with the biopsy from a normal control. This is the first reported association of NSF with hypercalcemia caused by elevated 1,25(OH)2D levels. This metabolic disturbance should be sought in future cases to determine a connection between NSF, 1,25(OH)2D metabolism, and CYP27B1 activation in the skin, which may shed light on the pathogenesis of this unusual local and systemic fibrosing disorder. [source] Gadolinium-Induced Nephrogenic Systemic Fibrosis Is Associated with Insoluble Gd Deposits in Tissues: In Vivo Transmetallation Confirmed by MicroanalysisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 12 2009Charu Thakral Background: Nephrogenic systemic fibrosis (NSF) is an extremely debilitating systemic fibrosing disorder affecting renal failure patients. The association of NSF with gadolinium (Gd) containing magnetic resonance contrast agents was noted in 2006. Gd deposition in skin biopsies was demonstrated shortly thereafter. Methods: We used automated scanning electron microscopy (SEM)/energy dispersive x-ray spectroscopy for in situ quantitative analysis of insoluble Gd-containing deposits, recording multi-elemental composition and spatial distribution of detected features. Results: Gd was detected in all 29 patients (53 of 57 skin biopsies) with NSF, biopsied from 2 weeks to 3 years after Gd exposure. Gd concentration ranged from 1 to 2270 cps/mm2 and was detected predominantly in the deep dermis and subcutaneous fibrous septa. Gd was found associated with Ca, P and sometimes Fe or Zn. Patients with sequential biopsies showed persistence or increase of Gd in tissues (6 of 11). Transmission electron microscopy (TEM) identified the intracellular deposits in fibrocytes and macrophages. Conclusions: The demonstration of insoluble tissue deposits of Gd with co-associated elements clearly confirms in vivo transmetallation and dissociation of soluble Gd-chelates. Toxic Gd3+ may trigger fibrosis under permissive conditions, e.g., in renal insufficiency. Pathologists and clinicians need to be aware of this serious but preventable disease. [source] Special issue: Nephrogenic systemic fibrosisJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 6 2009Tim Leiner MD [source] Nephrogenic systemic fibrosis in liver disease: A systematic review,JOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 6 2009Sameer M. Mazhar MD Abstract Nephrogenic systemic fibrosis (NSF) may develop in patients with liver disease, a fact highlighted by Food and Drug Administration (FDA) announcements cautioning against the use of gadolinium-based contrast agents (GBCAs) in select liver disease patients. The purpose of this systematic literature review is to characterize the risk of NSF in patients with liver disease. All published articles on NSF from September 2000 through August 2008, were identified via PubMed searches and examination of articles' reference lists. Two reviewers independently read each article and identified unique patients with biopsy-proven or suspected NSF. Data on demographics, liver status, renal status, and GBCA exposure were collected. A total of 324 articles were reviewed, with 108 articles containing case descriptions of 335 unique NSF patients. After excluding the 95/335 (28%) patients in whom the presence or absence of liver disease was uncertain, liver disease was confirmed present in 41/239 (17%) patients. Renal insufficiency could be assessed in 35 of the liver disease patients; severe renal insufficiency, defined as a glomerular filtration rate (GFR) or estimated GFR (eGFR) <30 mL/min/1.73 m2 or dialysis requirement, was present in 34/35 (97%) patients. The lone patient who developed NSF with mild/moderate renal insufficiency was atypical and received a total gadodiamide load of 0.76 mmol/kg over a 10-week period periliver transplantation. The published medical literature demonstrates that patients with liver disease who develop NSF also have severe renal insufficiency, suggesting that liver disease does not confer a risk for NSF beyond that of the underlying renal insufficiency. J. Magn. Reson. Imaging 2009;30:1313,1322. © 2009 Wiley-Liss, Inc. [source] Renovascular imaging in the NSF EraJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 6 2009Giles Roditi MD Abstract The detection of the association between nephrogenic systemic fibrosis (NSF), a rare but potentially