Malignant Hypertension due to Musculo-Mucoid Intimal Hyperplasia of Intrarenal Arteries: ABSENCE OF RENAL FIBRINOID NECROSIS

• Published in: Circulation research 1975-06, Vol.36 (6 Suppl 1), p.I-133-I-144
• Main Authors: Pitcock, James A, Johnson, James G, Share, Leonard, Hatch, Fred E, Acchiardo, Sergio R, Black, William D, Muirhead, E E
• Format: Article
• Language: English
• Subjects: Acute Kidney Injury - etiology; Adult; African Continental Ancestry Group; Basement Membrane - ultrastructure; Female; Glycosaminoglycans - metabolism; Humans; Hyalin - metabolism; Hyperplasia; Hypertension, Malignant - etiology; Hypertension, Malignant - therapy; Juxtaglomerular Apparatus - pathology; Kidney - pathology; Kidney Glomerulus - pathology; Kidney Glomerulus - ultrastructure; Kidney Tubules - pathology; Male; Microcirculation - pathology; Microscopy, Electron; Middle Aged; Nephrectomy; Papilledema - etiology; Renal Artery - pathology; Renal Artery Obstruction - complications; Renal Artery Obstruction - pathology; Renin - blood
• ISSN: 0009-7330; 1524-4571
• DOI: 10.1161/01.RES.36.6.133

Severe essential hypertension in a subset of American black subjects is associated with marked stenosis of interlobular arteries and arterioles of the kidneys, observed by renal biopsy and binephrectomy specimens. The interlobular arterial stenosis is caused by marked thickening of the intima due mainly to the presence of smooth muscle cells, basement membrane material, and acid mucopolysaccharide. Because of this makeup, we propose the term “musculo-mucoid intimal hyperplasia” for this lesion.The media of these arteries appears maximally dilated, and by electron microscope displays degenerative changes of the smooth muscle cells. The arterioles are thickened, due mainly to hyalinization, but also due to the musculo-mucoid change (onionskin effect). The smooth muscle cells are degenerated and atrophic. These patients do not exhibit fibrinoid necrosis of the arteries, arterioles, and glomeruli, presumably because of the rapidity of the development of the arterial stenotic lesion. Accordingly, the glomeruli are destroyed by ischemia, and there is no evidence of glomerulitis (no “Kombinations” form ofFahr).A unifying hypothesis concerning renal hypertensive arterial disease is suggested by these studies. This hypothesis places the main emphasis for all the morphological expressions of the intrinsic visceral vasculature on changes involving the main functional unit of the vessel wall, the medial smooth muscle.