Nephrogenic systemic fibrosis L90.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 08.06.2021

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Fibrosis nephrogenic systemic; Nephrogenic fibrosing dermatopathy; Nephrogenic fibrosing dermopathy; Nephrogenic systemic fibrosis; NSF

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Cowper, 2000

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Rare, idiopathic, acquired, acutely onset, sclerosing disease of the skin, subcutaneous fatty tissue and musculature (more rarely is involvement of internal organs) which occurs almost exclusively in patients with advanced chronic renal insufficiency (GFR<30ml/min/1.73m2) after administration of gadolinium-containing contrast media (gadodiamide, gadopentetate dimeglumin).

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Rare; currently about 200 cases have been reported in world literature, although a significantly higher incidence can be assumed. The incidence is clearly increasing. No gender preference (in contrast to systemic scleroderma).

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Etiologically unexplained, probably multifactorial, sclerosing systemic disease, which frequently occurs in renal dysfunction. Connections with gadolinium (a metal chelate complex), which is used in nuclear spin examinations, are increasingly being described, so that the suspicion that causality exists in this case is substantiated. The chelation of gadolinium reduces its toxicity and enables renal excretion. The thermodynamic and kinetic stability of the chelate complex defines the release of free gadolinium ions. In advanced renal insufficiency, metabolic situations occur, which often favour acidosis and hyperphosphatemia. These in turn lead to "transmetallization", i.e. the release of gadolinium ions from the chelate complex, which then have a toxic effect in the tissue. A multifactorial genesis is also discussed, consisting of terminal renal failure, gadolinium, plasticizers in dialysis systems and chronic infections (sepsis, osteomyelitis, chronic hepatitis). In larger studies it was shown that patients with NSF had a significantly higher number of MRIs than a control group.

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Mostly occurring in patients with renal insufficiency. Frequently occurring in haemodialysis patients or after kidney transplantation, rarely also occurring after liver transplantation. No preference of one sex.

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The prediction site is the lower extremities. Also trunk and upper extremities. However, organ involvement (heart, lungs, liver) has also been reported in very extensive forms of the disease.

Clinical features
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Integument: Plaque-like and/or diffuse, board-hard (woody) thickening and induration of the skin, subcutis and underlying muscles with brownish, yellowish discoloration. Occasional papules and subcutaneous nodules. Skin lesions begin on the extremities, then spread to the trunk. Face usually excluded. Frequent flexion contractures in the course with need for a wheelchair. Burning pain, itching.

Extracutaneous manifestations: weakness, muscle pain. Extensive fibrosis and calcification of the diaphragm, the psoas muscle and the kidneys have been observed in isolated cases.

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Cell-rich, fibrotic restructuring of the dermis and subcutaneous fatty tissue. The subcutaneous tissue is crossed by plump CD34+ fibroblast-rich septa. The fine-tissue image (because it is much richer in cells) is only partially reminiscent of the image of morphea.

Differential diagnosis
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Differentiation from scleromyxedema is necessary.
Scleromyxoedema nephrogenic systemic fibrosis
systemic local
Face mostly affected Face free
Paraproteins in serum no paraproteins in serum
Plasma cells in the infiltrates No plasma cells
Mucin multiplied no mucin
Fibroblasts reduced Fibroblasts increased
Other differential diagnoses: Toxic-oil syndrome; Eosinophilic-myalgie syndrome; Eosinophilic fasciitis.

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Treatment of kidney disease. Individual spontaneous healings with improved kidney situation are reported. Individual experiments with high-dose steroids and plasmapheresis, chemotherapeutic agents (cyclophosphamide, melphalan), superficial X-ray radiation, thalidomide, tracrolimus, mycophenolate, oral retinoids have proven to be problematic and, above all, not very successful. The best results seem to be achieved by extracorporeal photopheresis (possibly in combination with acitretin). Overall, the therapeutic results are currently not satisfactory.

Radiation therapy
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The successful use of UVA1 in nephrogenic systemic fibrosis has been reported. Own experience with this therapy modality could not confirm the effectiveness of the method in this indication.

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The majority of patients show a progressive course of disease, which ultimately leads to immobility (wheelchair) due to contractures. Apparently, only an improvement of the kidney situation (usually only achievable by kidney transplantation) can lead to the suspension of the disease.

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Prevention is important! Strict indication for patients with terminal renal failure.

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  2. Cowper SE, Bucala R (2003) Nephrogenic fibrosing dermopathy: suspect identified, motive unclear. Am J Dermatopathol 25: 358
  3. Galan A et al (2006) Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy). Curr Opin Rheumatol 18: 614-617
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  11. Ting WW et al (2003) Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol 139: 903-906
  12. Swartz RD et al (2003) Nephrogenic fibrosing dermopathy: a novel cutaneous fibrosing disorder in patients with renal failure. Am J Med 114: 563-572


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Last updated on: 08.06.2021