Zusammenfassung
Nichtbakterielle Knochenentzündungen (NBO) manifestieren sich als solitäres Krankheitsbild
oder können in etwa 20% der Fälle mit weiteren inflammatorischen Erkankungen vergesellschaftet
sein. Am häufigsten werden assoziiert entzündliche Hauterkrankungen oder chronisch-entzündliche
Darmerkrankungen (IBD) gefunden. Die Variabilität dieser Krankheitsbilder erscheint
groß, vergleichbar mit den idiopathischen Arthritiden. Darüber hinaus sind mittlerweile
syndromale Symptomenkomplexe mit sterilen Osteitiden bekannt geworden, die sich bereits
im Säuglingsalter manifestieren, und die genetisch identifiziert werden konnten. Diese
werden heutzutage den sogenannten Autoinflammations-Erkrankungen zugeordnet. Rekurrierende
Episoden – ausgelöst durch Entzündungsmechanismen der angeborenen Immunität, Fehlen
von spezifischen Autoantikörpern sowie das Fehlen von exogenen Auslösern – sind Kriterien
für diese Definition. NBO sind bislang größtenteils weder pathophysiologisch noch
genetisch definiert. Anhand der verfügbaren Literatur und eigener Befunde zeigt diese
Arbeit auf, dass sterile Osteitiden auch ohnen syndromalen Kontext wohl als auto-inflammatorisch
bezeichnet werden können. Verschiedene klinische Verlaufsformen werden anhand von
Entzündungs-Laborparametern und anhand der verfügbaren genetischen Befunde dargestellt.
Angesichts der Dynamik und der neuen Erkenntnisse, die heute die molekularbiologische
Forschung täglich vorwärts treiben, erhebt sich aber auch die Frage, ob Autoimmunität
und Autoinflammation tatsächlich zu trennen sind. Es bleibt spannend, welche der nichtbakteriellen
Osteitiden und welche der anderen rheumatischen Erkrankungen wo einzuordnen sein werden.
Es könnte sich zeigen, dass etliche bislang ätiologisch unklare Krankheitsbilder dem
rheumatisch-inflammatorischen Formenkreis zugerechnet werden müssen.
Abstract
Non-bacterial osteitis (NBO) can be observed as a single symptom of disease or may
be associated with other inflammatory conditions, mainly of the skin and/or bone.
The variability of clinical presentations seems comparable to rheumatic arthritis.
Meanwhile sterile osteitis was described as a feature of genetically defined inflammatory
syndromes with manifestations in very early childhood. These are defined as autoinflammatory
diseases. Recurrent episodes triggered by innate immune mechanisms, in the absence
of specific autoantibodies and without exogeneous triggers, are criteria for this
classification. Non-syndromal non-bacterial inflammatory bone lesions are neither
pathophysiologically nor genetically well defined. With the help of the current literature
on this unexplored field and on the basis of our own clinical and laboratory findings,
it can be shown that also NBO without syndromal features may be classified as auto-inflammatory
disorders. Different clinical presentations are highlighted in view of inflammation
parameters and genetic findings – as far as available. The current impetus and vast
new findings in this field pose again and again the question whether auto-immunity
and auto-inflammation need to be separated from each other. Whether or not NBO and/or
other rheumatic diseases will have to be (re)classified remains an exciting topic.
Therefore, in the near future, so far non-defined disorders may be diagnosed as rheumatic-inflammatory
conditions.
Schlüsselwörter
Osteitis - CRMO - SAPHO - Inflammation
Key words
osteitis - CRMO - SAPHO - inflammation
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Korrespondenzadresse
PD Dr. med. Dr. Annette Friederike Jansson
Oberärztin Rheumatologie
Dr. von Haunersches
Kinderspital
Lindwurmstraße 4
80337 München
Telefon: + 49/089/5160 7848
Fax: + 49/089/5160 3964
eMail: annette.jansson@med.uni-muenchen.de
