Pulmonale segmentale mediolytische Arteriopathie

Abstract

Segmental mediolytic arteriopathy (SMA) is defined as non-inflammatory arteriopathy with mediolysis due to segmental loss of media and consecutive formation of vascular gaps. Up to now, less than 40 cases of visceral and cerebral SMA and, to our knowledge, only one case of pulmonary SMA have been reported. We present the history of a 21 year old female patient, admitted to hospital with hemoptysis, but without other symptoms. Apart from two lesions in the sixth and tenth pulmonary segment, documented by CT and interpreted as colliquations, there were no other clinical and laboratory findings. Repeated bronchoscopy supplied no further information. Histomorphology of the resected lesion revealed SMA without evidence of vasculitis. Wegener's disease could be excluded. The aetiology of the disease is still unknown. Acute vasospasm (due to inappropriate reactions to catecholamine or endothelial dysfunction), as well as SMA as a precursor or subtype of fibromuscular dysplasia, are two theories still under discussion.

Segmental mediolytische Arteriopathie – Ursachenforschung bei unter 60-jährigen Patienten mit Aneurysmen und Dissektionen

“…In einer von Pathologen aufgearbeiteten Übersicht über die Lokalisation der betroffenen Gefäßabschnitte finden sich etwa 40 Fälle weltweit, die eine abdominelle SMA aufwiesen [3]. [14,15].…”

“…Main findings were that the vessel wall layering was destroyed and that capillarisation was initiated from the adventitial layer. [3,9].…”

“…Der Fall eines 24-jährigen stellt eine Rarität dar[3]. Alle epikardialen SMA-Verläufe endeten mit myokardialer Ischämie letal.…”

“…Alle epikardialen SMA-Verläufe endeten mit myokardialer Ischämie letal. Ebenfalls als Rarität wurde 2006 eine pulmonale SMA von Pathologen beschrieben, die bei einer 21-jährigen mit Hämoptysen auffiel[3]. Zur adäquaten Therapieplanung stellt die schwierige Diagnosestellung eine Herausforderung bei den meist jungen Patienten dar.…”

Segmental arterial mediolysis of left gastric artery: a case report and review of pathology

“…SAM characteristically involves small to medium sized muscular abdominal and visceral arteries, but pulmonary [2,5] and cerebral [6] cases are also reported. Both genders are equally affected with predominance in the middle aged and the elderly [7-11].…”