1/113. Pneumatosis intestinalis in association with connective tissue disease.Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/113. November 1998--70 year old woman with SLE, paraproteinemia and polyneuropathy.A 70 year old woman developed a sensory-motor polyneuropathy 12 years after almost continuous treatment with hydrochloroquine for SLE. sural nerve biopsy disclosed abundant lipid inclusions in schwann cells and vessels against a background of axonal degeneration. After cessation of therapy the patient improved moderately. This case underscores the usefulness of nerve biopsy in the study of management of patients with peripheral neuropathy associated with systemic illnesses.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/113. Vertebrobasilar artery territory infarction as an initial manifestation of systemic lupus erythematosus.cerebral infarction is a well-documented complication of systemic lupus erythematosus (SLE), that usually occurs several years after the diagnosis of SLE. To our knowledge, however, strokes associated with vertebrobasilar artery involvement were not reported to present as an initial manifestation of SLE. We report two patients, who presented with vertebrobasilar territory infarction as an initial manifestation of SLE. Patient 1 was a 16-year-old girl, who developed dysarthria and ataxia. MRI showed multiple infarcts in the pons, cerebellum and thalamus. Four-vessel cerebral angiography showed multifocal stenoses in the vertebral and basilar arteries with beaded appearance. Patient 2 was a 26-year-old woman, who developed headache associated with dysarthria, dizziness and ataxia. MRI showed multiple infarcts in the cerebellum, medulla, pons, midbrain and thalamus. cerebral angiography revealed occlusion of both vertebral arteries at the first cervical vertebral level with non-visualization of the basilar artery. Both patients were diagnosed as having SLE supported by laboratory results. Although rare, posterior circulation stroke can present as an initial manifestation of SLE, which may be attributed to vasculitis or dissection in the vertebral/basilar artery.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/113. Vasculitis-induced membranous fat necrosis.Membranous fat necrosis (MFN) a distinct degenerative process of adipose tissue, is characterised by the presence of membranocystic lesions (MCLs) superimposed on a background of typical traumatic-type fat necrosis. MCLs are cysts, of varying size and shape, that are lined by an eosinophilic, crenulated membrane, having the staining properties of ceroid. Although MFN has been documented in varying systemic adipose tissue sites and in tumours, the pathogenesis of this pathological curiosity is unknown. To date, an ischemic basis for MFN has been the most proximate, and atherosclerosis and venous insufficiency, due to large and medium vessel disease, have been the most popular underlying clinical disorders. Although systemic vasculitis has been quoted as the underlying ischemic disorder in some patients, vasculitis has not been commented on nor demonstrated in tissue sections in association with MFN. In,reporting vasculitis-induced MFN, we document the occurrence of MFN in association with uncommon causes of vasculitis, namely: 1) Granulomatous vasculitis in a post-herpetic zosteriform scar; 2) cytomegalovirus-induced vasculitis in the clinical setting of systemic lupus erythematosus; and 3) Lymphocytic vasculitis in a tetanus toxoid immunization site reaction.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/113. Idiopathic portal hypertension associated with systemic lupus erythematosus.A case of idiopathic portal hypertension (IPH) associated with systemic lupus erythematosus (SLE) is reported in a 38-year-old man who had been diagnosed with SLE and treated for 18 years. Esophageal varices. found in 1994 on endoscopic examination, had been followed up for 2 years. On July 16, 1996, he was admitted to Nagoya University Hospital because there was a high risk of bleeding from the esophageal varices due to severe thrombocytopenia. As partial splenic embolization had temporarily controlled the thrombocytopenia, splenectomy and devascularization of the stomach vessels were performed after endoscopic ligation of the esophageal varices. Histological specimens of wedge biopsied liver showed chronic inactive hepatitis without cirrhosis. The presence of anticardiolipin antibody, indicated by positivity for lupus anticoagulant, was suggestive of the presence of a common immunological mechanism in the etiology of SLE and IPH.