Showing papers on "Fibrinoid necrosis published in 1992"

Lupus Nephritis: Prognostic Factors in Children

Abstract: As newer treatment modalities become available for patients with severe lupus nephritis, it becomes increasingly important to identify patients at risk for renal failure. In this study, the records of 90 children presenting with systemic lupus erythematosus over a 13-year period were reviewed. Nineteen were lost to follow-up prior to completion of the study. Of the 71 remaining children, 16 (22%) progressed to chronic renal failure. Persistent hypertension lasting greater than 4 months, anemia, abnormalities of the urinalysis, and elevated serum creatinine level were significantly associated with progression to renal failure. Sex, race, age, abnormalities of creatinine clearance, and 24-hour urine protein collection were not associated with progression to renal failure. Renal biopsies were obtained in 45 children. Biopsies were initially classified according to World Health Organization criteria. Diffuse proliferative glomerulonephritis was significantly associated with progression to renal failure. The 45 biopsies available were reviewed by one of the authors and categorized by activity and chronicity indices. Both the active lesions of fibrinoid necrosis, synechiae, tubular casts, and vasculitic lesions and the chronic lesion of glomerular sclerosis correlated with progression to renal failure. Of the 16 children who progressed to renal failure, 2 had cadaver kidney transplants and are well 5 years posttransplant; 4 had fulminant lupus and died within 1 month of commencing dialysis; 10 began chronic dialysis. Five of the 10 children on chronic dialysis died from sepsis. These data suggest that children with systemic lupus erythematosus who undergo dialysis do poorly. This alters the risk-to-benefit ratio of newer, more aggressive treatment protocols which should be considered in children whose clinical, laboratory, and renal biopsy findings suggest eventual progression to renal failure.

Systemic necrotizing vasculitis in nine young beagles.

Abstract: A systemic necrotizing vasculitis of unknown etiopathogenesis may be termed juvenile polyarteritis syndrome (JPS). The syndrome has been recognized primarily in young Beagles used for toxicologic studies. We studied 9 young Beagles with JPS. Affected dogs had fever (40 to 41.5 C), anorexia, and signs of pain in the cervical area. They had a characteristic hunched stance, and were unwilling to move. Laboratory abnormalities in all dogs included nonregenerative anemia, hypoalbuminemia, and leukocytosis characterized by a mature neutrophilia. Analysis of CSF revealed a moderate to severe neutrophilic pleocytosis and a mildly high protein concentration in most dogs. Signs of disease resolved rapidly with high doses (2.2 mg/kg of body weight, PO) of prednisone. If untreated, clinical signs and laboratory abnormalities had a remitting and relapsing course in most dogs. Findings at necropsy included necrotizing arteritis with fibrinoid necrosis, periarteritis, thrombosis, and intimal proliferation that most frequently affected small- to medium-sized vessels in the cervical spinal cord, mediastinum, and heart. An immune-mediated pathogenesis for this disease is suspected.

Concurrent temporal arteritis and Churg-Strauss syndrome.

Abstract: We describe the case of a 41-year-old man who presented with clinical and histopathologic evidence of temporal artery lesions associated with the Churg-Strauss syndrome. Pathological examination of the temporal artery showed panarteritis without giant cell formation or fibrinoid necrosis. We review the world literature concerning the vasculitides with features that overlap giant cell arteritis (GCA) and polyarteritis nodosa (PAN) and classify into 2 sub-groups PAN with unusual temporal artery localization and GCA with variably disseminated arterial injuries. These cases emphasize the fact that not all arteritis involving the temporal arteries is GCA. Only 3 cases with temporal artery involvement and concurrent Churg-Strauss syndrome have been published.