life-threatening disease only encountered in patients with severely impaired renal function, and the previous administration of some Gd-chelates has cast a shadow on the administration of Gd-chelates in patients with chronic renal failure. So far, contrast-enhanced MR-angiography (MRA) was considered the best diagnostic modality in patients with suspected renal disease. This review explores the most appropriate use of renal MRA with a focus on newly developed nonenhanced MRA techniques. Nonenhanced MRA techniques mainly based on SSFP with ECG-gating allow for acceptable spatial resolution to visualize at least the proximal parts of the renal arteries. In addition functional renal imaging techniques and their current clinical role are critically appreciated. J. Magn. Reson. Imaging 2009;30:1323,1334. © 2009 Wiley-Liss, Inc. [source] Nephrogenic systemic fibrosis: The need for accurate case reportingJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 5 2009Alberto Spinazzi MD [source] Gadolinium-based contrast agents and their potential role in the pathogenesis of nephrogenic systemic fibrosis: The role of excess ligandJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 5 2008Martin A. Sieber PhD Abstract Purpose To investigate the role of excess ligand present in gadolinium (Gd) -based contrast agents in the development of nephrogenic systemic fibrosis (NSF). Using a dosing regimen to simulate the exposure seen in patients with severe renal impairment, we investigated the effect of excess ligand on Gd-deposition and the depletion of endogenous ions. Materials and Methods Gadodiamide and gadoversetamide were formulated with 0%, 5%, and 10% excess ligand. Forty-two, healthy, male Hannover Wistar rats received daily intravenous injections of each formulation over a period of 20 days. At the end of the study, histopathological analysis of the skin was performed and the concentrations of Gd, Zn, and Cu were measured in several tissues. The levels of Zn in the urine were also measured. Results The most severe skin lesions were observed after injection of formulations containing 0% free ligand and in those animals with the highest Gd concentrations in the skin. There were no significant reductions in the levels of Zn or Cu observed in the skin; however, the levels of Zn in the urine were elevated following administration of formulations with the highest amount of excess ligand. Conclusion Our findings suggest that there is an inverse correlation between the amount of excess ligand present in Gd-containing contrast agents and the amount of Gd in the tissue, and further underline the importance of the inherent stability of these agents in the development of NSF. J. Magn. Reson. Imaging 2008;27:955,962. © 2008 Wiley-Liss, Inc. [source] Safety update on the possible causal relationship between gadolinium-containing MRI agents and nephrogenic systemic fibrosisJOURNAL OF MAGNETIC RESONANCE IMAGING, Issue 5 2007Michael Pedersen PhD [source] Gadolinium and nephrogenic systemic fibrosis: Association or causation (Review Article)NEPHROLOGY, Issue 3 2008JAGADEESH KURTKOTI SUMMARY: With widespread availability of magnetic resonance imaging (MRI), it has become standard practice for patients with severe renal impairment or previous severe reactions to iodine-containing contrast media to receive gadolinium-based MRI contrast agents instead of traditional radiographic contrast agents, particularly for magnetic resonance angiography. However, there is growing concern about the use of gadolinium contrast agents in the presence of severe renal insufficiency, because of increasing reports of nephrogenic fibrosing dermopathy (NFD)/nephrogenic systemic fibrosis (NSF), associated with the exposure to certain gadolinium-containing contrast agents. In this review we explore the causal link between gadolinium exposure and NSF, using an established system of epidemiological criteria proposed by Bradford Hill. Though the current evidence makes gadolinium a strong suspect as an aetiologic agent for NSF in the presence of severe renal failure, the die is not cast yet. At this stage there needs to be cautious approach to the use of gadolinium-containing contrast agents in the presence of severe renal failure (glomerular filtration rate <30 mL/min per 1.73 m2). [source] The Outcome of Patients with Nephrogenic Systemic