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Nephrogenic fibrosing dermopathy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor-in-Chief: Brian Blank

Synonyms and keywords: nephrogenic systemic fibrosis

Overview

Overview



Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Overview

Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis is a rare and serious syndrome that involves fibrosis of skin, joints, eyes, and internal organs. Its cause is not fully understood, but it seems to be associated with exposure to gadolinium (which is frequently used as a contrast substance for MRIs) in patients with severe kidney failure. It does not have a genetic basis.

References

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Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Historical Perspective

The first cases of NFD have been identified in 1997 as a fibrotic disorder of the skin in patients with renal failure .[1]As an independent disease entity, NFD was first described in 2000.[2] Since then, systemic involvement has been described in some patients with NFD, resulting in use of the term nephrogenic systemic fibrosis (NSF); NFD and NSF have been used to describe the same condition [3]. No clear etiology has been established for NFD, and little is known about its pathogenesis or natural history. This report describes the largest geographic cluster of NFD that has been identified and provides evidence that exposure to a gadolinium-containing contrast agent is a risk factor for the development of the disease. While skin involvement is on the foreground, the process may involve any organ and resembles diffuse scleroderma or systemic sclerosis. [4]

In 2006, the link between NFD and gadolinium-containing contrast agents was made.[5][6][7] As a result, gadolinium-containing contrast is now considered contraindicated in patients with an estimated glomerular filtration rate (a measure of renal function) under 60 and especially under 30.[8]

References

  1. ↑ Cowper SE (2003). “Nephrogenic fibrosing dermopathy: the first 6 years”. Current Opinion in Rheumatology. 15: 785–790.
  2. ↑ Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE (2000). “Scleromyxoedema-like cutaneous diseases in renal-dialysis patients”. Lancet. 356 (9234): 1000–1. PMID 11041404.
  3. ↑ Ting WW, Stone MS, Madison KC, Kurtz K. Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol 2003;139: 903-6.
  4. ↑ Mendoza FA, Artlett CM, Sandorfi N, Latinis K, Piera-Velazquez S, Jimenez SA (2006). “Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature”. Semin. Arthritis Rheum. 35 (4): 238–49. doi:10.1016/j.semarthrit.2005.08.002. PMID 16461069.
  5. ↑ Grobner T (2006). “Gadolinium-a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis?”. Nephrol. Dial. Transplant. 21 (4): 1104–8. doi:10.1093/ndt/gfk062. PMID 16431890.
  6. ↑ Marckmann P, Skov L, Rossen K; et al. (2006). “Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging”. J. Am. Soc. Nephrol. 17 (9): 2359–62. doi:10.1681/ASN.2006060601. PMID 16885403.
  7. ↑ “Nephrogenic fibrosing dermopathy associated with exposure to gadolinium-containing contrast agents-St. Louis, Missouri, 2002-2006”. MMWR Morb. Mortal. Wkly. Rep. 56 (7): 137–41. 2007. PMID 17318112.
  8. ↑ Kanal E, Barkovich AJ, Bell C; et al. (2007). “ACR guidance document for safe MR practices: 2007”. AJR. American journal of roentgenology. 188 (6): 1447–74. doi:10.2214/AJR.06.1616. PMID 17515363.

