Sonographic Signs of Amyloidosis

Zusammenfassung

Einleitung: Amyloidosen stellen lebensbedrohliche systemische Erkrankungen dar, die auch junge Patienten betreffen können. Die sonografischen Zeichen sind vielfältig und bisher nicht gut charakterisiert. Patienten: 30 Patienten mit unterschiedlichen Formen einer Amyloidose (19 Patienten ATTR-Amyloidose, 11 Patienten mit anderen generalisierten Amyloidose-Formen) wurden sonografisch evaluiert. Bei allen Patienten wurde die Amyloidose histologisch verifiziert. Ergebnisse: Typische Zeichen des Herzbefalls sind Zeichen einer Polyserositits in Form eines Perikard- und/oder Pleueraergusses sowie die in manchen Fällen sonografisch erkennbaren subendokardialen echoreichen Ablagerungen. Der Darmbefall ist initial kurzstreckig und segmental. Auch hier finden sich typischerweise fokale echoreiche Ablagerungen in der Darmwand. Weitere sonografische Zeichen sind: spontane, variabel echogene subkapsuläre Leber- und Milzeinblutungen sowie vielfältige Nierenveränderungen. Diskussion: Typische sonografisch erkennbare Veränderungen finden sich insbesondere am Herzen, dem unteren Gastrointestinaltrakt, der Leber, der Milz und den Nieren, die in Form eines Pictoral Assays dargestellt werden.

Abstract

Introduction: Amyloidosis is a rare group of diseases with a variety of symptoms that occurs not only in multimorbid elderly but also in young patients. However, sonographic signs of amyloidosis have not been described so far. Patients and Methods: 30 patients with different forms of amyloidosis (19 patients with TTR-amyloidosis (familial amyloid Polyneuropathy [FAP]), 11 patients with other forms of systemic amyloidosis) were sonographically evaluated in a standardised fashion. In all patients amyloidosis was histologically verified. Results: Typical signs of cardiac amyloidosis were myocardial thickness, pericardial effusion, pleural effusion and typical echorich subendocardial depositions. Signs of kidney infiltration were stage dependent and rather unspecific. Spontaneous subcapsular haemorrhages were the typical signs of liver and spleen infiltration. Intestinal affection was characterised by patchy-like inhomogeneous depositions. Rare sites of amyloidal affection could also be verified, e. g. gallbladder and thyroid infiltration. Discussion: Typical sonographic signs of amyloidosis could be found in the heart, bowel wall, liver, spleen, gallbladder and thyroid and were illustrated as pictorial assays.

Schlüsselwörter

Ultraschall - Sonografie - Amyloidose - Pictorial Assay - Blutungen - Doppler

Key words

ultrasound - sonography - amyloidosis - pictorial assay - haemorrhage - doppler

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