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/113. Acute mitral regurgitation from ruptured chordae tendineae in systemic lupus erythematosus.A patient with systemic lupus erythematosus developed acute mitral regurgitation due to ruptured chordae tendineae, requiring mitral valve replacement. Typical changes of Libman-Sacks endocarditis were observed in the excised mitral valve. Immunofluorescent studies revealed antinuclear antibody and deposits of immunoglobulins and complement within small vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/113. Protein-losing enteropathy associated with hypocomplementemia and anti-nuclear antibodies.We report a case of protein-losing enteropathy associated with an autoimmune disorder, presumably systemic lupus erythematosus. Although typical manifestations of systemic lupus erythematosus were lacking, a high serum cholesterol level, a low serum complement level, positivity for anti-nuclear antibody, and positivity for anti-single-strand dna antibody suggested an autoimmune mechanism as the cause of the condition. Although immunohistological examination of duodenal and ileal biopsy specimens failed to reveal deposits of immune complex or complement in the vessels, capillary hyperpermeability was suspected as the mechanism of the condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/113. Interstitial cystitis and ileus in pediatric-onset systemic lupus erythematosus.A girl aged 11 years presented with autoimmune hemolytic anemia with thrombocytopenia, and subsequently developed severe abdominal pain, vomiting, and pollakiuria. X-ray findings of her abdomen demonstrated paralytic ileus with intestinal wall thickening. Intravenous pyelography revealed bilateral hydroureter with mild hydronephrosis and contracted bladder. Pathological examination of her bladder revealed interstitial cystitis, with evidence of focal deposition of IgG and C3 in a granular pattern on small blood vessel walls. She was diagnosed as having systemic lupus erythematosus (SLE) associated with paralytic ileus and chronic interstitial cystitis. Although initiation of high-dose prednisolone therapy resulted in a gradual improvement in clinical symptoms, reducing the dosage of prednisolone caused a relapse. To our knowledge, the combination of paralytic ileus and chronic interstitial cystitis is quite uncommon in pediatric-onset SLE.- - - - - - - - - - ranking = 4.3686546018305keywords = blood vessel, vessel (Clic here for more details about this article) |
9/113. Small vessel thrombosis without major thrombotic events in systemic lupus erythematosus patients with antiphospholipid syndrome.antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
10/113. role of lipids in the progression of renal disease in systemic lupus erythematosus patients.Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease marked by immune-complex mediated lesions in small blood vessels of various organs, especially the kidneys, although other factors may also be implicated in the pathogenesis of the disease. This article focuses on the role of lipids in the progression of glomerular, vascular and tubulo-interstitial lesions in two patients with lupus nephritis associated with pronounced hyper- and dyslipidemia. The pathogenesis of progressive glomerulosclerosis in both patients appears to be multifactorial. In addition to immune complex mediated lupus glomerulonephritis, progressively active in the first patient, severe nephrotic-range persistent proteinuria, arterial hypertension associated with hyperfiltration and hyperperfusion injuries and, to a minor extent, hyper- and dyslipidemia were observed. Immunological and non-immunological factors were shown to contribute to the development of tubulo-interstitial lesions. In both patients, in addition to local immune deposits, prominent tubulo-interstitial lipid deposits were probably causally related to both hyperlipidemia and the increased permeability of the glomerular filtration barrier. Tubular lesions were highlighted by intracytoplasmic lipid droplets as well as small cleft-like spaces found to be impacted in the tubular lumina. They were seen to penetrate tubular epithelial cells and eventually lodge in the interstitium, surrounded by mononuclear cell infiltrates and foam cells. In both patients, hypertensive angiopathy and extraglomerular vascular immune deposits were demonstrated. In addition, in the second patient, arteriolar and small arterial hyaline was found at the age of 28 years to be full of lipids and calcium precipitates, suggesting a peripheral atherosclerosis-like process which never occurs as a natural age-related condition. In conclusion, all parts of the nephron may be involved in the pathogenetic process causally related or influenced by hyper- or dyslipidemia. Associated either with endothelial cell injury and consequent insudation of lipids in the vascular walls, glomerular filtration barrier injury with hyperfiltration, or tubulo-interstitial lipid deposition, the mechanism of tissue damage by lipids in all parts of the nephron shares similarities with the pathogenesis of systemic atherosclerosis.- - - - - - - - - - ranking = 4.3686546018305keywords = blood vessel, vessel (Clic here for more details about this article) |
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