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Classification

Classification

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References

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

Intravenously administered contrast agents are used routinely for MRI studies; the contrast agents contain gadolinium (a paramagnetic heavy metal), which is bound to a chelating agent. The mechanism for possible gadolinium-associated NFD is unknown; however, one hypothesis is that the gadolinium ions might dissociate from the chelate and result in a fibrotic reaction [1]. Five gadolinium-based contrast agents are available in the United States; the first was approved for use in 1988 [2]. Adverse events associated with these agents typically are minor (e.g., nausea); severe effects such as allergic reactions or tissue necrosis as a result of extravasation are rare. In addition, gadolinium-containing contrast agents are believed to be less nephrotoxic than iodinated contrast agents used for computed tomography (CT) imaging [3]. Excretion of gadolinium-containing contrast agents primarily occurs renally; the amount of contrast eliminated from the body after dialysis has not been well-evaluated. Two studies suggest that 65%-78% of gadolinium-containing contrast might be cleared after one hemodialysis session and 98% after three sessions [4][5]. Peritoneal dialysis might achieve less effective gadolinium-contrast clearance than hemodialysis. In one study, 69% of total gadolinium-containing contrast was excreted after 22 days in patients undergoing continuous ambulatory peritoneal dialysis [6]. Delayed clearance might prolong the duration of gadolinium-containing contrast exposure among patients undergoing peritoneal dialysis. However, patients undergoing peritoneal dialysis have not been previously reported to be at higher risk for NFD than patients undergoing hemodialysis. The chronic peritoneal dialysis outpatients in this investigation had higher estimated NFD attack rates than chronic hemodialysis outpatients. No controls who had gadolinium-containing contrast exposure underwent primarily peritoneal dialysis.

References

  1. ↑ Marckmann P, Skov L, Rossen K, et al. Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging. J Am Soc Nephrol 2006;17:2359-62.
  2. ↑ Food and Drug Administration. Public health advisory: gadolinium-containing contrast agents for magnetic resonance imaging (MRI): Omniscan, OptiMARK, Magnevist, ProHance, and MultiHance. Available at http://www.fda.gov/cder/drug/advisory/gadolinium_agents.htm.
  3. ↑ Runge VM. Safety of approved MR contrast media for intravenous injection. J Magn Reson Imaging 2000;12:205-13
  4. ↑ Joffe P, Thomsen HS, Meusel M. Pharmacokinetics of gadodiamide injection in patients with severe renal insufficiency and patients undergoing hemodialysis or continuous ambulatory peritoneal dialysis. Acad Radiol 1998;5:491-502
  5. ↑ Okada S, Katagiri K, Kumazaki T, Yokoyama H. Safety of gadolinium contrast agent in hemodialysis patients. Acta Radiologica 2001;42: 339-41.
  6. ↑ Joffe P, Thomsen HS, Meusel M. Pharmacokinetics of gadodiamide injection in patients with severe renal insufficiency and patients undergoing hemodialysis or continuous ambulatory peritoneal dialysis. Acad Radiol 1998;5:491-502

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Causes

Causes

Overview

Life Threatening Causes

Common Causes

Causes by Organ System

Cardiovascular No underlying causes
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drug Side Effect Gadofosveset, gadopentetate, gadoterate, gadoxetate, Gadoteridol
Ear Nose Throat No underlying causes
Endocrine No underlying causes
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic No underlying causes
Hematologic No underlying causes
Iatrogenic No underlying causes
Infectious Disease No underlying causes
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic No underlying causes
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal/Electrolyte No underlying causes
Rheumatology/Immunology/Allergy No underlying causes
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous No underlying causes

Causes in Alphabetical Order

References

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Differentiating Nephrogenic fibrosing dermopathy from other Diseases

Differentiating Nephrogenic fibrosing dermopathy from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]

Overview

Nephrogenic fibrosing dermopathy must be differentiated from other diseases that cause skin thickening and edema such as scleredema, scleromyxedema, eosinophilic fasciitis, scleroderma, drug induced scleroderma, scleroderma overlap syndromes, diabetic cheiroarthropathy, myxedema and Chronic graft-versus-host disease.

Differentiating Nephrogenic fibrosing dermopathy from other Diseases

Nephrogenic fibrosing dermopathy must be differentiated from other diseases that cause skin thickening and edema such as scleredema, scleromyxedema, eosinophilic fasciitis, scleroderma, drug induced scleroderma, scleroderma overlap syndromes, diabetic cheiroarthropathy, myxedema and Chronic graft-versus-host disease.

