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Nephrogenic systemic fibrosisL90.5
Synonym(s)
Fibrosis nephrogenic systemic; Nephrogenic fibrosing dermatopathy; Nephrogenic fibrosing dermopathy; Nephrogenic systemic fibrosis; NSF
HistoryThis section has been translated automatically.
Cowper, 2000
DefinitionThis 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/EpidemiologyThis 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).
EtiopathogenesisThis 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.
ManifestationThis 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.
LocalizationThis 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 featuresThis section has been translated automatically.
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.
HistologyThis 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 diagnosisThis section has been translated automatically.
Differentiation from scleromyxedema is necessary.
Other differential diagnoses: Toxic-oil syndrome; Eosinophilic-myalgie syndrome; Eosinophilic fasciitis.
| 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 |
TherapyThis 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 therapyThis 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/forecastThis 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.
LiteratureThis section has been translated automatically.
- Baron PW et al (2003) Nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis. Am J Dermatopathol 25: 204-209
- Cowper SE, Bucala R (2003) Nephrogenic fibrosing dermopathy: suspect identified, motive unclear. Am J Dermatopathol 25: 358
- Galan A et al (2006) Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy). Curr Opin Rheumatol 18: 614-617
- Haemel AK et al (2011) Update on nephrogenic systemic fibrosis: are we making progress? Int J Dermatol 50:659-666
- High WA (2007) Gadolinium is detectable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol 56: 21-26
- Kobus S et al (2010) Nephrogenic systemic fibrosis-10 cases from the Bochum clinic. JDDG 11: 935
- LeBoit PE (2003) What nephrogenic fibrosing dermopathy might be. Arch Dermatol 139: 928-930
- Mackay-Wiggan JM et al (2003) Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease). J Am Acad Dermatol 48: 55-60.
- Ortega LM et al.(2009) Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis? What do we know and what do we have to learn?. Nefrologia 29:109-117.
- Richmond H et al. (2007) Nephrogenic systemic fibrosis: relationship to gadolinium and response to photopheresis. Arch Dermatol 143: 1025-1030
- Ting WW et al (2003) Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol 139: 903-906
- Swartz RD et al (2003) Nephrogenic fibrosing dermopathy: a novel cutaneous fibrosing disorder in patients with renal failure. Am J Med 114: 563-572