Pathologie idiopathischer entzündlicher Muskelerkrankungen

Zusammenfassung

Die Myositis ist definiert als Entzündung des gefäßführenden interstitiellen Bindegewebes des Skelettmuskels mit Beteiligung der Muskelfasern. Dabei kann der inflammatorische Prozess auf die Skelettmuskulatur beschränkt sein oder andere Organsysteme miteinbeziehen. Klinisch geht sie einher mit Muskelschwäche, Muskelatrophien und gelegentlich auch Muskelschmerzen. Eine frühzeitige Diagnosestellung und Therapie sind für die Prognose essenziell. Zu den idiopathischen entzündlichen Muskelerkrankungen des Kindesalters zählen neben der juvenilen Dermatomyositis, der Polymyositis und den sogenannten Overlap-Syndromen im Rahmen systemischer Autoimmunerkrankungen auch seltenere Erkrankungen wie die nekrotisierende autoimmune Myopathie, fokale Myositiden wie die Makrophagen-Myofasziitis oder die granulomatöse Myositis. Die sporadische Einschlusskörperchen-Myositis ist im Kindesalter nicht prävalent.

Summary

Myositis is defined as inflammation of the interstitial connective tissue in the skeletal muscle with involvement of the muscle fibers. The inflammatory process can be restricted to the muscle itself or involve other organs. Clinical symptoms are muscle weakness, muscle atrophy, and occasionally muscle pain. An early diagnosis and start of treatment are essential for the prognosis. Idiopathic inflammatory myopathies in childhood include juvenile dermatomyositis, polymyositis, and overlap syndromes in the context of other systemic autoimmune diseases, but also more rare conditions like necrotising autoimmune myopathy, focal inflammatory myopathies like macrophagic myofasciitis, and granulomatous myositis. Sporadic inclusion body myositis does not occur in childhood.

Schlüsselwörter

Entzündliche Muskelerkrankungen - juvenile Dermatomyositis - Polymyositis - nekrotisierende autoimmune Myopathie - Makrophagen-Myofasziitis - granulomatöse Myositis

Keywords

Inflammatory myopathies - juvenile dermatomyositis - polymyositis - necrotising autoimmune myopathy - macrophagic myofasciitis - granulomatous myositis

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