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Vasculitis
Kinds of Vasculitis Selected AbstractsPARANEOPLASTIC VASCULITIS AND COEXISTENT TROUSSEAU'S SYNDROME SECONDARY TO PANCREATIC CARCINOMAJOURNAL OF AMERICAN GERIATRICS SOCIETY, Issue 9 2006Radcliffe Lisk MRCP No abstract is available for this article. [source] Urticaria Vasculitis Induced by Diode Laser Photo-EpilationDERMATOLOGIC SURGERY, Issue 11 2000G.A. Moreno-Arias MD [source] Necrotizing Vasculitis: A Cause of Aortic Insufficiency and Conduction System DisturbanceECHOCARDIOGRAPHY, Issue 7 2003Miquel Gómez Pérez M.D. Cardiac involvement in vasculitis syndromes is uncommon. We describe a 50-year-old male who presented with progressive dyspnea and myalgies. Echocardiogram revealed significant thickening of aortic root, aortic cusps, and anterior mitral valve leaflet, with severe aortic regurgitation that required aortic valve replacement. Furthermore, this patient suffered progressive atrioventricular block that needed implantation of a pacemaker. The study performed disclosed the presence of necrotizing vasculitis positive for perinuclear antineutrophil cytoplasmic antibody. (ECHOCARDIOGRAPHY, Volume 20, October 2003) [source] Cutaneous sarcoid-like granulomas with alveolar hemorrhage and c-ANCA PR-3INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 9 2004Natividade Rocha MD A 28-year-old woman, employed as a leather factory worker, noted asymptomatic, well-delimited plaques on both knees, 6 years ago. The plaques were violaceous with a smooth surface. One appeared over a post-traumatic scar from childhood (Fig. 1). Two years later, she began to complain of symptoms suggestive of polyarthritis, first of the small joints of the hands (proximal interphalanges) and then of the larger joints (wrists, elbows, and knees). She was diagnosed with rheumatoid arthritis and began treatment with nonsteroidal anti-inflammatory drugs for 1 month without any change. Deflazacort, 12 mg/day, and hydroxychloroquine, 400 mg/day, were administered for 3 months, with improvement of her articular complaints, but not her skin lesions. Figure 1. Well-delimited, violaceous plaques with a smooth surface on the knees, one over an old post-traumatic scar One year later, she complained of dysphonia, which remitted spontaneously after some weeks. After one additional year, she noted papules, with similar characteristics to the plaques, on the elbows, and two well-delimited orange-to-brown plaques on the forehead (Fig. 2). Figure 2. Orange,brown plaques symmetrically placed on the forehead During the fifth year of the disease, she was referred for the first time to a dermatologist, who biopsied one of the knee lesions. The histologic result was compatible with "sarcoid granuloma." At that time, she presented with skin lesions as her only complaint. Sarcoidosis was suspected based on a chest X-ray, which revealed hilar lymphadenopathy and diffuse accentuation of the interstitium. In November 2000, she suddenly developed fever (40 °C), cough with hemoptysis, dysphonia, and subcutaneous nodules on the palmar surface of the fingers of both hands that were painless, well-delimited, 5 mm in diameter, and firm (Fig. 3). She reported a weight loss of 12 kg in the previous 3 months. Pulmonary condensation was found on auscultation, and she had palpable hepatomegaly. Peripheral lymphadenopathy was not present. Figure 3. Painless, well-delimited, firm subcutaneous nodules on the palmar surface of the fingers Laboratory investigations revealed normochromic, normocytic anemia (hemoglobin, 7.7 g/dL), iron deficit, a white blood cell count of 16,000/µL with neutrophilia, an erythrocyte sedimentation rate of 130 mm/h, elevation of liver enzymes, a slight increase in angiotensin-converting enzyme (ACE) level (72 U/L), hypergammaglobulinemia (IgG, 3350 mg/dL), antinuclear antibody (ANA) of 1 : 320, and a slight increase in CD4 and decrease in CD8 lymphocytes with normal cellular morphology in blood. Renal function, urine sediment, urine and serum calcium, complement (C4), dsDNA, antimitochondrial antibody, direct and indirect Coombs test, antineutrophil cytoplasmic antibody (ANCA), tuberculin skin tests, viral markers of hepatitis B, C, and human immunodeficiency virus (HIV), electrocardiogram (ECG), ophthalmic examinations, and culture for infectious agents in blood and sputum were all normal or negative. Computed tomography (CT) scan showed an infiltrate in the upper right pulmonary lobule with a central cavity and bilateral hilar lymphadenopathy (Fig. 4). Homogeneous hepatosplenomegaly was present. The bronchoalveolar lavage (BAL) showed