Fibrosing vasculitis in Wegener's granulomatosis : ultrastructural and immunohistochemical analysis of the vascular lesions

This study of two cases of pulmonary Wegener's granulomatosis (WG) focuses on the ultrastructural aspects of the vascular wall injury and on the immunohistochemical characterization of the perivascular connective matrix. The iterative waves of endothelial cell necrosis and regeneration are demo...

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Bibliographic Details
Published in:Virchows Archiv : an international journal of pathology 1995-12, Vol.427 (4), p.385-393
Main Authors: GINDRE, D, PEYROL, S, RACCURT, M, SOMMER, P, LOIRE, R, GRIMAUD, J. A, CORDIER, J. F
Format: Article
Language:English
Subjects:
Arteries - chemistry
Biological and medical sciences
Extracellular Matrix Proteins - analysis
Granulomatosis with Polyangiitis - pathology
Humans
Lung - blood supply
Lung - chemistry
Male
Medical sciences
Microscopy, Electron
Middle Aged
Pulmonary Fibrosis - pathology
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Vasculitis - pathology
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Summary:This study of two cases of pulmonary Wegener's granulomatosis (WG) focuses on the ultrastructural aspects of the vascular wall injury and on the immunohistochemical characterization of the perivascular connective matrix. The iterative waves of endothelial cell necrosis and regeneration are demonstrated by the multilamellar appearance of the basal lamina. Neutrophils infiltrate the vessel wall and myofibroblasts are recruited to injured vessels. The perivascular connective matrix associates basement-membrane like and fibrillar material with fibrin deposits. The initiation of the fibrosing process was assessed by the visualization of matrix molecules involved in targeting (p-fibronectin), organizing (cellular fibronectin and tenascin) and stabilizing (lysyl-oxidase) the fibrogenic activity. These elementary lesions affect different levels of the vascular tree, and capillaritis is involved in the extension of the pathological process. Lysyl-oxidase labelling reveals the fibrosing front which is located on the border of dense fibrosis. The markers of fibrosing activity disappear in the areas of fibrosis following vasculitis and/or ischaemic necrosis and/or granulomatosis. Vasculitis plays a major role in both the genesis and progression of the fibrosis observed in the late stage of WG.
ISSN:0945-6317
1432-2307
DOI:10.1007/BF00199387