| Abstract|| |
Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, is a rare fibrosing disorder of skin and internal organs that occurs in the context of renal insufficiency, particularly in those who have undergone dialysis. It was first described in 1997 in the United States. The first non Western series was reported from Kolkata, India in 2005 the etiology is unknown. High-dose erythropoietin and use of gadolinium-containing contrast media are currently acknowledged to be important risk factors. The pathogenesis is multifactorial. The current hypothesis supports the role of circulating fibroblast precursors (fibrocytes) and the elaboration of circulating fibrogenic factors ( e.g., transforming growth factor beta 1). The clinical characteristics resemble scleromyxedema with a typical distribution pattern of its own. The histopathological hallmarks are spindle-shaped cells in the background of dermal fibrosis and deposition of collagen and mucin. Dual immunolabelling with CD34 and procollagen is diagnostic. There are no standard, specific and universally satisfactory treatments. The natural history is progressive with systemic manifestations conferring a poor outcome.
Keywords: Dermopathy, erythropoietin, nephrogenic fibrosis, gadolinium, scleromyxedema
|How to cite this article:|
Panda S. Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy: A decade-old disease. Indian J Dermatol 2007;52:125-30
|How to cite this URL:|
Panda S. Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy: A decade-old disease. Indian J Dermatol [serial online] 2007 [cited 2019 Dec 12];52:125-30. Available from: http://www.e-ijd.org/text.asp?2007/52/3/125/35089
| Background|| |
Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy (NFD), is a novel, acquired, rare, idiopathic, progressive, scleromyxedema-like fibrosing disorder of the skin. It is increasingly being recognized to have fibrosing systemic manifestations that are known to occur almost exclusively in the context of renal insufficiency, many of the patients being on dialysis therapy or having undergone renal transplantation.,
In 1997, a team at Yale University led by Shawn Cowper first documented NSF as a pseudosclerodermatous fibrosing entity limited to skin in association with renal failure. Subsequent autopsy studies have identified apparent multiorgan fibrosis with involvement of skeletal muscle, myocardium, lungs, kidneys, testes, dura mater etc.
While the specific cause of NSF remains to be established beyond doubt, the pathogenesis appears to be multifactorial with the postulated involvement of circulating fibrocytes. The natural history of the disease is fairly uncertain, although it is generally acknowledged to be a disabling and sometimes fatal disorder. Similarly, no standard therapy currently exists. Rare cases of partial-to-complete spontaneous resolution have been reported in the absence of specific therapy.
| Nomenclature|| |
In 2000, the primary report by Cowper et al. in the Lancet carried the rather unwieldy appellation of the condition, viz. 'scle-romyxedema-like disease in renal-dialysis patients'. Some time later, it came to be known as 'nephrogenic fibrosing dermopathy'. Still later, Jimenez et al. and Levine et al. suggested 'dialysis-associated systemic fibrosis' as a new and more accurate name for this disease; however, it was pointed out that this hardly solves the nosological confusion as the condition may emerge even in patients without dialysis.
With the increasing recognition that the fibrosis associated with NFD can extend beyond dermis and, as part of a systemic fibrosing disorder, it can indeed involve a plethora of internal organs, in 2005, Daram et al. suggested that 'nephrogenic systemic fibrosis' would be a more appropriate term for this disease entity. Currently, NFD and NSF are being interchangeably used to describe the same condition. However, as the review of the recent literature on the subject suggests, more and more authors are favoring the use of NSF to designate the condition.
| Epidemiology|| |
Since its first description ten years back, almost all the reports and series devoted to NSF have involved cases in the Western hemisphere (North America, mainly the United States and Europe). , The geographical cluster expanded in 2004 when NFD was documented in a woman of Chinese descent from Singapore.  The following year, the first and as of now the largest series of NFD cases in the Eastern hemisphere was reported from Kolkata.  This series has since grown further to the current total of seven. , Around 175 cases have been notified thus far in the Yale University NSF registry. The age of patients with NSF ranges from 8 to 87 years at the time of onset, with a mean age of 46.4 years.  NSF has been known to affect children, although very rarely. 
| Etiology|| |
Though the risk factors for NFD have been studied extensively, a definite correlation is yet to be established. Possible correlations with severity of renal failure, history of hypothyroidism, thrombotic episodes, dependent edema and vascular procedures have been reported. 