a slight lymphocytic increase predominantly of CD8 cells and hemosiderosis. Stains for infectious agents, including acid-fast bacillus, fungi, Mycoplasma, and Legionella, were negative. Three biopsies from the forehead, elbows, and knees showed well-formed noncaseating epithelioid cell granulomas with giant cells of the Langhans type in the dermis, suggestive of sarcoidosis (Figs 5 and 6). A fourth biopsy from a finger nodule demonstrated inflammatory infiltration of the dermis and necrosis with cellular debris. Vasculitis was not seen (Fig. 7). Figure 4. Computed tomography scan showing an infiltrate in the upper right pulmonary lobule with a central cavity Figure 5. Beneath a flattened epidermis, several sarcoid granulomas composed of epithelioid histiocytes and several multinucleated giant cells of Langhans type can be seen (hematoxylin and eosin, ×10) Figure 6. Less well-formed sarcoid granulomas in a hyperkeratotic area, surrounded by a sparse rim of lymphocytes (hematoxylin and eosin, ×20) Figure 7. Foci of necrosis and fibrinoid degeneration with some neutrophil infiltration and nuclear dusting (hematoxylin and eosin, ×40) The patient was treated with a broad-spectrum empirical antimicrobial (levofloxacin, 500 mg daily intravenously) over 12 days, with prompt improvement in her symptoms and remission of the forehead and finger lesions. Nevertheless, on the first evaluation after hospitalization, the CT scan showed persistence of the pulmonary cavity (Fig. 8). A repeat ANCA determination was positive (cytoplasmic pattern, c-ANCA) at 1 : 640 by indirect immunofluorescence (IIF). Antiproteinase-3 antibody was demonstrated at 78 by enzyme-linked immunosorbent assay (ELISA). Figure 8. Computed tomography scan showing persistence of the pulmonary cavity She underwent an open lung biopsy which revealed intra-alveolar hemorrhage and scanty noncaseating epithelioid cell granulomas of the sarcoidosis type in the peripheral blood vessels without vasculitis. A diagnosis of Wegener's granulomatosis was made and she began prednisolone (1 mg/kg/day) and oral cyclophosphamide (2 mg/kg/day). One year later, she is asymptomatic, the skin lesions have completely remitted, c-ANCA is negative, and the CT scan shows partial regression of the pulmonary cavity. [source] Localized Chronic Fibrosing Vasculitis or Localized Erythema Elevatum Diutinum?JOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L. Clarke Localized chronic fibrosing vasculitis is a rare dermatosis that histologically resembles late-stage erythema elevatum diutinum (EED) but has a different clinical presentation. A 62-year-old male presented with bilateral nodules on his heels that first appeared two years ago and over the past six months had become extremely painful. He denied any recent trauma to the sites, and his medical history was significant only for diabetes mellitus, coronary artery disease, and osteoarthritis. Physical exam demonstrated focally ulcerated violet-red three-centimeter nodules on the medial aspects of both heels. Biopsy revealed dense concentric and lamellar fibrosis with foci of leukocytoclastic debris and a sparse infiltrate of histiocytes, neutrophils, eosinophils, and lymphocytes. No granulomas were present, and histochemical stains and tissue cultures for microorganisms were negative. Imaging studies showed no evidence of underlying osteomyelitis, cellulitis, or abscess formation. Laboratory studies demonstrated a markedly elevated IgA level that was shown to be polyclonal on serum immunofixation studies. All peripheral blood cell counts were normal. Thorough evaluations for systemic vasculitides and connective tissue disorders were negative. A diagnosis of localized chronic fibrosing vasculitis was made. This case illustrates the clinicopathologic overlap between this disorder and EED. [source] Intracranial Vasculitis and Multiple Abscesses in a Pregnant WomanJOURNAL OF NEUROIMAGING, Issue 3 2001Mutlu Cihangiroglu ABSTRACT Cerebral vasculitis is an unusual disorder with many causes. Infectious causes of cerebral vasculitis are predominantly bacterial or viral in nature. Purulent bacterial vasculitis is most often a complication of severe bacterial meningitis. The patient is a 25-year-old African American female, 25 weeks pregnant, who presented to the neurology service after a consult and referral from an outside hospital. She had a 1-month history of right sixth nerve palsy. Initial workup included a negative lumber puncture and a noninfused magnetic resonance imaging (MRI). Three days later, the patient developed right-sided migraine headaches and right third nerve