Zeitschrift für Rheumatologie

, Volume 78, Issue 2, pp 183–189 | Cite as

Treatment of adult idiopathic inflammatory myopathies with conventional immunosuppressive drugs

Results of a retrospective study
  • G. KeyßerEmail author
  • S. Zierz
  • M. Kornhuber



To gain information about the efficacy of immunosuppressive drugs as first-, second-, and third-line treatment of idiopathic inflammatory myopathies (IIM).


112 treatment cycles of 63 patients with dermatomyositis (n = 23), polymyositis (n = 33), overlap syndromes (n = 4), and undifferentiated connective tissue diseases (n = 3) were analyzed by retrospective chart analysis. Data regarding muscle strength, muscle enzymes, treatment duration, and treatment discontinuation were collected.


Azathioprine (38 cycles) and methotrexate (MTX; 24 cycles) were applied significantly longer than glucocorticoid monotherapy (9 cycles; 25 ± 21, 26 ± 29 and 7 ± 4 months, respectively; p < 0.05). MTX and azathioprine achieved a significant reduction of serum creatine kinase (CK), with MTX showing more marked effects. Treatment cycles with immunosuppressants other than MTX or azathioprine (n = 22) or with combinations of immunosuppressive drugs (n = 19) were mostly applied as third-line therapy, indicating their application in more refractory cases. Significant improvement of muscle strength was confined to MTX and azathioprine and to the first-line treatment. 8% of MTX patients withdrew due to the lack of efficacy, compared with 29% of patients taking azathioprine and 6 of 9 patients taking glucocorticoid monotherapy. In the 12 patients with Jo-1 syndrome, MTX treatment was effective for a longer time than azathioprine (44 ± 21 months vs. 27 ± 24 months, p < 0.05).


Our data confirm the effectiveness of MTX and azathioprine in the treatment of inflammatory myopathies and stress the importance of a potent first-line therapy.


Polymyositis Dermatomyositis Methotrexate Azathioprine Jo-1-syndrome 

Behandlung von idiopathischen entzündlichen Muskelerkrankungen des Erwachsenen mit konventionellen Immunsuppressiva

Ergebnisse einer retrospektiven Studie



Ziel der Analyse war die Prüfung der Effizienz von immunsuppressiven Medikamenten als Erst‑, Zweit-, und Drittlinientherapie der idiopathischen inflammatorischen Myopathien (IIM).


Retrospektiv erfolgte die Analyse von 112 Behandlungszyklen bei 63 Patienten mit Dermatomyositis (n = 23), Polymyositis (n = 33), Überlappungssyndromen (n = 4) und undifferenzierten Bindgewebserkrankungen (n = 3). Daten zu Muskelkraft, Muskelenzymen, Behandlungsdauer und Gründen des Therapieabbruchs wurden erfasst.


Azathioprin (38 Zyklen) und Methotrexat (MTX, 24 Zyklen) wurden signifikant länger angewendet als eine Glukokortikoid-Monotherapie (9 Zyklen; 25 ± 21, 26 ± 29 und 7 ± 4 Monate, p < 0,05). MTX und Azathioprin bewirkten eine signifikante Reduktion der Serumkreatinkinase (CK), die bei MTX ausgeprägter war. Behandlungsregime mit anderen Immunsuppressiva als MTX oder Azathioprin (n = 22) sowie die Kombination mehrerer Immunsuppressiva (n = 19) wurden meist als Drittlinientherapie angewendet und waren somit refraktären Fällen vorbehalten. Signifikante Verbesserungen der Muskelkraft traten nur bei MTX- und Azathioprinbehandlung und nur nach dem ersten Therapiezyklus auf. Wegen mangelnder Effektivität brachen 8 % der MTX-behandelten Patienten und 29 % der Patienten unter Azathioprin die Behandlung ab, ebenso 6 der 9 Patienten unter Glukokortikoid-Monotherapie. Bei den 12 Patienten mit Jo-1-Syndrom war die MTX-Behandlung länger effektiv als die Gabe von Azathioprin (44 ± 21 Monate vs. 27 ± 24 Monate, p < 0,05).


Die vorliegenden Daten bestätigen die Effektivität von MTX und Azathioprin in der Behandlung von IIM und betonen die Bedeutung einer potenten Erstlinientherapie.


Polymyositis Dermatomyositis Methotrexat Azathioprin Jo-1-Syndrom 



We kindly thank Ms. Ulrike Loebe for her contribution to extracting the data.

Compliance with ethical guidelines

Conflict of interest

G. Keyßer, S. Zierz, and M. Kornhuber declare that they have no competing interests.

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Internal MedicineUniversitätsklinikum HalleHalle (Saale)Germany
  2. 2.HELIOS Klinik SangerhausenSangerhausenGermany
  3. 3.Clinic for NeurologyUniversitätsklinikum HalleHalle (Saale)Germany

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