Fibrosis after Successful Kidney TransplantationAMERICAN JOURNAL OF TRANSPLANTATION, Issue 3 2010N. Leung Nephrogenic systemic fibrosis (NSF) is a debilitating disease in patients with severely diminished kidney function. Currently, no standard treatment exists but improvement has been reported after restoration of kidney function. We retrospectively studied 17 NSF patients with and without successful kidney transplantation (KTx) to evaluate the effects of KTx on NSF. Nine of the 11 KTx developed NSF pretransplant whereas two developed NSF immediately after KTx with delayed graft function. Two of the six dialysis patients had previous failed kidney transplants. Age and sex were well matched. All but one patient was dialysis dependent at the time of NSF. Median follow-up was 35 months for KTx patients and 9 months for dialysis patients. Kidney transplants achieved adequate renal function with median serum creatinine of 1.4 (0.9,2.8) mg/dL and a glomerular filtration rate of 42 (19,60) mL/min/1.73 m2. NSF improved in 54.6% of the transplanted patients and 50% of the nontransplanted patients (p = 0.86). Two KTx patients had complete resolution of their symptoms whereas four had partial improvement. Improvement in the dialysis patients was all partial. Successful KTx did not insure improvement in NSF and in fact appeared to have no significant benefit over dialysis. [source] Gadolinium Is Not the Only Trigger for Nephrogenic Systemic Fibrosis: Insights From Two Cases and Review of the Recent LiteratureAMERICAN JOURNAL OF TRANSPLANTATION, Issue 10 2007I. M. Wahba Nephrogenic systemic fibrosis (NSF) is an emerging fibrosing disease with serious consequences in patients with acute and chronic kidney disease including solid organ and renal transplant recipients. It has recently been linked to gadolinium exposure. Almost all recently reported cases of NSF were found to be preceded by gadolinium administration, which led the FDA to issue a warning against the use of gadolinium in patients with moderate-to-severe reduction in the glomerular filtration rate. We report two organ transplant recipients who developed NSF and in whom extensive record review failed to document any prior gadolinium exposure. We then critically review the recently published literature linking NSF and gadolinium and we propose other possible triggers. We conclude that gadolinium is not the only trigger for NSF, and that the search for other triggers should be sought. We believe that this information is an important addition to the NSF literature, such that the definitive etiology and pathogenesis of NSF can be researched. [source] Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic UpdateAMERICAN JOURNAL OF TRANSPLANTATION, Issue 9 2006M. Maloo 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. [source] Induction of the expression of profibrotic cytokines and growth factors in normal human peripheral blood monocytes by gadolinium contrast agentsARTHRITIS & RHEUMATISM, Issue 5 2009Peter J. Wermuth Objective Nephrogenic systemic fibrosis (NSF) is a severe fibrosing disorder occurring in patients with renal insufficiency. The majority of patients with this disorder have documented exposure to magnetic resonance imaging contrast agents containing Gd. The purpose of this study was to examine the effects of gadolinium diethylenetriaminepentaacetic acid bismethylamide (Gd[DTPA-BMA]; Omniscan) as compared with Gd-DTPA and GdCl3 on the expression and production of cytokines and growth factors by normal human peripheral blood monocytes in vitro and to examine whether conditioned media from Gd-exposed peripheral blood monocytes could induce a profibrotic phenotype in dermal fibroblasts. Methods Normal human peripheral blood monocytes isolated by Ficoll-Hypaque gradient centrifugation and plastic adherence were incubated with various concentrations of Gd[DTPA-BMA], Gd-DTPA, or GdCl3. Gene expression of interleukins 4, 6, and 13, interferon-,, tumor necrosis factor ,, transforming growth factor ,, connective tissue growth factor, and vascular endothelial growth factor were assessed by real-time polymerase chain reaction (PCR) analysis. Production and secretion of cytokines and growth factors by Gd compound,exposed monocytes was quantified by enzyme-linked immunosorbent assay proteome multiplex arrays. The effects of conditioned media