palsy. The angiogram revealed diffuse irregularity and narrowing of the petrous, cavernous, and supraclinoid portions of the internal carotid and right middle cerebral arteries. Shortly thereafter, an MRI examination revealed diffuse leptomeningeal enhancement and abscess and a right parietal subdural empyema. Infectious vasculitis secondary to purulent meningitis has a rapidly progressive course and presents with cranial nerve palsy with involvement of the cavernous sinus. Although the association of this disease with pregnancy has not been established, it should be recognized that the early imaging studies may be negative or discordant and follow-up imaging might be necessary. [source] Cerebral and Oculorhinal Manifestations of a Limited Form of Wegener's Granulomatosis With c-ANCA,Associated VasculitisJOURNAL OF NEUROIMAGING, Issue 1 2001Peterus Thajeb MD ABSTRACT The authors report on cerebral and oculorhinal manifestations in a patient with a cytoplasmic pattern of antineutrophil cytoplasmic autoantibody (c-ANCA),associated vasculitis. Recurrent Tolosa-Hunt syndrome, cavernous sinus syndrome, Raeder's paratrigeminal neuralgia, and seizures were the major clinical manifestations. Brain MRI showed localized enhancing lesions initially in the cavernous sinus and later in the convexity pachymeninges. The lesions disappeared following 9 months of oral prednisolone (15 mg/day) and cyclophosphamide (100 mg/day) therapy. The presence of c-ANCA, demonstration of vasculitis, and depositions of immunoglobulin G (IgG) and fibrinogen in the vessel walls of pachymeninges of the patient confirmed an immune-mediated cause of the vasculitis. Cranial pathology without renal and pulmonary involvement suggests a variant of Wegener's granulomatosis, which is called the "limited" form of Wegener's granulomatosis. [source] Systemic Vasculitis with Severe Cutaneous Manifestation as a Suspected Idiosyncratic Hypersensitivity Reaction to Fenbendazole in a CatJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 3 2008S. Jasani First page of article [source] Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitidesRESPIROLOGY, Issue 2 2004Sakae HOMMA Objective: The association of pulmonary fibrosis (PF) with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitides has not been well documented. The aim of this study was to assess the clinicopathological characteristics of PF in patients who tested positive for MPO-ANCA. Methodology: In this study, 31 patients (17 males and 14 females; mean age, 69 years) diagnosed as having PF with positive MPO-ANCA levels ranging from 10 to 840 EU with a mean of 112.5 EU, were evaluated clinicopathologically. Results: Among 31 patients with PF, 22 had underlying systemic diseases such as collagen vascular diseases, while nine had unknown aetiology. Evidence of glomerulonephritis was demonstrated in 14 patients. The clinical features were a history of dry cough and/or fine crackles in all 31 patients. Chest CT scans showed honeycombing in the lung bases in 26 patients. The histopathological features of the diseased lung tissues in all 11 autopsied cases were compatible with the usual interstitial pneumonia (UIP) pattern. Vasculitis was confirmed in bronchial arteries and/or pulmonary arterioles in five patients. The mortality was as high as 13 of the 31 patients. The causes of death were: deterioration of PF in five (two of whom were associated with pulmonary haemorrhage), lung cancer in two, pneumonia in four, and digestive tract bleeding in two. The survival rates in PF with MPO-ANCA-negative collagen vascular diseases, cryptogenic fibrosing alveolitis (CFA), and PF with positive MPO-ANCA, were compared. The 5-year survival rate in PF with positive MPO-ANCA was worse than in PF with MPO-ANCA-negative collagen vascular diseases and was the same for CFA. Conclusion: Although there was no correlation between MPO-ANCA titres and the activity of PF, this study demonstrated that the presence of positive MPO-ANCA was an unfavorable prognostic factor in patients with PF. [source] The Long-Term Prognosis of Renal Transplant in Patients With Systemic VasculitisAMERICAN JOURNAL OF TRANSPLANTATION, Issue 9 2007G. Moroni Little information is available about the long-term outcome of renal transplantation in patients with systemic vasculitis (SV). We compared the outcomes of 19 renal transplant recipients with SV with those of 38 controls matched for time of transplantation, age, gender and source of donor. The mean post-transplant follow-up was 58 ± 57 months for vasculitic patients and 61 ± 49 months for controls. The actuarial 10-year patient survival was 