There is a school of thought that strongly avers that NSF, being a novel condition, must be related to a comparatively new drug, diagnostic agent or environmental exposure. Following this hypothesis, exposure to newly discovered medications, namely, high-dose (>18,000 U) erythropoietin therapy and gadolinium-containing contrast agents that are used for magnetic resonance imaging (MRI) studies have been studied and subsequently attributed to the development of NSF. ,
An Austrian study correlated NSF with gadolinium exposure, renal insufficiency and concurrent acidosis,  whereas a Danish study calculated a strong link between gadolinium exposure and subsequent onset of NSF in renal insufficiency patients with or without acidosis ( n = 13 patients with NSF, odds ratio (OR) of 32.5 for gadolinium exposure).  Alarmed by these results, the US Food and Drug Administration (USFDA) recently warned against the use of gadolinium-containing contrast material in patients with advanced renal disease (glomerular filtration role (GFR)≤15cc/minute), particularly with coexistent acidosis. 
| Gadolinium: Pathogenic Trigger or Innocent Bystander?|| |
Following the issuance of a public health advisory by the USFDA in June 2006 regarding the use of gadolinium-containing contrast agents in patients with renal failure,  the FDA Medwatch system received as many as 90 reports of NFD possibly related to them, as on December 25, 2006. 
From the time it was first suggested that gadolinium might have a role to play in the causation of NSF, attempts have been made to elucidate an exact mechanism for the dermal and systemic manifestations of this gadolinium toxicity,  but only with limited success so far. Gadolinium is a paramagnetic heavy metal, which is bound to a chelating agent ( e.g., iron) within the contrast agent. One hypothesis is that gadolinium might dissociate from the chelate and result in a fibrotic reaction. 
The reports relating gadolinium to the development of NSF basically suffer from a few common limitations. First, NFD/NSF is a rare condition. Therefore, the small sample size and the limited nature of positive and negative controls of the case-control studies limit the power to demonstrate statistically significant associations for variables other than gadolinium-containing contrast agents. For example, High and colleagues, who have published the results of a provocative study  demonstrating the presence of gadolinium within the tissue of NSF patients, could have made the study much more interesting if they could analyze the tissues taken from individuals without renal disease, who had received gadolinium-containing contrast material. Furthermore, the high concentration of iron in NSF tissue that they reported may point to yet another potential culprit: the chelators (carrier molecules) used to deliver gadolinium rather than the metal itself. Other investigators have also identified gadolinium in areas of calcium phosphate deposition within the blood vessels of patients with NSF, adding another potential piece to this jigsaw puzzle. 
Second, even the CDC report  that presented data collated from the largest cluster of NFD cases identified to date contains one serious flaw: it used the date of disease diagnosis rather than the date of disease onset, ostensibly because the actual date of disease onset is unknown. To identify the exposures that preceded the actual date of disease onset, exposures as early as one year before the date of diagnosis were included. However, this might have resulted in the inclusion of gadolinium exposures that were not related to the development of NFD, in other words, false-positives.
Finally, the gadolinium hypothesis fails to account for cases with acute renal disease never having dialysis.
Thus, the true nature of the association between gadolinium and NSF is yet to be completely clarified. Till the time this is done, it is prudent to follow the USFDA advice by avoiding the use of gadolinium-containing contrast material in patients with renal insufficiency.
| Pathogenesis|| |
The current model for evolution of NFD/NSF supports a role for aberrantly functioning circulating fibrocytes (fibroblast precursors) together with the elaboration of fibrogenic factors. The presence of fibroblast-like cells positive for CD34 and CD45RO and scattered CD68-positive cells in the affected tissues provide proof for this hypothesis. Immunohistochemical staining also showed the expression of factor XIIIA, CD31, smooth muscle actin as well as colloidal iron.  The similarity of these markers with that of scleromyxedema provokes the search for similar fibrogenic factors in the evolution of both these conditions. Dual immunohistochemical staining for CD34 and procollagen in the spindle cells of NFD suggest that the dermal cells of NFD may represent circulating fibrocytes recruited to the dermis, as originally demonstrated by Cowper.  The role of the fibrogenic factors was underlined by the increased expression of transforming growth factor beta1 (TGF/β1) in affected skin and muscles, together with large numbers of CD68 +/ factor XIIIA+ dendritic cells in those tissues. Decreasing plasma levels of TGFβ1 after plasmapheresis appear to correlate with the amelioration of the clinical condition. Accumulation of dermal mucin - an amorphous gelatinous substance composed primarily of hyaluronan (HA) and sulfated glycosaminoglycans (GAGs) - is one of the histopathological hallmarks of NSF. It has been found that fibroblasts derived from active lesions of NFD synthesize elevated levels of GAGs, particularly HA, in comparison with normal controls, while the serum from the patients with NFD stimulates GAG synthesis, including HA synthesis, both by control and patient fibroblasts.  This implies that fibroblasts from patients with active NFD are either activated to synthesize elevated levels of HA or they contain another cell type, possibly derived from circulating fibrocytes. In addition, this also supports the presence of a serum-derived factor that stimulates production of sulfated GAGs and HA by fibroblasts. This putative factor could be TGFβ1. Further, basic scientific and translational studies are necessary to understand the disease mechanism.
