1/33. Cogan's syndrome: unsuccessful outcome with early combination therapy.Interstitial keratitis and vestibuloauditory symptoms (vertigo and hearing loss) are the typical signs of Cogan's syndrome, a rare inflammatory vascular disease. Signs of vasculitis in many organ systems may appear, among which neurologic problems are sometime predominant. The efficacy of glucocorticoids on the ocular and systemic symptoms is established, but their effect on hearing loss is unknown. We describe a case of Cogan's syndrome with neurological involvement in which early treatment with combination therapy (prednisolone and cyclosporin) failed to bring ear inflammation under control.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/33. Acute blast crisis with EBV-infected blasts, in a patient with chronic myeloid leukemia, and vasculitis.Unless they undergo transplantation, all patients with chronic myeloid leukemia (CML) will eventually develop a late phase of acute blast crisis (ABC). Although additional chromosomal abnormalities to the philadelphia (Ph) chromosome may herald ABC in many CML cases, the mechanisms leading to this fatal event are obscure. Viral etiology, including the Epstein-Barr virus (EBV) has never been implicated in the pathogenesis of ABC in CML. iloprost is an analogue of epoprostenol (prostacyclin; PGI2) commonly used for the treatment of peripheral vascular diseases and acts via inhibition of platelet activation, and by vasodilation. A case of ABC with blasts of undetermined lineage showing EBV infection in a male patient with Ph positive CML is described here. This unusual event developed during a course of treatment with the prostacyclin analogue, iloprost administered for vasculopathic leg ulcers. The proliferating blasts stained positively by immunohistochemistry only for the leukocyte common antigen (LCA/CD-45), and the EBV-latent membrane protein 1 (LMP-1). The only chromosomal abnormality detected by cytogenetic analysis was the conventional Ph-chromosome. It is suggested that ABC in this case of CML, was associated with EBV-activated blasts of undetermined lineage.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/33. Acute cytomegalovirus infection complicated by vascular thrombosis: a case report.We present a case report of a previously healthy adult with cytomegalovirus infection that was complicated by extensive mesenteric arterial and venous thrombosis. To our knowledge, this is the first reported case of this syndrome in an immunocompetent individual who had no predisposing risk factors for thrombosis, and it demonstrates the propensity for cytomegalovirus to be involved in vascular disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/33. Three-dimensional volume-rendering CT angiography in vasculitis: spectrum of disease and clinical utility.Spiral computed tomographic angiography (CTA) coupled with three-dimensional volume-rendering image processing is a less invasive alternative to conventional catheter angiography. The technique has been used successfully in a variety of vascular diseases. In this pictorial essay, we review the CTA findings in selected cases of vasculitis. Technical considerations and the potential clinical value of this method are discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/33. Cardiovascular calcifications in pediatric patients receiving maintenance dialysis.Cardiovascular disease is a major cause of morbidity and mortality in adult patients with end-stage renal disease receiving maintenance dialysis. Coronary artery calcifications (CAC) contribute to the high prevalence of cardiac disease and are associated with hyperphosphatemia, an elevated calcium-phosphorus product (CaxP), and prolonged time on dialysis. Chronic inflammation and malnutrition are also associated with an increased risk for development of cardiac calcifications. Young adults receiving maintenance dialysis develop cardiac calcifications at a degree out of proportion to healthy adults of the same age and gender. Many of these young adults initiated dialysis as children or teenagers. risk factors associated with the development of CAC are also seen in the pediatric dialysis population. To date, reports of cardiac calcifications in pediatric patients receiving maintenance dialysis are limited to post-mortem studies. We present two pediatric patients with ANCA-positive vasculitis diagnosed with cardiac calcifications while receiving maintenance dialysis. hyperphosphatemia and an elevated CaxP product were seen in both patients and probably contributed to the development of extraskeletal calcifications. In addition, both patients had an underlying systemic inflammatory disease and significant weight loss/malnutrition that may have contributed to the early and rapid onset of cardiac calcifications.