The pathogenesis of radiation myelopathy: Widening the circle

Abstract: The fact that the spinal cord can react to insult in a limited number of ways is again demonstrated in the article by Powers et al. (6). These authors show that the canine spinal cord reacts to radiation in much the same way as other experimental animals and humans, and that dogs apparently have latent periods that are comparable with other large animals, with the exception of the pig, of course. The new findings in this report are the reactions of the meninges and the dorsal root ganglia. Although many attempts have been made to categorize radiation lesions of the spinal cord, no classification scheme has met with wide spread acceptance. The reasoning behind the attempts at categorization is that better definition of the histopathology may assist in better understanding of the pathogenesis. The reasons that this effort has been largely unsuccessful are (a) not enough analysis as been directed at the changes during the latent period, (b) after the injury has advanced far enough to cause signs, the histological lesions are varied in both age and morphology, and (c) the pathogenesis of many different types of insult lead to similar lesions in the CNS. In pathology as in art, diferent interpretations can be given to the same image. The fundamentals of the pathology of radiation myelopathy are: the lesions are nearly always confined to the white matter; the primary lesions are confined to the irradiated portion of the cord with dying back and Wallerian degeneration occurring outside the field; a mononuclear inflammatory response is often observed, vascular changes may include hyaline thickening, telangiectasia, fibrinoid necrosis, vascular occlusion, focal hemorrhage, and hemorrhagic necrosis; parenchymal responses include demyelination and gliosis; and white matter necrosis may occur without apparently significant vascular injury. We would like to address each of these fundamentals in turn. Because of the overwhelming propensity for changes to occur in the white matter, it is unlikely that extramedullary arteries or arterioles are significantly involved in the pathogenesis. The grey matter is exquisitely sensitive to disruption of its oxygen supply and would show any such arterial changes to a greater degree than the white matter. Because lesions tend to be located in lateral motor tracts and edema is often found in active lesions, it can be argued effectively that the vascular related changes are more typical of a venous lesion than an arterial one. Some support for this argument can be found in the study by Reinhold et al. (8) who observed that myelopathy patients had significantly lower blood pressure than the cohort of nonmyelopathy patients. The fact that the lesions of radiation myelopathy are confined to the irradiated field argues against a role for an autoimmune response. Furthermore, the autoimmune theory has difficulties explaining, as do other theories of the pathogenesis of this injury, why the white matter necrosis involves axonal degeneration as well as demyelination. The mononuclear inflammatory response that is frequently observed in radiation lesions of the spinal cord seem to be a more common finding in human cases than in experimental animals. If this is true, then it may indicate that there is some difference in the pathogenesis between the human and animal models of human radiation myelopathy. Because it is difficult to differentiate between infiltrating blood monocytes and reactive resident microglia, it is not possible to determine what fraction of literature reports of mononuclear infiltrates represents a traditional inflammatory response and what fraction is a microglial response. The interpretation of inflammatory/ immune responses is further complicated by the fact that it is commonly impossible to differentiate lymphocytes from glial cells in sections of formalin-fixed paraffin embedded tissue (3). The vascular contribution to the radiation damage of the spinal cord is still unresolved. The unambiguous vas-

Nephrosclerosis from childhood to old age, a viewpoint

Abstract: Cortical arteries of the kidney in benign nephrosclerosis are characterized by arterial intimal fibroplasia whereas arterioles are affected by hyalinization; these features overlap in the tiniest terminal arteries. In malignant nephrosclerosis, the characteristic lesions are mucoid edema and fibrinoid necrosis respectively. A fifth kind of vasculopathy is marked by reduced calibre and increased wall thickness to diameter ratio; although this is often called “hypertrophy”, wall mass may not be increased, and the name “structural autoregulation” has been proposed in its place. Fibroplasia is found to some degree in every elderly person, even those who never experienced high blood pressure; blood pressure is elevated in those who manifest more than what is usual for the age. A review of reports concerning hypertension in subjects who lack evidence of nephrosclerosis suggests that such subjects may be statistical outliers who are likely to demonstrate regression to the mean in blood pressure rather than subsequent progression of renovasculopathies. It is proposed that uneven sclerosis of arteries may cause heterogeneity of nephrons so that some nephrons are sufficiently ischemic to evoke elevation of blood pressure by Goldblatt mechanisms, while other nephrons have structural autoregulation. A kidney with a small population of ischemic nephrons may show normal overall average function by available clinical methods. The etiology of arterial intimal fibroplasia in aging normotensives is unknown. That unknown etiology could, in theory, explain hypertension as merely the consequence of the more extreme degrees of benign renovasculopathies. A novel kind of epidemiological approach is proposed for pursuing clues to the unknown etiology.

