Progrediente pulmonale Beteiligung bei amyopathischer Dermatomyositis

Zusammenfassung

In dieser Fallserie werden 3 Patienten mit einer Dermatomyositis bzw. Polymyositis autoimmuner Genese vorgestellt, die nach Entwicklung einer rasch progredienten interstitiellen Lungenbeteiligung trotz Immunsuppressiva innerhalb von Monaten verstarben. Alle 3 Patienten hatten ein passendes Antikörperprofil – bei 2 wurden Myositis-spezifische anti-melanoma-differentiation-associated-gene (MDA5)-Antikörper und bei der dritten Myositis-assoziierte Anti-Ku-Antikörper nachgewiesen. Dermatomyositis und Polymyositis sind seltene Autoimmunerkrankungen mit unterschiedlichen klinischen Manifestationen und Antikörperkonstellationen. Insbesondere das Vorliegen von MDA5-Antikörpern ist mit einer rasch progredienten pulmonalen Beteiligung und einer schlechten Prognose assoziiert.

Abstract

In this case series we present three patients with autoimmune dermatomyositis or polymyositis with rapid-progressive interstitial pulmonary involvement. Despite intensive escalation of the immunosuppressive therapy the patients developed acute respiratory distress syndrome with lethal outcome only a few months after diagnosis. All three patients had increased myositis-specific or myositis-associated antibodies – two patients were detected with anti-melanoma differentiation-associated gene (MDA5) antibodies and the third patient with anti-Ku antibodies. Dermatomyositis and polymyositis are rare autoimmune diseases with variable clinical manifestations and several different antibody constellations. In particular the presence of anti-MDA5 antibodies is associated with progressive pulmonary involvement, complicated progression and poor prognosis.

Schlüsselwörter

Dermatomyositis - Polymyositis - interstitielle Lungenerkrankung - MDA5 - amyopathische Dermatomyositis

Key words

dermatomyositis - polymyositis - interstitial lung disease - MDA5 - amyopathic dermatomyositis

Publication History

Article published online:
19 March 2020

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