| Clinical Features|| |
As noted previously, NSF/NFD occurs mostly in patients with chronic renal failure (CRF) and very rarely in those with acute renal failure (ARF) too. A majority of the cases have been described in renal dialysis or renal transplant patients. Only five cases have been reported in which patients had ARF and never required dialysis.  Three cases have been reported to occur following liver transplantation for hepatitis B and C virus-induced cirrhosis. , All these patients had encephalopathy, refractory ascites and malnutrition prior to transplantation. All these patients, however, had renal dysfunction and required hemodialysis before and after transplantation. In a large report, chronic ambulatory peritoneal dialysis (CAPD) patients have been claimed to have higher estimated NFD attack rates than chronic hemodialysis (HD) patients.  The reason advanced is that peritoneal dialysis might achieve less effective gadolinium-contrast clearance than hemodialysis. Occasionally, NFD has been reported to occur in the background of systemic lupus erythematosus (SLE) nephropathy and antiphospholipid syndrome related thrombotic glomerulopathy, at times even without receiving dialysis. 
NFD is usually a diagnosis of exclusion;  however, the condition does have distinct clinical and histopathologic findings.
| Cutaneous Findings|| |
Indurated plaques with a 'woody' feel is the most characteristic finding of this condition. Sometimes brownish hyperpigmentation develops on the plaques. Extremities as well as the trunk may be affected. In general, the lower extremities are involved more frequently and to a greater extent. The spectrum of manifestations is diverse. Other than plaques and induration, erythema, edema, peau d'orange appearance, nodules and papules are also frequently observed.  In advanced cases, there is significant thickening and hardening of skin with the development of acral swelling and flexion contractures. Face is characteristically spared. In a series of nine patients, as many as eight had characteristic yellow scleral plaques.  At times, the patients exhibit a distinctive physical appearance, with elbows and knees angled inward with a concomitant complaint of a loss of a range of motions. In early cases, there might not be any finding other than circumscribed xerotic appearance of skin. Cases showing rippled hyperpigmentation and those resembling papular mucinosis have been reported, particularly from India. ,
| Systemic Involvement|| |
Recent reports have favoured the view that NSF is actually a systemic fibrosing disease with preferential cutaneous manifestations.  However, most of the multiorgan fibrosis involving, inter alia, striated muscles, lungs, kidneys, testes, dura mater, microvasculature, subcutaneous tissue, diaphragm, pericardium, great vessels of the heart and myocardium are autopsy findings. This implies that systemic involvement is mostly asymptomatic. However, there is a frequent elevation of the markers of systemic inflammation, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Systemic involvement might also be a cause of mortality in NSF/NFD, e.g., by causing respiratory failure secondary to diffuse diaphragmatic infiltration. In one series, five out of six patients with lung involvement had a reduced diffusion capacity for carbon monoxide on pulmonary function testing.  There is a report of a patient developing progressively painful hyperkeratotic spicules on both the thighs and bone metaplasia was later confirmed. 
Laboratory investigations might reveal considerably increased circulating immune complexes, antinuclear antibodies in low titer and anti-double-stranded DNA antibodies even in the absence of SLE or other connective tissue disorders.  In patients having severe limitation of movements, computerized tomography showed fibrosis of the fascia and muscles, and electromyography showed mild to severe myopathic changes, thereby demonstrating that the limitation was caused predominantly by skin tightness and induration and by joint contractures rather than muscle weakness.  In recent case reports, circulating antiphospholipid antibodies have been proposed as markers of NSF. Systemic manifestations are generally understood to confer poor prognosis with a high mortality rate. 
| Histopathological Findings|| |
A spindle-cell proliferation infiltrating through the dermis and subcutis with varying degrees of mucin deposition in the dermis is the histopathological hallmark. There is dermal and subdermal thickening (as opposed to hyperkeratosis) and fibrosis with excessive dermal fibroblasts and abundant collagen bundles separated by prominent clefts. There is also an increase in elastic fibers. There may be lack of an inflammatory infiltrate. The reticular dermal fibroblastic spindle cells (postulated to be derived from the circulating fibrocytes) are of varied morphology: they may be plump large bipolar epithelioid, dendritic or stellate.  The absence of plasma cells on histology helps to differentiate NSF from scleromyxedema. The end stage of NSF may be exceptionally characterized by the presence of bone metaplasia. Sparse, small multinucleated histiocytes were the noticeable findings in our Indian series.  Focally, there may be zones of calcium deposition in dermal collagen without vessel calcification. Histopathology of affected muscles reveals a spectrum of mild to severe fibrosis, degenerating fibers and chronic inflammatory cells.
