Myositisspezifische Antikörper bei juveniler Dermatomyositis

Myositis-specific antibodies associated with juvenile dermatomyositis

Zusammenfassung

Hintergrund

Die juvenile Dermatomyositis (jDM) ist eine seltene Autoimmunerkrankung, klinisch charakterisiert durch typische Hautveränderungen und Muskelschwäche. Bei einigen Patienten können myositisassoziierte (MAA) oder myositisspezifische Antikörper (MSA) nachgewiesen werden. Diese sind bedeutsam für Krankheitsspektrum und Prognose.

Methodik

Bei 12 aktuell betreuten jDM-Patienten des Rheumazentrums Sankt Augustin erfolgte eine Untersuchung auf MAA und MSA mittels eines Line-Immunoassays.

Ergebnisse

Bei 10 von 12 Patienten konnten insgesamt 15 Myositisantikörper nachgewiesen werden. Je 3 Patienten hatten Mi2-, SRP- oder NXP2-Antikörper, 2 hatten TIF-1γ-Antikörper und je 1 Patient Jo1- oder Mi2β-Antikörper. Zwei Patienten hatten zusätzlich PM-Scl-Antikörper. Bei den 10 Patienten mit nachgewiesenen Antikörpern zeigte sich eine gute Phänotyp-Serotyp-Korrelation mit Abweichung von den in der Literatur beschriebenen Phänotypen bei 3 Patienten.

Schlussfolgerung

Der häufige Nachweis von spezifischen Antikörpern und die gute Korrelation mit den in der Literatur beschriebenen Phänotypen zeigen, dass die Bestimmung von MSA ein wichtiges diagnostisches Hilfsmittel ist, um Verlauf, Komplikationen und Outcome abzuschätzen und frühzeitig eine adäquate Therapie einzuleiten.

Abstract

Background

Juvenile dermatomyositis (JDM) is a rare autoimmune disease associated with typical skin changes and muscle weakness. Within the framework of the diagnostics, myositis-associated (MAA) and myositis-specific antibodies (MSA) can be detected. These are important for the assessment of the course of the disease and the prognosis.

Method

In this study we searched for MAA and MSA by means of a line immunoassay in 12 currently supervised JDM patients in the Rheumatism Center Sankt Augustin.

Results

In 10 of the 12 patients a total of 15 myositis antibodies were detected where 3 patients each had Mi2, SRP or NXP2 antibodies, 2 had TIF-1γ antibodies and Jo1 or Mi2β antibodies were found in 1 patient each. Of the patients two had additional PM-Scl antibodies. In the 10 patients with detected antibodies, a good phenotype-serotype correlation was found with deviation from the phenotypes described in the literature in only 3 patients.

Conclusion

The frequent detection of certain antibodies and the good correlation with those phenotypes described in the literature, show that the determination of MSA is an important diagnostic tool to assess the course, complications and outcome and to initiate adequate therapy at an early stage.

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Correspondence to K. Eising.

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K. Eising, J. Peitz, N. Unterwalder, C. Meisel und G. Horneff geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben.

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U. Müller-Ladner, Bad Nauheim

U. Lange, Bad Nauheim

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Eising, K., Peitz, J., Unterwalder, N. et al. Myositisspezifische Antikörper bei juveniler Dermatomyositis. Z Rheumatol 77, 735–740 (2018). https://doi.org/10.1007/s00393-017-0415-0

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Schlüsselwörter

  • Juvenile Dermatomyositis
  • Myositisspezifische Antikörper
  • Myositisassoziierte Antikörper
  • Phänotyp-Serotyp-Korrelation

Keywords

  • Juvenile dermatomyositis
  • Myositis-specific antibodies
  • Myositis-associated antibodies
  • Phenotype serotype correlation