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Systemic lupus erythematosus with hepatic aneurysm, Valsalva sinus aneurysm and associated polyangiitis: aneurysmal wall remodeling with dense fibrosis and calcification mediated by residual smooth muscle cellsDepartment of Pathology, Saiseikai Central Hospital, Tokyo, Japan, kazutoyamazaki{at}aol.com Isolated case reports have demonstrated the combination of hepatic aneurysm and systemic lupus erythematosus (SLE). The author experienced a rare autopsy case of a 36-year old Japanese male with SLE, a hepatic aneurysm, a Valsalva sinus aneurysm and associated polyangiitis. In the histopathological and ultrastructural examinations of the postmortem organs, most of the angiitis lesions were in the scar phase associated with the histopathological features of collagenous fibrosis with the diminution of the smooth muscle cells and elastic fibre layers. Massive calcification was seen on the wall and obliterative changes in the lumen with calcified thrombi. In the dense collagenous stroma of the aneurysmal wall, residual atrophic smooth muscle cells with immunohistochemical (HHF35, alpha-smooth-muscle actin, vimentin and desmin almost-) and ultrastructural features (spindle-shapedcells with a few rER and rich intracytoplasmicfilaments with peripheraldense patches)were assumed to be involvedin the histogenesisof the aneurysmalwall. Massivecalcification and degenerationor dynamic remodelingof the extracellularmatrices in the aneurysmalwall might be mediated by the residual smooth muscle cells. It was suspected that the generalized polyangiitis as a complication of SLE might have involved the intrahepatic arteries and Valsalva sinus wall and subsequently generated the aneurysm.
Key Words: collagen • extracellular matrix • lamina • medial degeneration • myofibroblast
Lupus, Vol. 13, No. 1,
54-59 (2004) |
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