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„Maligne“ Hypertonie und atypisches hämolytisch-urämisches Syndrom (aHUS)

“Malignant” hypertension and atypical hemolytic uremic syndrome (aHUS)

Zusammenfassung

Die schwere („maligne“) Hypertonie mit funktioneller Schädigung von Herz, Gehirn oder Niere ist eine seltene, aber sehr gefährliche Erkrankung. Das Kennzeichen der Erkrankung ist nicht nur der schwere Bluthochdruck, sondern auch die damit verbundene Organschädigung. Der hohe Blutdruck führt zu einer Schädigung des Endothels in großen und kleinen Gefäßen und induziert damit Endothelzelldysfunktion mit Vasokonstriktion, Inflammation und Thrombose. Diese Bluthochdruckkrisen können durch unterschiedliche Mechanismen ausgelöst werden. Einer der Mechanismen ist die thrombotische Mikroangiopathie (TMA). Bei der TMA, v. a. bei Störungen des Komplementsystems, kommt es zu einer Schädigung des Endothels in der Mikrozirkulation mit Organversagen. Gleichzeitig ist eines der klinischen Kennzeichen der Erkrankung der hohe Blutdruck. Die Diagnostik einer TMA, welche dem klinischen Symptom schwerer Hypertonie zugrunde liegt, ist nicht einfach. Es bedarf einer sehr sorgfältigen klinischen Untersuchung der Patienten, um das Ausmaß der Organschädigung sowie die Progression frühzeitig festzuhalten. Begleitende Laboruntersuchungen geben Hinweise, aber bislang gibt es keine eindeutige Labordiagnostik für die komplementvermittelte TMA. Bei Verdacht auf atypisches hämolytisch-urämisches Syndrom (aHUS) sollten frühzeitig Inhibitoren des Komplementsystems eingesetzt werden.

Abstract

Severe or malignant hypertension with functional damage to the heart, brain or kidneys is a rare but very dangerous disease. The disease is characterized not only by the severe high blood pressure but also by the accompanying organ damage. The high blood pressure leads to damage to the endothelium in large and small vessels and therefore induces endothelial cell dysfunction with vasoconstriction, inflammation and thrombosis. These high blood pressure crises can be triggered by a variety of mechanisms. One of these mechanisms is thrombotic microangiopathy (TMA). The TMA, particularly in disorders of the complement system, leads to damage to the endothelium in the microcirculation with organ failure. Simultaneously, one of the clinical features of the disease is the high blood pressure. The diagnostics of TMA, which is caused by the clinical symptom of severe hypertension, are not easy. It necessitates a very meticulous clinical examination of the patient in order to determine the extent of organ damage and the progression at an early stage. Concomitant laboratory investigations provide indications but so far there are no certain laboratory diagnostics for complement-mediated TMA. When an atypical hemolytic uremic syndrome (aHUS) is suspected inhibitors of the complement system should be initiated as early as possible.

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Correspondence to Hermann Haller.

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H. Haller und J. Kaufeld geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Hermann Haller, Hannover

Joachim Hoyer, Marburg

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Haller, H., Kaufeld, J. „Maligne“ Hypertonie und atypisches hämolytisch-urämisches Syndrom (aHUS). Nephrologie 17, 246–254 (2022). https://doi.org/10.1007/s11560-022-00591-w

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Schlüsselwörter

  • Hypertensiver Notfall
  • Sekundäre Hypertonie
  • Thrombotische Mikroangiopathie
  • Mikrozirkulation
  • Komplement

Keywords

  • Hypertensive emergency
  • Secondary hypertension
  • Thrombotic microangiopathy
  • Microcirculation
  • Complement system