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Pigmentierte villonoduläre Synovialitis

Eine seltene Differenzialdiagnose der synovialen Gelenkschwellung

Pigmented villonodular synovitis

A rare differential diagnosis of synovial joint swelling

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Zusammenfassung

Hintergrund

Die pigmentierte villonoduläre Synovialitis (PVNS) ist eine seltene proliferative Erkrankung der Binnenhaut großer und kleiner Gelenke. Um ein optimales Behandlungsergebnis zu erzielen, sind eine spezifische Diagnostik und ein gezieltes therapeutisches Vorgehen notwendig.

Fragestellung

Die vorliegende Arbeit soll eine Übersicht über Epidemiologie, Ätiopathogenese und Diagnostik der PVNS liefern sowie aktuelle Therapie- und Behandlungsempfehlungen aufzeigen.

Material und Methoden

Es erfolgte eine systematische Literaturrecherche über die Datenbank des National Center for Biotechnological Information (http://www.ncbi.nlm.nih.gov/pubmed). Gegenstand der Suche waren unabhängig von der Evidenz der jeweiligen Arbeiten randomisierte klinische und experimentelle Studien, systematische und nichtsystematische Übersichtsarbeiten, Expertenmeinungen sowie Fallberichte bezüglich der PVNS.

Ergebnisse

Die Differenzialdiagnose der PVNS sollte v. a. bei rezidivierenden blutigen Gelenkergüssen bedacht werden. Die Ursache der Erkrankung ist bis heute nicht genau geklärt. Die endgültige Diagnose kann letztlich nur histologisch gestellt werden. Um repräsentatives histologisches Gewebe für die Diagnose zu gewinnen, sollte im Vorfeld der Probeentnahmen ein MRT mit den entsprechenden Häm-Sequenzen durchgeführt werden. Die Erkrankung neigt abhängig von den verschiedenen Formen zu einer hohen Rezidivrate. Therapeutisch sollte deshalb eine vollständige Synovektomie erfolgen.

Diskussion

Als operatives Vorgehen werden arthroskopische und offene Verfahren beschrieben, die z. T. kontrovers hinsichtlich Komplikations- und Rezidivrate diskutiert werden. Adjuvante interventionelle Therapieformen wie die Radiosynoviorthese werden zur Senkung der Rezidivquote empfohlen.

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.

Material and 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.

Conclusion

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.

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Authors and Affiliations

  1. Abteilung für Orthopädische Rheumatologie in der Orthopädischen Universitätsklinik, Friedrich-Alexander-Universität Erlangen-Nürnberg, Rathsberger Str. 57, 91054, Erlangen, Deutschland

    K. A. Jendrissek, T. Hotfiel & B. Swoboda

  2. Pathologisches Institut, Universitätsklinikum Erlangen, Erlangen, Deutschland

    S. Söder

  3. Institut für Radiologie, Universitätsklinikum Erlangen, Erlangen, Deutschland

    R. Janka

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Correspondence to K. A. Jendrissek.

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K. A. Jendrissek, T. Hotfiel, B. Swoboda, S. Söder und R. Janka geben an, dass kein Interessenkonflikt besteht.

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Jendrissek, K.A., Hotfiel, T., Swoboda, B. et al. Pigmentierte villonoduläre Synovialitis. Z Rheumatol 75, 157–165 (2016). https://doi.org/10.1007/s00393-015-0028-4

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