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/33. hiv and vasculitis: case report.Manifestation of vasculitis and hiv is now being increasingly being observed. However, clinicians need to be conscious of the possible direct association of this virus with vascular disease. It would appear that the nervous and musculoskeletal systems are most commonly affected. No systematic data exist on the benefit of pharmacological agents currently used and there is an axiomatic need to study these agents in terms of the risk/benefit.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/33. A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease.A previously healthy 14-year-old Korean male presented with striking hemorrhagic acral livedo associated with anti-ribonucleoprotein antibodies. His skin biopsy revealed a striking lymphocytic vascular reaction with mild superficial perivascular lymphocytic infiltrates, perivascular extravasation of red blood cells, and leukocytoclastic debris. Lymphocytic vasculitis (LV) is defined in different ways by different authors. The above biopsy findings do not fulfill the criteria for LV of all authors. Chronic lymphocyte-mediated endothelial cell injury may play an important role in the pathogenesis of collagen vascular disease. Cell-mediated cytotoxicity against endothelial cells or other vessel wall components is thought to be the possible pathologic mechanism of LV. We present a patient with early undifferentiated connective tissue disease (EUCTD) with hemorrhagic acral livedo, who finally developed systemic lupus erythematosus. Even though the histopathologic findings do not meet the most rigorous definition of LV, LV may a presenting sign of EUCTD. The purpose of this report is to suggest reconsideration of the diagnostic criteria of lymphocytic vasculitis.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/33. asbestosis and probable microscopic polyangiitis.Several inorganic dust lung diseases (pneumoconioses) are associated with autoimmune diseases. Although autoimmune serological abnormalities are common in asbestosis, clinical autoimmune/collagen vascular diseases are not commonly reported. A case of pulmonary asbestosis complicated by perinuclear-antineutrophil cytoplasmic antibody (myeloperoxidase) positive probable microscopic polyangiitis (glomerulonephritis, pericarditis, alveolitis, multineuritis multiplex) is described and the possible immunological mechanisms whereby asbestosis fibres might be relevant in induction of antineutrophil cytoplasmic antibodies are reviewed in the present report.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
9/33. vasculitis in the antiphospholipid syndrome. A cause of ischemia responding to corticosteroids.A 63-year-old woman with atherosclerotic peripheral vascular disease and the lupus anticoagulant developed ischemia of the right lower extremity, requiring progressive amputations. Pathologic specimens revealed inflammatory vasculitis in multiple arteries. Her serum showed anticardiolipin antibodies in high titer. Treatment with high-dose corticosteroids reversed the ischemic process. In patients with antiphospholipid antibodies, thrombosis is the most common pathologic finding associated with cutaneous lesions and/or gangrene. vasculitis, although uncommon, is known to occur and may respond to corticosteroid therapy.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/33. Combination of massive mucinosis, dermatomyositis, pyoderma gangrenosum-like ulcer, bullae and fatal intestinal vasculopathy in a young female.Cutaneous mucinosis secondary to autoimmune collagen vascular disease is well recognized, but manifestation as cellulitis-like massive cutaneous mucinosis preceding dermatomyositis is unusual. Here we report a 21-year-old Taiwanese woman with a large, rapid onset, painful erythematous, edematous plaque, which histopathologically revealed septal panniculitis with fat necrosis and massive mucin deposition. Incapacitated muscle weakness of proximal extremities, generalized edema, heliotrope erythema, and Gottron's papules developed in a short period of time with high titers of serum muscle enzyme. Serological titers of ANA, anti-dsDNA, anti-ENA panels, and erythrocyte sedimentation rate, however, all showed unremarkable results. diagnosis of dermatomyositis was confirmed by electromyographic findings of myopathy. As the disease progressed, large, deep cutaneous ulceration and vesiculobullous lesions also developed. In spite of aggressive treatment, the patient died 9 months after the disease onset, probably due to the complication of gastrointestinal ischemia and perforation.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
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