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Showing papers on "Fibrinoid necrosis published in 2006"


Journal ArticleDOI
TL;DR: A lesion characterized in its acute form by fibrinoid necrosis appears to be important in causing both lacunar infarcts and primary intracerebral bleeds, and advances in molecular genetics may prove instrumental in understanding the cause of this lesion and therefore in designing its targeted prevention.
Abstract: Lacunar infarcts and "hypertensive" primary intracerebral hemorrhages, collectively often referred to as hypertensive small vessel strokes, constitute about one third of all strokes. However, despite their public health importance, their etiopathogenesis remains ill-understood. Like all strokes, they are a heterogeneous entity, but the autopsy pathology evidence suggests that the majority are caused by a limited number of cerebral small vessel lesions. Small vessel atherosclerosis is causally implicated in a proportion of lacunar infarcts, although modern concepts of atherosclerotic plaque biology and natural history have yet to be applied to small cerebral vessels. A lesion characterized in its acute form by fibrinoid necrosis appears to be important in causing both lacunar infarcts and primary intracerebral bleeds. Advances in molecular genetics may prove instrumental in understanding the cause of this lesion and therefore in designing its targeted prevention.

230 citations


Journal ArticleDOI
TL;DR: Biopsies performed on patients with suspected vasculitic neuropathy allowed the diagnosis of ‘probable vasculitis’ if associated with at least one of the following features: regenerating small vessels, endoneurial purpura, asymmetric nerve fiber loss, and/or asymmetric acute axonal degeneration.
Abstract: We reviewed 202 biopsies performed on patients with suspected vasculitic neuropathy, of which 24 Churg-Strauss cases are studied separately. Specimens from the superficial peroneal nerve and peroneus brevis muscle were taken simultaneously by one incision. Without taking into account constitutional signs, systemic involvement was present in 131 patients, whereas the remaining 47 corresponded to non-systemic patients with lesions limited to peripheral nervous system and adjoining muscles. Diagnosis of panarteritis nodosa or microscopic polyangiitis, according to the size of involved vessels, was attested by an infiltration of vessel walls by inflammatory cells associated with fibrinoid necrosis or sclerosis. Microvasculitis was diagnosed when inflammatory infiltration concerned small vessels with few or no smooth-muscle fibers and without any necrosis. Microvasculitis was present in 11 of 46 non-systemic cases, and this predominance is statistically significant. Isolated perivascular cell infiltrates in the epineurium were considered not significant but allowed the diagnosis of 'probable vasculitis' if associated with at least one of the following features: regenerating small vessels, endoneurial purpura, asymmetric nerve fiber loss, and/or asymmetric acute axonal degeneration. Necrotizing vasculitis was visible in 60 cases: in nerve (16 cases), in muscle (19 cases), and both (25 cases). Microvasculitis was present in 25 cases: in nerve (19 cases), muscle (four cases), or both (two cases). Moreover, granulomatous vasculitis was found in the nerve of one non-systemic patient presenting also sarcoid granulomas in muscle. There were 24 'probable vasculitis' and 68 negative cases. Muscle biopsy improved the yield of definite vasculitis by 27%.

112 citations


Journal ArticleDOI
TL;DR: Although strict control of blood sugar has not been shown to reduce the overall incidence of stroke in diabetics, careful management of other associated risk factors, particularly hypercholesterolemia and hypertension, are imperative for the prevention of strokeIn diabetic patients.
Abstract: Diabetes is a major risk factor for stroke and is associated with an increase in overall stroke mortality The metabolic syndrome associated with insulin resistance is also a significant risk factor for stroke The etiology of stroke in diabetics is frequently microvascular disease from fibrinoid necrosis, which causes small subcortical infarcts designated as lacunar strokes Diabetics also have an increased incidence of large vessel intracranial vascular disease Although strict control of blood sugar has not been shown to reduce the overall incidence of stroke in diabetics, careful management of other associated risk factors, particularly hypercholesterolemia and hypertension, are imperative for the prevention of stroke in diabetic patients

