[Pulmonary segmental mediolytic arteriopathy].
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.
Müller
A M
AM
Institut für Pathologie der Ruhr-Universität Bochum, Berufsgenossenschaftliche Kliniken Bergmannsheil. mueller@kinderpatho.klinik.uni-mainz.de
Kullmann
H J
HJ
D000328:Adult; D000783:Aneurysm / Q000473:pathology; D001167:Arteritis / Q000473:pathology*; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D005352:Fibromuscular Dysplasia / Q000473:pathology; D006469:Hemoptysis / Q000209:etiology / Q000473:pathology; D006801:Humans; D008171:Lung Diseases / Q000473:pathology*; D011650:Pulmonary Alveoli / Q000473:pathology; D011651:Pulmonary Artery / Q000473:pathology*; D012008:Recurrence; D014057:Tomography, X-Ray Computed; D017539:Tunica Intima / Q000473:pathology; D017540:Tunica Media / Q000473:pathology*; D014661:Vasoconstriction / Q000502:physiology