from the Gd compound,exposed monocytes on the phenotype of normal human dermal fibroblasts were examined by real-time PCR and Western blotting. Results The 3 Gd-containing compounds stimulated the expression and production of numerous cytokines and growth factors by normal human peripheral blood monocytes. Conditioned media from these cells induced a profibrotic phenotype in normal human dermal fibroblasts. Conclusion The 3 Gd-containing compounds studied induce potent cellular responses in normal human peripheral blood monocytes, which may participate in the development of tissue fibrosis in NSF. [source] Nephrogenic systemic fibrosis and gadolinium exposure: Association and lessons for idiopathic fibrosing disordersARTHRITIS & RHEUMATISM, Issue 10 2007Shawn E. Cowper No abstract is available for this article. [source] Nephrogenic systemic fibrosis in a gadolinium-naive renal transplant recipientAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 1 2008Namrata S Anavekar SUMMARY A 36-year-old woman presented with a 2-year history of multiple, raised, brown papules and indurated skin over her lower legs. She had received a renal transplant 11 years earlier, and had a history of recurrent deep vein thromboses despite a negative thrombophilic screen. The patient had no history of exposure to gadolinium. Histology at this time revealed a light perivascular lymphoid infiltrate, activated fibroblasts and prominent capillary vessels. The patient re-presented 1 year later with persistence of these lesions, in the setting of worsening renal function requiring haemodialysis. Repeat skin biopsies demonstrated increased dermal collagen and angiogenesis. The dermatopathological findings, in association with renal insufficiency and multiple deep vein thromboses, led to the diagnosis of nephrogenic systemic fibrosis. [source] Gadolinium-induced nephrogenic systemic fibrosis in a patient with an acute and transient kidney injuryBRITISH JOURNAL OF DERMATOLOGY, Issue 3 2008R.E. Kalb Summary Nephrogenic systemic fibrosis (NSF) describes a characteristic fibrosing disorder which typically presents with indurated plaques on the trunk and extremities of patients with advanced renal disease. We present a case of biopsy-confirmed NSF in a patient with severe acute kidney injury with no prior history of renal disease. A 64-year-old man with an acute and severe decrease in glomerular filtration rate underwent magnetic resonance imaging studies with gadolinium contrast (OmniscanÔ) and subsequently developed NSF. His renal disease had normalized at the time his skin disease developed. Skin biopsies revealed findings of NSF and scanning electron microscopy with energy-dispersive X-ray spectroscopy confirmed insoluble gadolinium within lesional tissue. [source] Possible role of gadolinium in nephrogenic systemic fibrosis: report of two cases and review of the literatureCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2007Y. L. Lim Summary Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy (NSF/NFD) is a rare fibrosing disorder that occurs in patients with renal failure. It is associated with significant mortality and morbidity. Patients typically present with painful or pruritic indurated plaques involving the limbs and trunk, with sparing of the face. Severity and rapidity of cutaneous progression correlate with poorer prognosis. To date, the management of NSF/NFD remains anecdotal. The aetiological link in NSF/NFD is also yet to be confirmed, but renal dysfunction seems a common feature. Following recent reports of a possible causative role of gadolinium, we present two patients with histologically confirmed NSF/NFD, who had exposure to gadolinium-containing contrast agents 1,2 months before onset of disease. Severity of renal impairment, lack of immediate dialysis after exposure and cumulative dose of gadolinium are possible factors influencing the development of NSF/NFD. The process of transmetallation of gadolinium chelates may occur in patients with renal impairment, leading to precipitation of free gadolinium in the dermis or other organs, causing tissue injury that ultimately leads to the clinical manifestations of NSF/NFD. Although the causative role is not proven, gadolinium-containing contrast agents should be used only if clearly necessary in patients with renal failure. [source] | ||||||||||