30 citations


Journal ArticleDOI
TL;DR: To the first report of a case of classic PAN accompanied by SAH in which MPO-ANCA proved negative, a 65-year-old man with classic polyarteritis nodosa who developed subarachnoid hemorrhage and died.
Abstract: We report a 65-year-old man with classic polyarteritis nodosa (PAN) who developed subarachnoid hemorrhage. Polyarteritis nodosa was strongly suspected, however, the biopsy specimens of kidney and sural nerve showed no findings of vasculitis and the serum titer of antimyeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA) was negative. Cranial magnetic resonance angiography showed no findings of aneurysms. He developed subarachnoid hemorrhage (SAH) during the course and died. Autopsy confirmed fibrinoid necrosis in the medium-sized artery of multiple organs. To our knowledge, this is the first report of a case of classic PAN accompanied by SAH in which MPO-ANCA proved negative.

24 citations


Journal ArticleDOI
TL;DR: The diagnosis of limited Wegener's granulomatosis was considered, although antineutrophil cytoplasmic antibody (c-ANCA) test was negative 2 weeks after orchiectomy, and the patient showed an excellent response after local complete excision.
Abstract: A case is presented of Wegener's granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener's granulomatosis was considered, although antineutrophil cytoplasmic antibody (c-ANCA) test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.

17 citations


Journal ArticleDOI
TL;DR: Extraintestinal manifestations and inflammatory responses that may be disproportionate to the degree of intestinal inflammation provide clues to the presence of an underlying primary systemic vasculitis, and these data suggest that selective visceral angiography plays a key role in the diagnosis of vasculopathy in this context.
Abstract: Objectives: Indeterminate intestinal inflammation may result from a variety of inflammatory conditions in addition to ulcerative colitis and Crohn disease. The primary systemic vasculitides may present with intestinal inflammation and an indeterminate colitis. We set out to describe a series of children with primary systemic vasculitis who initially presented with clinical features suggestive of inflammatory bowel disease (IBD) to establish criteria that might help discriminate between IBD and primary systemic vasculitis. Methods: Ten children (6 boys, median age at presentation 8.9 years, range 0.9-14.5 years) satisfied inclusion criteria. Results: All had abdominal pain, weight loss, diarrhea (6 of 10 bloody) and laboratory evidence of a severe acute phase response. Extraintestinal clinical features included vasculitic rash, renal impairment, myalgia, testicular pain and polyarthritis. Endoscopy showed vascular changes or other macroscopic findings suggestive of vasculitis in 5 of 10 patients. Gut histology revealed indeterminate chronic inflammatory mucosal changes and one patient with small artery fibrinoid necrosis in the submucosal vessels. Extraintestinal biopsy was performed in 6 patients and had a higher yield for the demonstration of vasculitis than intestinal biopsy. The results of selective visceral angiography was suggestive of vasculitis in all patients, but was normal in 7 cases of treatment-unresponsive classic IBD. Treatment comprised corticosteroid and azathioprine in all patients. Cyclophosphamide was given to 7 of 10 patients. Conclusions: Extraintestinal manifestations and inflammatory responses that may be disproportionate to the degree of intestinal inflammation provide clues to the presence of an underlying primary systemic vasculitis, and these data suggest that selective visceral angiography plays a key role in the diagnosis of vasculitis in this context. It is important to identify and treat any vasculitic component because failure to do so may result in consequential morbidity or mortality.

15 citations


Journal ArticleDOI
TL;DR: This patient had an unusual neurological disorder related to a vasculopathy, probably mediated by a circulating neoplastic clone of activated T cells, and Identical T-cell clones were identified in the duodenum, stomach, lymph nodes, and spleen.
Abstract: Celiac disease is an enteropathy occurring in genetically predisposed individuals due to a dietary intolerance to gluten. Patients with celiac disease may develop a neurological disorder of unknown cause, although autoimmune mechanisms are suspected. We report on a 56-year-old man with celiac disease, who became refractory to a gluten-free diet and died of a rapidly progressive encephalopathy. Magnetic resonance imaging indicated focal lesions of the cerebellum and brainstem, and electrodiagnostic studies suggested an axonal neuropathy. Autopsy revealed a flattened small-bowel mucosa with intraepithelial lymphocytosis, a spectrum of degenerative changes of the intra-abdominal and mediastinal lymph nodes, including cavitary degeneration, and splenomegaly. Histologically, the lymph nodes showed pseudocyst formation and lymphocytic vasculitis with fibrinoid necrosis, and sections of the brain exhibited fibrinoid degeneration of small blood vessels, sparse perivascular lymphocytic infiltrates, and perivascular ischemic lesions. Identical T-cell clones were identified in the duodenum, stomach, lymph nodes, and spleen. This patient had an unusual neurological disorder related to a vasculopathy, probably mediated by a circulating neoplastic clone of activated T cells.