87% in vasculitic patients and 90% in controls, death-censored graft survival were 84% and 100%, respectively. The risks of acute and chronic rejection, and arterial hypertension were not significantly different between the two groups. Infection was significantly more frequent in vasculitic patients (74% vs. 34%; p = 0.01). Seven patients (36.8%) had a recurrence of vasculitis in mean 45 months after renal transplant (0.076/patients/year). After recurrence, one patient had an irreversible humoral rejection, another died from hemophagocytosis and another restarted dialysis 1 year later. Long-term patient and renal allograft survival in vasculitic patients was good. Although graft function recovered in most relapsers after reinforcement of immunosuppression, one patient died and two lost graft function. [source] Pathogenesis of equine herpesvirus-1 infection in the mouse modelAPMIS, Issue 1 2009GEORG GOSZTONYI Equine herpesvirus-1 (EHV-1) is a major equine pathogen causing respiratory diseases, abortions and severe neurological disorders. The basis of neurological disturbances is, as in other organs, infection of endothelial cells, followed by vasculitis, thrombosis and ischaemic damage of the parenchyma. Here, a murine model was used to explore the mechanism of entry to, and spread within the brain, the cell affinity of the agent and the modulating role of the immune defence, which are all factors governing the pathogenesis of the neurological disease. Because controversial views exist about these mechanisms, we undertook a neuropathological study with intranasally infected adult mice. EHV-1 entered the brain through the olfactory neuroepithelium and along the olfactory nerves, and spread transsynaptically in rostro-caudal direction, using olfactory and limbic neuronal networks. Exclusively neurons were infected. The cellular immune reaction exerted a restraining effect on virus dissemination. Following nasal infection, the olfactory route was the major pathway for virus entry and dissemination, involvement of the trigeminal nerve in virus spread seems much less probable. In the adult mouse brain EHV-1 behaves as a typical neurotropic agent, using, similarly to other herpesviruses, the neuronal networks for dissemination. Vasculitis, the predominant type of lesion in natural infection, and endothelial cell positivity for EHV-1 were detectable only in the lung. Thus, this agent exhibits in the mouse a dual affinity: it is neurotropic in the brain, and endotheliotropic in visceral organs. Consideration of pathogenetic aspects of equine and experimental murine EHV-1 infections also helps a better understanding of human herpetic brain disease. [source] Refractory Progression of Coronary Aneurysms, a Case of Delayed Onset Kawasaki Disease as Depicted by Cardiac Computed Tomography AngiographyCONGENITAL HEART DISEASE, Issue 3 2010FACP, Shah Azmoon MD ABSTRACT Background., Kawasaki disease (KD) is an immune-mediated vasculitis of unknown etiology with self-limited clinical course that was first described in 1967 by Dr. Tomisaku Kawasaki. It is a disease of early childhood and rare past late adulthood but one that can have detrimental consequences when there is a delay in diagnosis and treatment. Cardiovascular complications causing increased morbidity and mortality may include coronary artery aneurysms, myocardial infarction, heart failure, arrhythmias, and peripheral artery occlusion. Case Presentation., Here, we present an atypical case of delayed onset KD in a young teenager. DS had visited three different emergency departments during the course of 2 weeks for unrelenting fevers. Despite multiple treatment protocols including immunoglobulin, steroids, and tumor necrosis factor-alpha antagonists, he continued to have progression of cardiovascular complications. While echocardiographic findings were suspicious for cardiac complications, a cardiac computed tomography (CT) angiography was able to clearly distinguish giant coronary aneurysms. Conclusion., Without prompt therapy, fever and manifestations of acute inflammation can last for several weeks to months with increased risk toward complications. The incidence of coronary artery aneurysms has been noted to be 25% in untreated patients with a mortality rate of up to 2%. Using low-dose protocols along with high spatial and temporal resolution of cardiac CT angiography may provide a useful and complimentary imaging modality in accurate diagnosis and follow-up of patients with KD. [source] P43 Acute urticaria to infliximabCONTACT DERMATITIS, Issue 3 2004Ana Giménez-Arnau Infliximab is a chimeric antitumor necrosis