1/45. 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) |
2/45. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study.We report a 54-year-old male, with a 5-year history of spreading asymptomatic generalized cutaneous telangiectases. The patient had no mucosal or nail involvement, no positive family history and no clinical evidence of systemic disease or bleeding diathesis. Histologically, the superficial small dermal blood vessels were dilated and showed thickened walls with hyaline perivascular material, staining as collagen. The vessel walls were PAS and colloidal iron stain positive, and immuno-histochemically lacked actin staining. collagen IV, fibronectin and laminin antibodies showed the material deposited around the basement membrane zone. Ultrastructurally, the vessels were post-capillary venules (PCV) and showed marked collagen deposition around the basal lamina. There were many abnormally banded widely spaced fibres with 100-150 nm periodicity (Luse bodies), in addition to regular banded collagen. pericytes were sparse and lacked intracytoplasmic filaments, and few veil or fibroblastic cells were seen embedded within the collagen. We believe this is a form of cutaneous microangiopathy not previously described, with distinct morphology and unique ultrastructural features. It may be due to a genetic defect with erroneous production of disorganized collagen in the cutaneous microvasculature. Dermatologists and Dermatopathologists should be aware of this unusual cutaneous vasculopathy.- - - - - - - - - - ranking = 0.48862187915537keywords = microangiopathy (Clic here for more details about this article) |
3/45. 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) |
4/45. 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) |
5/45. 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) |
6/45. Retinal angiopathy and polypoidal choroidal vasculopathy.PURPOSE: To describe the clinical and angiographic features of patients with polypoidal choroidal vasculopathy, exudative detachment of the macula, and an associated retinal microangiopathy. methods: Case series. RESULTS: Four patients with chronic exudative detachment of the macula with a variable degree of lipid deposition are described. The retina in the detached area, but not beyond, was noted to have a microangiopathy. There was capillary telangiectasia, microaneurysm formation, patchy nonperfusion, and intraretinal leakage. In each patient, there were no other retinal vascular changes in the fundus of either eye. The fluorescein angiogram showed subretinal leakage suspicious for occult choroidal neovascularization. The indocyanine green angiogram showed the presence of underlying polypoidal choroidal neovascularization, accounting for the exudative detachment. After photocoagulation, the retinal angiopathy improved, but not completely. CONCLUSION: Retinal microangiopathy may occur in a chronic macular detachment secondary to polypoidal choroidal neovascularization. The development of these secondary retinal changes is not clearly understood; however, hypoxia from the chronic detachment, a neurotoxic effect from the lipid deposition, or a biochemically induced microvascular abnormality from secretion of vasogenic mediators are possible mechanisms. indocyanine green angiography is helpful in making a definitive diagnosis. Clinicians should be aware that a retinal microangiopathy may occur in such eyes so that the proper diagnosis can be made and appropriate treatment administered.- - - - - - - - - - ranking = 1.9544875166215keywords = microangiopathy (Clic here for more details about this article) |
7/45. MPO-ANCA-associated pulmonary-renal vasculitis in a patient with diabetes mellitus.diabetes mellitus is one of the major causes of chronic renal failure. Typical findings of diabetic nephropathy are early hyperfiltration followed by microalbuminuria and overt proteinuria, resulting in a progressive decrease in glomerular filtration rate. Rapidly progressive glomerulonephritis has rarely been reported in patients with diabetes mellitus. Here, we describe a patient with MPO-ANCA-associated vasculitis, presenting with pulmonary-renal syndrome. Immunosuppressive treatment, including pulse methyl-prednisolone and cyclophosphamide, was administered and the disease was resolved.- - - - - - - - - - ranking = 0.033838695570473keywords = diabetic (Clic here for more details about this article) |
8/45. Perivascular inflammatory reaction to a Hemobahn stent-graft: diagnosis with 3D MR angiography.PURPOSE: To present a rare perivascular inflammatory reaction to a commercially produced polytetrafluoroethylene-covered stent and demonstrate the utility of 3-dimensional (3D) magnetic resonance angiography (MRA) in the diagnosis of this phenomenon. CASE REPORT: Three weeks after percutaneous deployment of a Hemobahn stent-graft to treat a high-grade stenosis and aneurysm of the proximal left superficial femoral artery (SFA), a 70-year-old diabetic man developed fever, pain, and local swelling of the left thigh. venous thrombosis was excluded by ultrasound imaging; a normal flow profile was seen in the left common and superficial femoral arteries. Blood analysis found elevated inflammatory markers. MRA revealed extensive soft-tissue edema and perivascular contrast enhancement around the left SFA, but the stent-graft was patent. The clinical symptoms resolved within 7 days after initiation of anti-inflammatory therapy. Follow-up MRA scans demonstrated significantly reduced inflammatory reaction over the next few months. CONCLUSIONS: Symptomatic perivascular inflammatory soft-tissue response to a stent-graft can be diagnosed with MR imaging studies.- - - - - - - - - - ranking = 0.033838695570473keywords = diabetic (Clic here for more details about this article) |
9/45. 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) |
10/45. 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) |
| | Next -> |