title: [Pigmented villonodular synovitis. A rare differential diagnosis of synovial joint swelling].
abstract: BACKGROUND
  Pigmented villonodular synovitis (PVNS) describes a rare disease caused by an abnormal proliferation of the synovial membrane in large and small joints. In order to achieve an optimal result of treatment it is necessary to carry out specific diagnostics and a targeted therapy approach.
  
  
  OBJECTIVE
  This article gives a review of the epidemiology, etiopathogenesis and diagnostic management of PVNS as well as presenting the current therapy and treatment recommendations.
  
  
  METHODS
  A systematic search of the literature was performed in the databank of the National Center for Biotechnology Information ( http://www.ncbi.nlm.nih.gov/pubmed ). The search targeted randomized clinical and experimental studies, systematic and non-systematic review articles, expert opinions and case reports related to PVNS, independent of the level of evidence attained by each study.
  
  
  RESULTS
  The differential diagnosis of PVNS should be considered in cases of recurrent hemorrhagic joint effusions. The cause of the disease has not yet been exactly clarified. The final diagnosis can ultimately only be confirmed by histological investigations. In order to obtain representative histological tissue samples for the diagnosis, magnetic resonance imaging (MRI) with the appropriate heme sequences should be carried out prior to taking samples. The management of PVNS is often difficult due to the high risk of recurrence depending on the various forms. In view of the high rate of recurrence, therapy should include a complete synovectomy.
  
  
  CONCLUSIONS
  For the surgical approach arthroscopic and open procedures have been described, which are currently controversially discussed with respect to the complication and recurrence rates. Adjuvant interventional therapy forms, such as radiosynoviorthesis are recommended to reduce the recurrence rate.
authors.affiliation: Abteilung für Orthopädische Rheumatologie in der Orthopädischen Universitätsklinik, Friedrich-Alexander-Universität Erlangen-Nürnberg, Rathsberger Str. 57, 91054, Erlangen, Deutschland. Andreas.Jendrissek@gmx.de.
authors.forename: K A
authors.initials: KA
authors.lastname: Jendrissek
authors.affiliation: Abteilung für Orthopädische Rheumatologie in der Orthopädischen Universitätsklinik, Friedrich-Alexander-Universität Erlangen-Nürnberg, Rathsberger Str. 57, 91054, Erlangen, Deutschland.
authors.forename: T
authors.initials: T
authors.lastname: Hotfiel
authors.affiliation: Abteilung für Orthopädische Rheumatologie in der Orthopädischen Universitätsklinik, Friedrich-Alexander-Universität Erlangen-Nürnberg, Rathsberger Str. 57, 91054, Erlangen, Deutschland.
authors.forename: B
authors.initials: B
authors.lastname: Swoboda
authors.affiliation: Pathologisches Institut, Universitätsklinikum Erlangen, Erlangen, Deutschland.
authors.forename: S
authors.initials: S
authors.lastname: Söder
authors.affiliation: Institut für Radiologie, Universitätsklinikum Erlangen, Erlangen, Deutschland.
authors.forename: R
authors.initials: R
authors.lastname: Janka
mesh_terms: D001182:Arthroscopy / Q000379:methods*; D001706:Biopsy / Q000379:methods*; D003131:Combined Modality Therapy / Q000379:methods; D003937:Diagnosis, Differential; D004487:Edema; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging / Q000379:methods*; D015995:Prevalence; D011878:Radiotherapy / Q000379:methods; D035583:Rare Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D063189:Symptom Assessment / Q000379:methods*; D013586:Synovitis, Pigmented Villonodular / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*
chemical_list: D007166:Immunosuppressive Agents
keywords: Haemarthrosis; Joint destruction; Magnetic resonance imaging; Recurrence of synovialitis; Synovectomy