factor-alpha monoclonal antibody used to treat Crohn's disease and rheumatoid arthritis. Acute infusion reactions, headache, fever, chills, urticaria and chest pain were seen in 17% of patients with infliximab compared with 7% of those receiving placebo. Other adverse cutaneous reactions are fungal dermatitis, eczema, seborrhoea, hordeolum, bullous eruption, furunculosis, periorbital oedema, hyperkeratosis, rosacea, verruca, skin pigmentation, alopecia, leukocytoclastic vasculitis, lichenoid drug eruption, erythema multiforme, perniosis-like eruption, granuloma annulare and acute folliculitis. Any pathogenic mechanism has been suggested. Patch test with infliximab can induce flare-up of lesions, nausea and malaise and suggest a percutaneous absortion. A sixty years-old man with atopy background and rheumatoid arthritis treated with Remicare®, infliximab who developed a severe acute urticaria with angioedema is presented. The lesions appearance after previous endovenous administrations and the worsening spreading wheals days after the injection clinically suggested an hypersensitivity mechanism. The protocolized study drug hypersensitivity performed showed only the Prick Test positivity with infliximab at 30/60 minutes. Patch test with infliximab was negative and any adverse event was reported. Actually the patient is treated with etanercept and this drug is well tolerated. This result suggested a type I hypersensitivity mediated reaction. Urticaria could be induced as immunologic reaction of the host against the murine part of infliximab, just as it hapens with other antichimeric antibodies. [source] Trigeminal Trophic Syndrome,Report of Four Cases and Review of the LiteratureDERMATOLOGIC SURGERY, Issue 5 2004Parrish Sadeghi MD Background. Trigeminal trophic syndrome is a unilateral, frequently crescent-shaped neurotrophic ulceration of the face occurring after injury to the trigeminal nerve. The appearance of the ulcers resembles other disease entities such as granulomatous disease, neoplasm, vasculitis, infection, and factitial dermatitis. Objectives. The objectives of this study are to increase awareness of this disorder and to emphasize the importance of eliciting a thorough neurologic history when evaluating facial ulcerations. Methods. Four cases are reported and, using MEDLINE, the English and non-English literature from 1982 to 2002 is reviewed. Results. Including this report, there have been 60 cases of trigeminal trophic syndrome reported from 1982 to 2002. The age at presentation ranged from 14 months to 93 years. Time of onset from injury to the trigeminal ganglion or its branches and the development of the ulcers ranged from 2 weeks to 30 years. One-third of the patients had undergone trigeminal nerve ablation for the treatment of trigeminal neuralgia and another third had a history of stroke. Other causes included craniotomy, head trauma, herpes infection. Conclusion. The majority of cases of trigeminal trophic syndrome are associated with a history of stroke or trigeminal nerve ablation. Successful surgical outcome can be achieved if the underlying neurologic pathology is addressed before the reconstructive procedure. [source] Erythema nodosum and erythema induratum (nodular vasculitis): diagnosis and managementDERMATOLOGIC THERAPY, Issue 4 2010Heidi Gilchrist ABSTRACT Erythema nodosum is the most common type of panniculitis; it may be due to a variety of underlying infectious or otherwise antigenic stimuli. The pathogenesis remains to be elucidated, but both neutrophilic inflammation and granulomatous inflammation are implicated. Beyond treating underlying triggers, therapeutic options consist mainly of nonsteroidal anti-inflammatory drugs, symptomatic care, potassium iodide, and colchicine. Erythema induratum (nodular vasculitis) is a related but distinctly different clinicopathologic reaction pattern of the subcutaneous fat. It is classically caused by an antigenic stimulus from Mycobacterium tuberculosis but may be associated with several other underlying disorders. After appropriate antimicrobial treatment in tuberculous cases, therapy for erythema induratum is similar to options for erythema nodosum. [source] Necrotizing vasculitis in a patient affected by autoimmune hyperthyroidism treated with propylthiouracilDERMATOLOGIC THERAPY, Issue 2010Angela Antonucci ABSTRACT Necrotizing vasculitis is a complex phenomenon because of an inflammation of small and larger vessels with polymorph infiltration within the vessel walls and leukocytoclasis, occurring in several autoimmune diseases. Propylthiouracil (PTU) is a medication commonly used to