15 citations


Journal ArticleDOI
TL;DR: Analysis of microscopic histopathological changes in cardiovascular tissues in DBA/2 mice suggests that CAWS-induced arteritis is a good model of not only Kawasaki disease but also other cardiovascular diseases such as valvular endocarditis.
Abstract: CAWS, a water-soluble extracellular polysaccharide fraction obtained from the culture supernatant of Candida albicans, is one of the fungal pathogen-associated molecular patterns (PAMPs) It has been reported to show potent activity inducing arteritis and coronaritis in mice Especially, CAWS-induced arteritis has a 100% incidence and severe mortality in the DBA/2 mouse strain This artificial vasculitis was reported to provide a good murine model of Kawasaki disease and other inflammatory vascular disease However, severe mortality was observed only in DBA/2 mice, which is a CAWS-sensitive strain In this study, to clarify the mechanisms of CAWS-induced arteritis and mortality, we investigated microscopic histopathological changes in cardiovascular tissues in DBA/2 mice Severe inflammatory infiltration was observed from the external elastic lamina in the aorta and proximal coronary arteries within 1 week after CAWS administration Severe stenosis of the aorta and coronary arteries was observed more than 3 weeks after CAWS administration Fibrinoid necrosis was observed in these vessel walls All CAWS-treated mice died between the fifth and twelfth week after administration Severe inflammatory change with aortic valve transformation suggested that CAWS-treated mice died of valvular endocarditis or cardiac dysfunction Based on the simple induction method and complete incidence, these data suggest that CAWS-induced arteritis is a good model of not only Kawasaki disease but also other cardiovascular diseases such as valvular endocarditis

14 citations


Journal ArticleDOI
TL;DR: When low-dose aspirin is taken, starting at the beginning of pregnancy in patients with poor obstetric history, there are still high frequencies of uteroplacental vascular and related villous lesions persisted on placental bed and it has no beneficial effects on perinatal outcomes in these patients.
Abstract: Objective: The aim of this study was to compare a low-dose aspirin treatment on placental and perinatal effects in the patients with poor obstetric history such as preeclampsia, intrauterine growth retardation (IUGR) in previous pregnancy. Study design: This retrospective study of 86 pregnant women was conducted between April 2002 and June 2005. In this study period 364 placentas were examined and the patients with poor obstetric history such as IUGR and preeclampsia were selected. Then the patients were assigned to three groups; group 1 (n = 30) was composed of women with no risk in previous pregnancy; group 2 (n = 27) was composed of patients with poor obstetric history (e.g., preeclampsia, IUGR) who were treated with aspirin and patients in group 3 (n = 29) had poor obstetric history without any treatment (patients who were started to follow-up after 14 weeks of gestation). Patients in group 2 were treated with a low-dose aspirin (80 mg/day) as soon as a urinary pregnancy test was positive. Treatment was usually stopped at 34 completed weeks of gestation. On histopathologic examination of the placenta, uteroplacental vascular pathologic features and secondary villous damage (such as fibrinoid necrosis of desidual vessels, villous infarct, severely increased villous fibrosis, severely increased syncytiotrophoblast knotting, obliteration of the vessel lumen, severely increased villous hypervascularity) and also lesions involving coagulation (such as excessive perivillous fibrin deposition, multiple occlusive thrombi in uteroplacental vessels, avascular villi ) were examined. Results: There were no significant differences between the groups with respect to maternal age, body mass index at the first trimester and delivery. Also there were no significant differences among groups with respect to placental weight, fetal height, weight, gestational week, umbilical artery pH, pO2, pCO2 and base excess status. The incidences of preeclampsia were 3.3, 7.4, 6.8% and the incidences of IUGR were 6.7, 11.1, 6.8% in the groups, respectively (P > 0.05 for both). Although the percentages of all pathologic findings were higher in groups 2 and 3, these differences were not statistically important. Conclusion: When low-dose aspirin is taken, starting at the beginning of pregnancy in patients with poor obstetric history, there are still high frequencies of uteroplacental vascular and related villous lesions persisted on placental bed. Also it has no beneficial effects on perinatal outcomes in these patients.