Differentiating Nephrogenic fibrosing dermopathy from other Diseases

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Skin thickening Raynaud phenomenon Heart burn Edema (swelling) Sclerodactyly Telangiectasia Impaired mobility Autoantibodies Blood indices
Renal diseases Nephrogenic fibrosing dermopathy (Nephrogenic systemic fibrosis)[1] +

(induration)

– – + + – –
  • N/A
  • N/A
Scleroderma[2][3] Limited cutaneous systemic sclerosis (CREST syndrome) +

(induration)

+ + +/- + + +/-
  • Nailfold microvascular changes on nailfold video capillaroscopy (NVC)[4]
  • Chest HRCT scan showing evidence of pulmonary fibrosis
  • Nailfold microvascular changes
  • Dilated capillary loops
  • Microhemmorhages
Diffuse cutaneous systemic sclerosis +

(induration)

+ + +/- + + +/-
  • Nailfold microvascular changes on nailfold video capillaroscopy (NVC)[6]
  • Chest HRCT scan showing evidence of pulmonary fibrosis
  • Nailfold microvascular changes
  • Dilated capillary loops
  • Microhemmorhages
Systemic diseases Scleredema

(Buschke’s disease)[7]

+ – – + – – +
  • N/A
  • N/A
Scleromyxedema

(lichen myxedematosus)[8][9]

+

(waxy yellow-red papules)

+/- +/- + – – –
  • N/A
  • N/A
Eosinophilic fasciitis[10][11] +

(orange peel-peau d’orange appearance)

– – + – – –
  • N/A
  • Normal appearance on nailfold video capillaroscopy (NVC)
  • Fasciitis present on the trunk sparing extremities
  • Visible collapse of superficial veins when the limb is elevated
  • Hands and feet are not involved
Chronic graft-versus-host disease[12][13] +

(induration)

– – – – – +
  • N/A
Drug-induced scleroderma[14][15] + + +/- +/- + +/- –
  • N/A
  • N/A
  • N/A
Scleroderma overlap syndromes[16][17][18][19] Scleroderma-systemic lupus erythematosus overlap +

(rash)

+ + +/- + + +/-
  • N/A
Scleroderma-polymyositis overlap +

(rash)

+ + +/- + + +/-
  • Anti-Ro52 antibody
  • Antinuclear antibody (ANA)
  • Anti-Jo-1 antibody
  • Anti-SRP antibody
  • Anti-Mi-2 antibody
  • Anti-centromere antibody (ACA)
  • Anti-topoisomerase-I (Scl-70) antibody
  • Anti-RNA polymerase III antibody
  • N/A
Scleroderma-rheumatoid arthritis overlap +

(rash)

+ + +/- + + +/-
  • N/A
  • Anti-Ro52 antibody
  • Anti-CCP antibody
Endocrine disorders Diabetic cheiroarthropathy[21][22] +

(waxy skin)

– – – + – +
  • N/A
  • N/A
  • N/A
Myxedema[23] +

(coarse skin)