treat hyperthyroidism, but it is associated with various rare side effects, such as antineutrophil cytoplasm antibody-positive vasculitis. In the last decades, multiple cases of PTU causing antineutrophilic cytoplasmic antibody have been reported, some of them fatal. The present authors report the case of a 34-year-old Caucasian female affected by autoimmune hyperthyroidism treated with PTU, presenting an antineutrophil cytoplasm antibody-positive necrotizing vasculitis, with high levels of anticardiolipin antibodies that involved the upper arms and buttocks. The clinical manifestations improved after discontinuing of PTU and immunosuppressant treatment. [source] WEGENER'S GRANULOMATOSIS COMPLICATED WITH APHTHOID COLITISDIGESTIVE ENDOSCOPY, Issue 3 2006Yasushi Umehara A 58-year-old man was admitted with upper abdominal pain and high fever. There was no abnormality on chest X-ray, abdominal ultrasonography, abdominal CT and upper gastrointestinal endoscopy. Antineutrophil cytoplasmic antibodies (C-ANCA) titers were high and a chest CT scan depicted multiple nodules in the bilateral lungs. A diagnosis of Wegener's granulomatosis was therefore made. Three weeks after admission, diarrhea and bloody stool developed. Colonoscopy revealed many aphthoid lesions surrounded by redness in the entire colon. Although the biopsy from aphtha did not show vasculitis or granuloma, the aphthoid lesions were suspected as a complication of Wegener's granulomatosis. As a result of predonisolone medication (60 mg/day), the plasma C-reactive protein (CRP) and high fever improved promptly. In conclusion, although colonic involvement in a patient with Wegener's granulomatosis is extremely rare, it is important to keep in mind that colonic lesions might be due to vasculitis in ANCA-positive disease, such as Wegener's granulomatosis. [source] Echocardiographic Follow-Up of Patients with Takayasu's Arteritis: Five-Year SurvivalECHOCARDIOGRAPHY, Issue 5 2006María Elena Soto M.D, Ms.Sc. Takayasu's arteritis (TA) is a primary vasculitis that causes stenosis or occlusion, rarely aneurysm and distal ischemia. This study was undertaken to examine cardiovascular damage using echocardiography and determine the causes of morbid-mortality in Mexican Mestizo patients with TA. Seventy-six patients were studied by transthoracic echocardiography. Left ventricular diameters, parietal thickness, systolic function, and wall motion were analyzed, also, valvular lesions and aorta features were assessed. Thickness of the interventricular septum was 12 mm ± 3 (8,19), and that of posterior wall was 12 mm ± 2 (9,18). The average left ventricular diastolic diameter was 47 mm ± 7 (33,68) and the left ventricular systolic diameter 32 mm ± 8 (16,64). The left ventricular ejection fraction was of 57 ± 11%. Left ventricular concentric hypertrophy was found in 28 (50%) of the 56 hypertensive patients. The five-year survival of patients with left ventricular concentric hypertrophy was 80%, compared to 95% in patients without hypertrophy (P = 0.00). Abnormal wall motion was found in 15 patients. Thirty-one patients had aortic regurgitation, 19 had mitral regurgitation, 13 had tricuspid regurgitation, and 10 and pulmonary hypertension. Six patients had aneurysms of ascending aorta and 7 stenosis of descending aorta. Thirteen of 76 patients died (17%), 85% were hypertensive, and 9% also had acute myocardial infarction (AMI). Echocardiography, a noninvasive technique, shows a great utility in detection and follow-up of cardiovascular manifestations in patients with TA. New techniques, more sensitive toward detecting the early stages of left ventricular dysfunction, are promising to limit left ventricular hypertrophy development. [source] Neurologic manifestations of ulcerative colitisEUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2007R. Scheid Ulcerative colitis (UC) has traditionally been considered to be an inflammatory disease limited to the colonic mucosa. However, since it has been shown that UC is frequently accompanied by various extraintestinal disorders, there is increasing evidence that UC may also manifest in the nervous system. The following review focuses particularly on these possible manifestations of UC, both in the peripheral (PNS), and in the central nervous system (CNS). A systematic literature search according to the MEDLINE database was performed for this purpose. Although a reliable differentiation may clinically not always be possible, three major pathogenic entities can be differentiated: (i) cerebrovascular disease as a consequence of thrombosis and thromboembolism; (ii) systemic and