13 citations


Journal ArticleDOI
TL;DR: The main diagnostic challenge is the differentiation of RG from granuloma anulare, especially if clinical information on the site of removal, known diseases, duration of illness, medication and existing American College of Rheumatology (ACR) criteria are missing.
Abstract: Rheumatoid granuloma (RG) is histomorphologically defined as a subcutaneous palisading granuloma with central fibrinoid necrosis. Clinically, it presents as a nodule typically localized at pressure points near the joints. From the rheumatic pathological point of view, the main diagnostic challenge is the differentiation of RG from granuloma anulare, especially if clinical information on the site of removal, known diseases, duration of illness, medication and existing American College of Rheumatology (ACR) criteria are missing. Other granulomatous lesions, such as mycobacterial infections, foreign body granulomas, necrobiosis lipoidica or sarcoidosis, can be differentiated from RG by histopathological criteria or by additional examinations such as pathogen specification or PCR. An immunohistochemical marker for the differential diagnosis of granulomas is not yet available. Diagnosis is based on conventional H-E staining, alcian blue-PAS staining, polarizing analysis or PCR. In the following article, the most important granulomatous entities in the differential diagnosis of RG are introduced and the main diagnostic characteristics are discussed.

9 citations


Journal ArticleDOI
TL;DR: The clinical course and sonographic characteristics of evolving necrotizing vasculitis of the testis are described, the literature is reviewed, and the implications for the diagnosis and management of this condition are discussed.
Abstract: The presentation of necrotizing vasculitis of the testis is variable and may mimic a testicular tumor. Necrotizing vasculitis of the testis is characterized by fibrinoid necrosis of the walls of small and medium-sized testicular arteries. We report a patient with isolated necrotizing vasculitis of the testis who presented with clinical imaging findings suspicious for testicular neoplasm. We describe the clinical course and sonographic characteristics of evolving necrotizing vasculitis of the testis, briefly review the literature, and discuss the implications for the diagnosis and management of this condition. © 2006 Wiley Periodicals, Inc. J Clin Ultrasound 34:99–107, 2006

Journal ArticleDOI
TL;DR: Lesions of subacute necrotising vasculitis were identified in all the tissues examined, but were most severe in the kidney, and a positive ELISA result for alpha toxin and negative ELISA results for beta and epsilon toxins were given.
Abstract: tion. A sample of small intestinal contents was collected to test for Clostridium perfringens exotoxins by ELISA (Naylor and others 1987, 1997, Martin and others 1988). A sample of kidney was collected for lead analysis by flame atomic absorption spectroscopy. Samples of kidney, liver, lung, cerebral cortex, pons, medulla and cerebellum were fixed in 10 per cent neutral buffered formalin, dehydrated using increasing concentrations of alcohol, cleared with xylene and embedded in paraffin wax. Sections of 4 μm thickness were cut and stained with haematoxylin and eosin for histopathological examination. No bacteria were isolated from the brain or lung. Examination of the small intestinal contents for C perfringens exotoxins gave a positive ELISA result for alpha toxin and negative ELISA results for beta and epsilon toxins. Lead was not detected in the kidney sample. At histopathological examination, lesions of subacute necrotising vasculitis were identified in all the tissues examined, but were most severe in the kidney. Vasculitis was characterised by infiltration of the blood vessel walls, mainly by mononuclear cells (Figs 1, 2), with fibrinoid necrosis of some affected vessels. Moderate interstitial mononuclear nephritis, with hyaline material in the tubule lumina and Bowman’s capsules, were found in kidney samples. All levels of the brain examined revealed perivascular cuffing with mononuclear cells, focal gliosis and mild non-suppurative meningitis. In the liver, increased mononuclear cellularity within portal areas was evident, and in the lung extensive Multisystemic necrotising vasculitis in a pygmy goat (Capra hircus)