Dual immunolabelling for CD34 and procollagen in the spindle cells of NSF, although not yet fully characterized as a diagnostic test, may provide a sensitive and specific method of diagnosis.
A tip for the dermatologists and dermatopathologists: punch biopsy is often inadequate to diagnose NSF and should be discarded in favour of full-thickness wedge biopsy including subcutaneous tissue.
| Differential Diagnosis|| |
NSF has to be chiefly differentiated from the primary cutaneous mucinoses (lichen myxedematosus, scleromyxedema and reticular erythematous mucinosis) and the secondary mucinoses (lupus erythematosus, dermatomyositis, scleroderma and granuloma annulare). While differentiation from some of these disorders ( e.g., lichen myxedematosus, granuloma annulare) is easy on the grounds of cutaneous morphology alone, differentiation from some of the others is not so very simple. The closest similarity is with scleromyxedema, where sometimes even microscopic difference is also hard to obtain. Association with renal disorder, characteristic lesional distribution (acral and truncal preferences, facial sparing), raised ESR and CRP, lack of plasma cells on histology, lack of paraproteinemia and characteristic immunohistochemistry with CD34 and procollagen tip the diagnosis in favour of NSF. The systemic nature of NSF may confound the diagnosis with systemic sclerosis; however, the serologic findings and histopathology provide the means of differentiation. Systemic lupus erythematosus is harder to distinguish as NFD may be associated with the former and antinuclear antibodies in low titer may be present in NSF per se . In this case, histopathology should provide the clue. Electromyogram may be necessary to differentiate from myopathies in some advanced cases. Eosinophilic fasciitis may also be a mimicker that is to be distinguished by deep biopsy. Pulmonary function testing in NSF may give a picture of restrictive lung disease in those with pulmonary involvement.
| Treatment|| |
No specific treatment modality has been consistently effective for this condition; however, there have been reports of improvement that occurred either spontaneously with renal recovery or after renal transplantation. There are contradictory reports of unchanged skin lesions despite the normalization of renal function.
Extracorporeal photopheresis (ECP), photodynamic therapy (PDT), high-dose intravenous immunoglobulin and other immunosuppressive therapies have been attempted with varying success. Among the immunosuppressives, the use of methylprednisolone and thalidomide are noteworthy.  There is a report on the marked improvement (softening of skin lesions, increase in joint motility) with four cycles of ECP and complete regression of the skin lesions with 16 cycles despite the constantly elevated renal values.  Plasmapheresis was moderately successful in treating three cases of NFD that developed following liver transplantation. 
In the absence of a standard, unequivocally effective treatment, prevention is of paramount importance. In this regard, avoidance of high-dose erythropoietin therapy and adherence to the USFDA guidelines on the use of gadolinium-containing contrast media in renal compromise are mandatory.
| Summary|| |
NSF, previously known as nephrogenic fibrosing dermopathy, was first described in 1997 in the United States as a scleromyxedematous fibrosing condition of the skin occurring in the background of renal insufficiency. The first and, as of now, the largest series in the non-Western population was reported from Kolkata in 2005. Gradually, it has come to be recognized as a form of systemic fibrosis with preferential cutaneous involvement. The etiology is unclear with high-dose erythropoietin therapy and use of gadolinium-containing contrast media in the context of advanced renal disease being considered nowadays as potential risk factors. The clinical characteristics are that of cutaneous fibrosis with a typical acral distribution, sparing the face. Histopathology shows dermal thickening and fibrosis with haphazard distribution of collagen and mucin and the presence of spindle-shaped fibroblasts and fibroblast-like cells. The diagnosis requires a high degree of clinical suspicion and a great degree of coordination between nephrologists, rheumatologists, dermatologists and dermatopathologists. There is no standard treatment, with a variety of physical therapies and immunosuppressives offering variable responses. Prevention by avoiding the currently acknowledged risk factors is thus of utmost importance.
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