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Dermatomyositis und juvenile Dermatomyositis

Dermatomyositis and juvenile dermatomyositis

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Zusammenfassung

Die Dermatomyositis (DM) ist eine entzündliche Multisystemerkrankung unklarer Ätiologie, die bereits im Kindesalter beginnen, aber auch erst im hohen Erwachsenenalter auftreten kann. Myalgien und Muskelschwäche können erst im Verlauf der Erkrankung auftreten oder bei manchen Formen sogar ganz fehlen. Klassische Zeichen an der Haut sind heliotroper Ausschlag, Wangenerythem, Gottron-Zeichen sowie Nagelbettgefäßveränderungen. Bei der Diagnosestellung spielen v. a. auch zunehmend die Bestimmung Myositis-spezifischer Autoantikörper eine Rolle. Die Muskelenzyme können erhöht sein, sind es jedoch bei ca. einem Drittel der Patienten nicht. In Absenz typischer klinischer und serologischer Befunde können zur Diagnosestellung weitere Untersuchungsmethoden, wie z. B. Kapillarmikroskopie, Magnetresonanztomographie, Elektromyographie, Haut- oder Muskelbiopsie, in Betracht gezogen werden. Je nach klinisch-serologischem Subtyp empfiehlt sich auch ein gastrointestinales oder kardiopulmonales Organscreening. Bei erwachsenen Patienten sollte zusätzlich auch eine altersadaptierte Tumorsuche durchgeführt werden. Neben Kortikosteroiden zur Induktionstherapie und konventionellen Basistherapeutika und Immunglobulinen spielen zunehmend auch Biologika und Small-molecule-Inhibitoren eine Rolle. Die Prognose der DM/JDM (juvenile Dermatomyositis) hat sich verbessert. Während sich die meisten Patienten zumindest partiell erholen, versterben einzelne, und eine Minderheit entwickelt eine persistierende Muskelatrophie oder eine ausgeprägte Kalzinose.

Abstract

Dermatomyositis (DM) is an inflammatory multisystem disease of unknown etiology, which can already occur in children but first onset can also be in older adulthood. Myalgia and muscle weakness can occur later in the course of the disease or even be completely absent in some forms. Classical signs on the skin include heliotrope rash, facial erythema, Gottron’s papules and nailfold capillary abnormalities. For the diagnosis, screening for the presence of myositis-specific autoantibodies has become increasingly more relevant. Muscle enzymes may be elevated but not in approximately one third of patients. In the absence of typical clinical or serologic findings, additional examination methods such as nailfold capillaroscopy, magnetic resonance imaging, electromyography, skin or muscle biopsies may help to establish the diagnosis. Depending on the clinical and serological subtype, additional screening for gastrointestinal or cardiopulmonary involvement should be considered. In adults, an age-appropriate tumor screening should also be performed. Apart from corticosteroids as induction therapy, biologics and small molecule inhibitors are gaining in importance in addition to conventional disease-modifying anti-rheumatic drugs and intravenous immunoglobulins. The prognosis for DM and juvenile DM (JDM) has improved. Most patients recover at least to some extent; however, a few patients die and a minority develop persisting muscle atrophy or severe calcinosis.

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Authors and Affiliations

  1. Kinderklinik, Medizinische Hochschule Hannover, 30623, Hannover, Deutschland

    Frank Dressler

  2. Universitätsklinik für Rheumatologie und Immunologie, Inselspital Bern, 3010, Bern, Schweiz

    Britta Maurer

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  1. Frank Dressler
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  2. Britta Maurer
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Correspondence to Frank Dressler or Britta Maurer.

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Dressler, F., Maurer, B. Dermatomyositis und juvenile Dermatomyositis. Z Rheumatol 82, 233–245 (2023). https://doi.org/10.1007/s00393-022-01205-5

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