– – + – – –
  • Anti-TPO antibody
  • Anti-Tg antibody
  • N/A
 Serum TSH

References

  1. ↑ Galan A, Cowper SE, Bucala R (November 2006). “Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy)”. Curr Opin Rheumatol. 18 (6): 614–7. doi:10.1097/01.bor.0000245725.94887.8d. PMID 17053507.
  2. ↑ LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F (February 1988). “Scleroderma (systemic sclerosis): classification, subsets and pathogenesis”. J. Rheumatol. 15 (2): 202–5. PMID 3361530.
  3. ↑ Black CM (August 1993). “Scleroderma–clinical aspects”. J. Intern. Med. 234 (2): 115–8. PMID 8340733.
  4. ↑ Cutolo M, Sulli A, Smith V (April 2013). “How to perform and interpret capillaroscopy”. Best Pract Res Clin Rheumatol. 27 (2): 237–48. doi:10.1016/j.berh.2013.03.001. PMID 23731933.
  5. ↑ 5.0 5.1 van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE (November 2013). “2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative”. Ann. Rheum. Dis. 72 (11): 1747–55. doi:10.1136/annrheumdis-2013-204424. PMID 24092682.
  6. ↑ Cutolo M, Sulli A, Smith V (April 2013). “How to perform and interpret capillaroscopy”. Best Pract Res Clin Rheumatol. 27 (2): 237–48. doi:10.1016/j.berh.2013.03.001. PMID 23731933.
  7. ↑ Rongioletti F, Kaiser F, Cinotti E, Metze D, Battistella M, Calzavara-Pinton PG, Damevska K, Girolomoni G, André J, Perrot JL, Kempf W, Cavelier-Balloy B (December 2015). “Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients”. J Eur Acad Dermatol Venereol. 29 (12): 2399–404. doi:10.1111/jdv.13272. PMID 26304054.
  8. ↑ Rongioletti F, Rebora A (February 2001). “Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema”. J. Am. Acad. Dermatol. 44 (2): 273–81. doi:10.1067/mjd.2001.111630. PMID 11174386.
  9. ↑ Gabriel SE, Perry HO, Oleson GB, Bowles CA (January 1988). “Scleromyxedema: a scleroderma-like disorder with systemic manifestations”. Medicine (Baltimore). 67 (1): 58–65. PMID 3336281.
  10. ↑ Herson S, Brechignac S, Piette JC, Mouthon JM, Coutellier A, Bletry O, Godeau P (June 1990). “Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma”. Am. J. Med. 88 (6): 598–600. PMID 2346160.
  11. ↑ Falanga V, Medsger TA (October 1987). “Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis”. J. Am. Acad. Dermatol. 17 (4): 648–56. PMID 3668010.
  12. ↑ Schaffer JV, McNiff JM, Seropian S, Cooper DL, Bolognia JL (October 2005). “Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum”. J. Am. Acad. Dermatol. 53 (4): 591–601. doi:10.1016/j.jaad.2005.06.015. PMID 16198778.
  13. ↑ Martires KJ, Baird K, Steinberg SM, Grkovic L, Joe GO, Williams KM, Mitchell SA, Datiles M, Hakim FT, Pavletic SZ, Cowen EW (October 2011). “Sclerotic-type chronic GVHD of the skin: clinical risk factors, laboratory markers, and burden of disease”. Blood. 118 (15): 4250–7. doi:10.1182/blood-2011-04-350249. PMC 3204741. PMID 21791415.
  14. ↑ Finch WR, Rodnan GP, Buckingham RB, Prince RK, Winkelstein A (1980). “Bleomycin-induced scleroderma”. J. Rheumatol. 7 (5): 651–9. PMID 6160247.
  15. ↑ Passiu G, Cauli A, Atzeni F, Aledda M, Dessole G, Sanna G, Nurchis P, Vacca A, Garau P, Laudadio M, Mathieu A (1999). “Bleomycin-induced scleroderma: report of a case with a chronic course rather than the typical acute/subacute self-limiting form”. Clin. Rheumatol. 18 (5): 422–4. PMID 10524560.
  16. ↑ Satoh M, Chan EK, Sobel ES, Kimpel DL, Yamasaki Y, Narain S, Mansoor R, Reeves WH (September 2007). “Clinical implication of autoantibodies in patients with systemic rheumatic diseases”. Expert Rev Clin Immunol. 3 (5): 721–38. doi:10.1586/1744666X.3.5.721. PMID 20477023.
  17. ↑ Moinzadeh P, Aberer E, Ahmadi-Simab K, Blank N, Distler JH, Fierlbeck G, Genth E, Guenther C, Hein R, Henes J, Herich L, Herrgott I, Koetter I, Kreuter A, Krieg T, Kuhr K, Lorenz HM, Meier F, Melchers I, Mensing H, Mueller-Ladner U, Pfeiffer C, Riemekasten G, Sárdy M, Schmalzing M, Sunderkoetter C, Susok L, Tarner IH, Vaith P, Worm M, Wozel G, Zeidler G, Hunzelmann N (April 2015). “Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis”. Ann. Rheum. Dis. 74 (4): 730–7. doi:10.1136/annrheumdis-2013-204487. PMC 4392314. PMID 24389298.
  18. ↑ Foocharoen C, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R (September 2016). “Clinical characteristics of scleroderma overlap syndromes: comparisons with pure scleroderma”. Int J Rheum Dis. 19 (9): 913–23. doi:10.1111/1756-185X.12884. PMID 27126733.
  19. ↑ Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM, Denton CP (November 2011). “Clinical and serological hallmarks of systemic sclerosis overlap syndromes”. J. Rheumatol. 38 (11): 2406–9. doi:10.3899/jrheum.101248. PMID 21844148.
  20. ↑ Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JM, Hobbs K, Huizinga TW, Kavanaugh A, Kay J, Kvien TK, Laing T, Mease P, Ménard HA, Moreland LW, Naden RL, Pincus T, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G (September 2010). “2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative”. Arthritis Rheum. 62 (9): 2569–81. doi:10.1002/art.27584. PMID 20872595.
  21. ↑ Seibold JR (November 1982). “Digital sclerosis in children with insulin-dependent diabetes mellitus”. Arthritis Rheum. 25 (11): 1357–61. PMID 6753855.
  22. ↑ Jelinek JE (April 1993). “The skin in diabetes”. Diabet. Med. 10 (3): 201–13. PMID 8485952.
  23. ↑ Heymann WR (June 1992). “Cutaneous manifestations of thyroid disease”. J. Am. Acad. Dermatol. 26 (6): 885–902. PMID 1607406.

