{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T02:55:46Z","timestamp":1767840946622,"version":"3.49.0"},"reference-count":18,"publisher":"Georg Thieme Verlag KG","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Eine seltene Form der Dermatomyositis ist die klinische amyopathische Dermatomyositis (CADM). Charakterisiert ist sie durch das Fehlen einer bzw. durch eine nur sehr gering ausgepr\u00e4gte Muskelbeteiligung. Dementsprechend sind die Kreatinkinase-Werte meist im Normalbereich. Typische Hautmanifestationen sind Gottron-Papeln und Mechanikerh\u00e4nde. Bei Nachweis eines MDa5 (Melanoma-differentiation-associated gene 5 intracellular pathogen sensor)-Antik\u00f6rpers ist die CADM h\u00e4ufig mit einer rasch progredienten und schweren Verlaufsform einer interstitiellen Lungenerkrankung assoziiert. In der Lungenfunktion l\u00e4sst sich eine Restriktion und eine Hypox\u00e4mie unterschiedlichen Ausma\u00dfes nachweisen. Die HRCT-Bildgebung ist nicht spezifisch. Es lassen sich u.\u200aa. Milchglas, Retikulationen und Konsolidierungen beobachten. In der bronchioloalveol\u00e4ren Lavage zeigen sich gelegentlich vermehrt Lymphozyten. Histologisch \u00e4hnelt das Bild einer nicht-spezifischen interstitiellen Pneumonie oder einer organisierenden Pneumonie. Die Therapie richtet sich nach der Schwere der Auspr\u00e4gung der klinischen Manifestation. Im Allgemeinen ist eine ausgepr\u00e4gte immunsuppressive Therapie notwendig. Meist m\u00fcssen verschiedene Immunsuppressiva kombiniert werden. Ein neuer Therapieansatz stellt die Verwendung des Januskinase-Inhibitors Tofacitinib dar. Zur Therapiekontrolle k\u00f6nnen der Ferritinspiegel und der MDa5-Antik\u00f6rper-Titer verwendet werden. Es besteht eine hohe Mortalit\u00e4t von bis zu 84\u200a%.<\/jats:p>","DOI":"10.1055\/a-1177-4209","type":"journal-article","created":{"date-parts":[[2020,7,14]],"date-time":"2020-07-14T22:49:54Z","timestamp":1594766994000},"page":"780-786","source":"Crossref","is-referenced-by-count":1,"title":["Die Diagnose liegt auf der Hand"],"prefix":"10.1055","volume":"74","author":[{"given":"C.","family":"Fisser","sequence":"additional","affiliation":[{"name":"Klinik und Poliklinik f\u00fcr Innere Medizin II, Universit\u00e4tsklinik Regensburg, Regensburg"},{"name":"Abteilung f\u00fcr Pneumologie, Fachklinik f\u00fcr Lungenerkrankungen Donaustauf, Donaustauf"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Wiest","sequence":"additional","affiliation":[{"name":"Klinik und Poliklinik f\u00fcr Innere Medizin II, Universit\u00e4tsklinik Regensburg, Regensburg"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"O. 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