Nephrogenic systemic fibrosis L90.5

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

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

History
This section has been translated automatically.

Cowper, 2000

Definition
This section has been translated automatically.

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).

Occurrence/Epidemiology
This section has been translated automatically.

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).

Etiopathogenesis
This section has been translated automatically.

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.

Manifestation
This section has been translated automatically.

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.

Localization
This section has been translated automatically.

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
This section has been translated automatically.

  • Integument: Plaque-shaped and/or diffuse, board-hard (wooden) thickening and hardening of the skin, subcutis and underlying muscles with brownish, yellowish discoloration. Occasionally also papules and subcutaneous nodules. Beginning of the skin changes on the extremities, then spreading to the trunk. Face usually left out. Often in the course of bending contractures with wheelchair use. Burning pain, itching.
  • Extracutaneous manifestations: weakness, muscle pain. In individual cases extensive fibrosis and calcification of the diaphragm, the psoas muscle and the kidneys have been observed.

Histology
This section has been translated automatically.

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
This section has been translated automatically.

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.

Therapy
This section has been translated automatically.

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
This section has been translated automatically.

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.

Progression/forecast
This section has been translated automatically.

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.

Note(s)
This section has been translated automatically.

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

Literature
This section has been translated automatically.

  1. Baron PW et al (2003) Nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis. At J Dermatopathol 25: 204-209
  2. Cowper SE, Bucala R (2003) Nephrogenic fibrosing dermopathy: suspect identified, motive unclear. At J Dermatopathy 25: 358
  3. Galan A et al (2006) Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy). Curr Opin Rheumatol 18: 614-617
  4. Haemel AK et al (2011) Update on nephrogenic systemic fibrosis: are we making progress? Int J Dermatol 50:659-666
  5. High WA (2007) Gadolinium is detectable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol 56: 21-26
  6. Kobus S et al (2010) Nephrogenic systemic fibrosis-10 cases at the Bochum clinic. JDDG 11: 935
  7. LeBoit PE (2003) What nephrogenic fibrosing dermopathy might be. Arch Dermatol 139: 928-930
  8. Mackay-Wiggan JM et al (2003) Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease). J Am Acad Dermatol 48: 55-60
  9. Richmond H et al (2007) Nephrogenic systemic fibrosis: relationship to gadolinium and response to photopheresis. Arch Dermatol 143: 1025-1030
  10. Ting WW et al (2003) Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol 139: 903-906
  11. Swartz RD et al (2003) Nephrogenic fibrosing dermopathy: a novel cutaneous fibrosing disorder in patients with renal failure. On J Med 114: 563-572

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020