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Epidemiology and Demographics

Epidemiology and Demographics

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References

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Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Risk Factors

Risk Factors

Although risk factors for NFD have not been studied extensively, possible correlations with severity of renal failure, thrombotic episodes, edema, and vascular procedures have been reported [1][2][3]. Recently, medication exposures such as erythropoietin and gadolinium-containing contrast agents have been identified as potential risk factors for NFD [4] [5] In May 2006, the Danish Medicines Agency reported 25 cases of NFD diagnosed in Europe among patients with recent exposure to gadolinium-containing contrast. In response, the Food and Drug Administration (FDA) issued a public health advisory in June 2006 regarding the use of these contrast agents ([gadopentetate dimeglumine (Magnevist), gadobenate dimeglumine (MultiHance), gadodiamide (Omniscan), gadoversetamide (OptiMARK), gadoteridol (ProHance)] in patients with moderate to end-stage renal disease [6]. As of December 25, 2006, the FDA MedWatch system had received 90 reports of NFD possibly related to gadolinium-containing contrast agents. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease.

References

  1. ↑ Cowper SE. Nephrogenic fibrosing dermopathy: the first 6 years. Curr Opin Rheumatol 2003;15:785-90.
  2. ↑ Ting WW, Stone MS, Madison KC, Kurtz K. Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol 2003;139: 903-6.
  3. ↑ Jan F, Segal JM, Dyer J, LeBoit P, Siegfried E, Frieden IJ. Nephrogenic fibrosing dermopathy: two pediatric cases. J Pediatr 2003;143: 678-81
  4. ↑ Marckmann P, Skov L, Rossen K, et al. Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging. J Am Soc Nephrol 2006;17:2359-62.
  5. ↑ Swaminathan S, Ahmed I, McCarthy JT, et al. Nephrogenic fibrosing dermopathy and high-dose erythropoietin therapy. Ann Intern Med 2006;145:234-5.
  6. ↑ Food and Drug Administration. Public health advisory: gadolinium-containing contrast agents for magnetic resonance imaging (MRI): Omniscan, OptiMARK, Magnevist, ProHance, and MultiHance. Available at http://www.fda.gov/cder/drug/advisory/gadolinium_agents.htm.

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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

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References

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | X-Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

Source

Source



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