Autoantikörper bei juveniler idiopathischer inflammat orischer Myopathie

Zusammenfassung

Klassische Myositis-spezifische Autoantikörper (MSA) gegen t-RNA-Synthetasen, Mi-2 und SRP lassen sich nur selten bei juvenilen idiopathischen inflammatorischen Myopathien (JIIM) nachweisen und trugen bisher wenig zur Diagnosestellung bei. Die Entdeckung neuer MSA wie TIF-1 gamma, NXP-2, MDA5 und SAE ermöglicht eine bessere Diagnostik und serologische Klassifikation der JIIM sowie prognostische Aussagen. MSA können bei etwa 60 % der JIIM nachgewiesen werden, wobei Antikörper gegen TIF-1 gamma und NXP-2 am häufigsten sind. Diese sind vor allem mit juveniler Dermatomyositis assoziiert, wobei Patienten mit NXP-2-Antiköpern jünger sind, häufiger eine subkutane Kalzinose entwickeln und einen schwereren Krankheitsverlauf zeigen. Kinder mit MDA5-Antikörper zeigen vorwiegend Hautmanifestationen und eine milde Form der Myositis. SAE-Antikörper sind extrem selten. Die Konzentration von Antikörpern gegen Jo-1, SRP und MDA-5 korreliert mit der Krankheitsaktivität. Das erweiterte Spektrum nachweisbarer MSA könnte in Zukunft die Notwendigkeit invasiver diagnostischer Methoden wie der Muskelbiopsie reduzieren und neue Möglichkeiten zur Therapie -stratifizierung und -überwachung schaffen.

Summary

The classical myositis specific antibodies (MSA) against t-RNA-synthetases, Mi-2, and SRP are infrequently detected in juvenile idipopathic inflammatory myopathy (JIIM) and have contributed little to the diagnosis until now. The discovery of new MSA such as those against TIF-1 gamma, NXP-2, MDA5 and SAE makes possible a better diagnostic and serological classification of JIIM as well as a better prognostic estimation. MSA can be detected in about 60 % of JIIM cases, being antibodies against TIF-1 gamma and NXP-2 the most frequent ones. These are primarily associated with juvenile dermatomyositis. Patients with NXP-2 antibodies are younger, develop more frequently subcutaneous calcinosis and show a more severe disease. Children with MDA5 antibodies show predominantly skin manifestations and a milder form of myositis. SAE antibodies are extremely rare. The concentration of antibodies against Jo-1, SRP and MDA-5 correlates with disease activity. The extended spectrum of detectable MSA might reduce in the future the necessity of invasive diagnostic procedures such as the muscle biopsy and might open new possibilities for the therapeutic stratification and survey.

Schlüsselwörter

Juvenile Myositis - neue Autoantikörper

Keywords

Juvenile myositis - new antibodies

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