Book ChapterDOI
01 Jan 2006
TL;DR: The American College of Rheumatology has proposed criteria for the diagnosis of hypersensitivity vasculitis, which corresponds to CSVV, and the histopathologic pattern is that of a leukocytoclastic Vasculitis—angiocentric, segmental inflammation with nuclear dust, endothelial-cell swelling, and fibrinoid necrosis.
Abstract: Publisher Summary Vasculitis refers to inflammation and necrosis of blood vessels, whether they are arteries, veins or both. It can be local or systemic, and may be primary or secondary to another disease process. The classic cutaneous manifestation of vasculitis is palpable purpura; however, the clinical manifestation greatly depends on the size of the vessel affected. Leukocytoclastic vasculitis is a pathological description used to describe inflammation of post-capillary venules and is clinically more correctly termed “cutaneous small vessel vasculitis (CSVV).” CSVV occurs equally in both sexes and at all ages. It is estimated that 10% of affected patients are children. The histopathologic pattern is that of a leukocytoclastic vasculitis—angiocentric, segmental inflammation with nuclear dust, endothelial-cell swelling, and fibrinoid necrosis. The American College of Rheumatology (ACR) has proposed criteria for the diagnosis of hypersensitivity vasculitis, which corresponds to CSVV.

Journal ArticleDOI
TL;DR: A 700-pound, 9-month-old Angus heifer from a feedlot presented with acute neurologic signs, characterized by circling, posterior weakness, and nonresponsiveness, followed by death, which is the first known case report of V. cholerae meningoencephalitis and cerebral abscessation in a bovine.
Abstract: A 700-pound, 9-month-old Angus heifer from a feedlot presented with acute neurologic signs, characterized by circling, posterior weakness, and nonresponsiveness, followed by death. Histologically, the frontal lobe and the thalamus contained multiple foci of liquefaction that contained numerous degenerative neutrophils and foamy macrophages. Some of these foci were centered on blood vessels that contained fibrin thrombi and exhibited varying degrees of fibrinoid necrosis of the vessel wall. There was adjacent axonal degeneration and neuronal necrosis characterized by pronounced cytoplasmic eosinophilia, peripheralization of the nuclei, and loss of Nissl substance. Aerobic culture of the brain yielded moderate growth of Vibrio species, which was determined to be Vibrio cholerae by polymerase chain reaction analysis of a 438-base pair fragment of the 16 S ribosomal RNA gene. V. cholerae are motile, gram-negative, curved rod-shaped bacteria. Some strains of V. cholerae are important food- and water-borne bacterial pathogens that produce an often fatal diarrhea in humans. This is the first known case report of V. cholerae meningoencephalitis and cerebral abscessation in a bovine.

Journal ArticleDOI
TL;DR: R-ACS in pregnancy may be associated with decreased placental growth in a dose dependent fashion, but did not lead to evident differences in histologic markers of placental inflammation, infarction, or necrosis.

Journal ArticleDOI
TL;DR: This is the first report of a renal crisis in a Korean SSc patient with RNAP I/III antibodies, and subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis.
Abstract: Scleroderma (SSc) renal crisis has been reported to be associated with anti-RNA polymerase I and III (RNAP I/III) antibodies in Caucasians and the Japanese. However, no report is available for Korean SSc patients. Here, we describe the case of a 65-yr-old female SSc patient who developed renal crisis and whose serum contained anti-RNAP I/III antibodies. She was finally diagnosed as having diffuse cutaneous SSc based on skin thickening proximal to the elbows and knees. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Despite the use of captopril and adequate blood pressure control, her renal function deteriorated. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. Serum anti-RNAP I/III antibodies were detected by radioimmunoprecipitation. This is the first report of a renal crisis in a Korean SSc patient with RNAP I/III antibodies.