Abstract
“Autoimmunity” is a designation dependent on the conventional immunological issue of self/non-self discrimination. Identification of novel target autoantigens is still an important issue ongoing in classical tissue-specific autoimmune bullous diseases and autoimmune connective tissue diseases. In contrast, synchronized with the paradigm shift of the fundamental aspect of immunity to danger sensing/signaling, distinct collagen-like diseases have been defined by the genetic mutations causing dysregulated innate immunity/inflammation and have been designated as “autoinflammatory” diseases. Due to the clinical and etiological similarities, the concept of autoinflammatory diseases has expanded to include non-hereditary collagen-like diseases, tissue-specific chronic idiopathic inflammatory diseases and metabolic diseases. On the other hand, various genetic causes of autoimmune diseases have been identified and the border of these two pathophysiologies is becoming obscure. Instead, a variable mixture of both autoimmunity and autoinflammation can cause each inflammatory phenotype with a variable level of antigen specificity.
Zusammenfassung
Unter „Autoimmunität“ versteht man konventionell das immunologische Problem der Diskriminierung von Selbst und Nicht-Selbst. Die Identifizierung weiterer Target-Autoantigene bei den klassischen gewebe-spezifischen autoimmunen bullösen Dermatosen und den autoimmunen Bindegewebserkrankungen bleibt auch weiterhin ein wichtiges Anliegen. Dabei hat sich das Verständnis der fundamentalen Aspekte der Immunologie hin entwickelt zum Themenkomplex der Gefahrenerkennung (danger sensing) und Signalübertragung. Bei den Kollagenosen wurden teils genetische Mutationen entdeckt, die verantwortlich zeichnen für Störungen der Immunität und Entzündungskaskade. Die autoinflammatorischen Erkrankungen wurden definiert. Aufgrund klinischer und ätiologischer Ähnlichkeiten wurde das Konzept der autoinflammatorischen Erkrankungen auf nicht hereditäre Bindegewebserkrankungen, gewebe-spezifische chronisch idiopathisch-entzündliche Erkrankungen und metabolische Erkrankung ausgedehnt. Andererseits wurden verschiedene genetische Ursachen der Autoimmunerkrankungen entdeckt, so dass das die Grenzen der klassischen Pathologien verschwimmen. In der Tat kann eine Mischung von Autoimmunität und Autoinflammation nahezu jeden Entzündungs-Phänotyp mit variablem Level der Antigenspezifität auslösen.
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Kanazawa, N., Tchernev, G. & Wollina, U. Autoimmunity versus Autoinflammation - Friend or Foe?. Wien Med Wochenschr 164, 274–277 (2014). https://doi.org/10.1007/s10354-014-0290-0
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DOI: https://doi.org/10.1007/s10354-014-0290-0