Hypertension and vascular dementia

Abstract: Postmortem surveys on patients treated for chronic hypertension often fail to demonstrate significant vessel changes. Nevertheless, hypertensive alterations in the brain can include infarcts and hemorrhages. Autopsies in a primary care hospital have shown that hypertension can affect arteries, arterioles, and capillaries in various patterns and degrees in the brain. These vascular lesions may be associated with large and small infarcts and hemorrhages in isolated or diffuse patterns. Widespread cerebral edema can occur with rapidly progressive hypertension. Atherosclerosis, arterial and arteriolar fibrinoid necrosis, and micro-aneurysms may be observed. Chronic hypertensive encephalopathy causes vascular dementia and can be associated with subcortical arterial and arteriolar leukoencephalopathy, leukoaraiosis and/or Binswanger's disease. Epidemiologic evaluations based on complete autopsy studies need to be correlated with compliance of therapy, appropriate diagnosis of hypertension, and its long-term effects on the nervous system. Although persistent poorly controlled hypertension is known to damage the brain both acutely and chronically, the effects of intermittent hypertension remain to be defined.

Spontaneous cardiomyopathy resembling acute rheumatic heart disease in an owl monkey.

Abstract: A wild-caught male Aotus vociferans died spontaneously during quarantine. Histologic examination of the heart showed several small myocardial arteries replaced with fibrinoid necrosis and adjacent inflammation that included lymphocytes and large histiocytic cells. Less often, similar inflammatory foci were observed in the myocardium interstitium. The lesions observed in the heart of the owl monkey resembled Aschoff bodies.

Studies on antinephritic effects of plant components. (5). Effects of pherodendrin on original and crescentic-type anti-GBM nephritis in rats.

Abstract: Effects of pherodendrin (OB-5) on anti-GBM nephritis were investigated. OB-5 (50 mg/kg/day, i.p.) prevented the urinary protein excretion in original and crescentic-type anti-GBM nephritis in rats. In addition, OB-5 also inhibited the elevation of serum creatinine, urea nitrogen and cholesterol contents in both models of nephritis. Histopathological observations indicated that OB-5 prevented the hypercellularity, crescent formation, adhesion and fibrinoid necrosis in the glomeruli of nephritic rats. OB-5 and cyclosporine A, a positive control drug, prevented the increase in the number of OX-1, CD8 and ED-1 positive cells in the glomeruli. These results indicated that OB-5 may be effective in human glomerulonephritis, and anti-nephritic mechanisms of OB-5 may be due to its inhibition of the activation of macrophages or cytotoxic T cells.

An autopsy case of untreated systemic lupus erythematosus with death from acute pulmonary hemorrhage

Abstract: An autopsy case of SLE died from acute and diffuse pulmonary hemorrhage is presented. A 50 year-old woman with SLE was admitted to our hospital because of high fever, butterfly rash, discoid skin lesions and renal dysfunction. She died from acute respiratory failure before initiation of the therapy with corticosteroid. Autopsy findings revealed a massive acute intrapulmonary hemorrhage. Histological study demonstrated a pulmonary arterial vasculitis with prominent fibrinoid necrosis at muscular pulmonary artery. No remarkable deposit of immunoglobulins and complements was found within the alveolar walls and pulmonary vessels by immunofluorescence and electron microscopy. Renal histology revealed diffuse proliferative glomerulonephritis with fibrinoid necrosis, crescent formation and wireloop lesions compatible with type IVb according to the WHO classification. The granular deposit of IgM, C3 and Clq, and electron dense deposit was found by immunofluorescence and by electron microscopy, respectively, in the kidney. The small arteries and veins in other organs, such as liver, spleen, bladder, ovary and rectum also revealed fibrinoid vasculitis. Acute infectious lesion was not observed in any tissue examined. The diffuse pulmonary hemorrhage in SLE could be one of the manifestations of active and severe systemic vasculitis.