In Reply to: Re: Case 0498_01 -- Unexplained lethal vasculitis posted by Pasteur Rasuli on April 10, 1998 at 12:39:21:
1) Have you seen a specific arteritis with this appearance and distribution?
No but we don't think this is an arteritis
2) Do you feel that this patient's spontaneous hepatic bleed, liver infarct, and/or renal failure are related to these findings?
They could all fit the diagnosis of SAM.
3) What would you have done for further evaluation?
Get the pathologists to review the histology - apparently there is no treatment except to resect infarcted or bleeding areas.
Probably this is something other than a vasculitis, especially with the absence of autopsy findings to indicate arteritis. The raised ESR could be the result of the acute bleed and infarction in the abdomen. Although it is difficult to see, even on the magnified images, your description of infarction, bleeding, segmental narrowing and dilatation is consistent with and is a good description of a case of segmental arterial mediolysis (SAM). This is rare (14 cases described by last year - is this the 15th to be described ? - we believe JG has the 16th case pending more detailed histology). Would suggest review of histology to look for this specifically - Useful references are: - Sakano et al, Clinical Radiology 1997;70:656-658 and Slavin et al, Modern Pathology 1995;8:287-294. If we get confirmation of JG's case we will submit to case club as well.