cerebral vasculitis; (iii) probably immune mediated neuropathy and cerebral demyelination. With the exception of thromboembolism and sensorineural hearing loss, evidence for a causal relationship relies merely on single case reports or retrospective case series. Considering the CNS-manifestations, similarities between UC-associated disorders of the white matter and acute disseminated encephalomyelitis (ADEM) are obvious. Epileptic seizures, unspecified encephalopathies and confusional states are most likely epiphenomena that have to be regarded symptomatic rather than as own entities. A prospective study on the neurologic aspects of UC would be very welcome. [source] Neurobrucellosis as an exceptional cause of transient ischemic attacksEUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2006A. Bingöl We report a series of four cases presented with transient ischemic attacks (TIA) or ischemic stroke as the predominant manifestation of neurobrucellosis (NB). Three of the patients were 20,28 years of age, and one patient was 53 years old. They all used to consume unpasteurized milk or its products. Two patients had systemic brucellosis in the past and received antibiotic treatment. Other causes of TIA including cardiac embolism, hypercoagulability, vascular malformations, systemic vasculitis, and infective endocarditis were excluded. NB was diagnosed with serological tests or cultures for Brucella in the cerebrospinal fluid. None of the patients had any further TIA after the initiation of specific treatment. NB should always be sought in young patients with TIA or ischemic stroke, especially if they have no risk factors for stroke and live in an endemic area for brucellosis, even if they do not have other systemic signs of brucellosis. [source] Intracerebral haemorrhage as a manifestation of Lyme Neuroborreliosis?EUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2003R. Scheid Lyme Neuroborreliosis (LNB) has repeatedly been reported to cause cerebral vasculitis. However, there is no reliable information about the incidence of cerebral vessel affection. The majority of reports deal with ischaemic consequences, and there are a few reports of subarachnoid haemorrhage (SAH). We report a case of otherwise unexplained intracerebral haemorrhage (ICH) where clinical and laboratory tests have shown LNB. Late stage LNB might not only cause ischaemic, but haemorrhagic stroke as well. [source] Different pathways leading to cutaneous leukocytoclastic vasculitis in miceEXPERIMENTAL DERMATOLOGY, Issue 6 2001C. Sunderkötter Abstract: To investigate the pathomechanisms of leukocytoclastic vasculitis (LcV) we compared mouse models of LcV with non-vasculitic irritant contact dermatitis (ICD). Criteria for LcV as met by the immune complex-mediated Arthus reaction (Art-r) were also fulfilled by the localized Shwartzman reaction (Shw-r) and by cutaneous Loxoscelism (Lox) (injection of venom from Loxosceles reclusa containing sphingomyelinase D). After depletion of PMN (by ,-irradiation) vessel damage could not be elicited in these models, distinguishing them from models of direct endothelial insult (necrotizing ICD). Depletion of complement could only delay, but not inhibit the Art-r, and did not change ICD, Lox or the Shw-r. The Shw-r exclusively revealed a sustained local expression of vascular adhesion molecules for 24 h in the preparatory phase (LPS s.c.), not observed in the Art-r, in Lox or ICD. Subsequent challenge with LPS i.p. was associated with upregulation of Mac-1 and ICAM-1 on PMN, but not of VLA-4 or LFA-1 (FACS analysis). Cytokines which were able to replace LPS in priming for LcV in the Shw-r (TNF-, and IL-1,) also induced sustained expression of adhesion molecules, whereas IL-12 and IFN-, did neither. Neutralizing IL-12 or IFN-, also inhibited neither LcV nor sustained expression of adhesion molecules, whereas anti-TNF-, inhibited both. Anti-TNF-, had no marked inhibitory effects in the Art-r, in Lox or ICD. Combined (but not separate) neutralization of both E-selectin and VCAM-1 by antibodies suppressed LcV independent from reducing influx of PMN, proving that their sustained expression is decisive for the Shw-r and interferes with normal diapedesis. Since Loxosceles venom is known to dysregulate diapedesis and degranulation of PMN in vitro, since adherent immune complexes activate PMN at the vessel wall, and since adhesion molecules are dysregulated in the Shw-r, we suggest that LcV develops when activation of PMN coincides with vascular alterations which interfere with normal diapedesis. [source] The application of knowledge discovery in databases to post-marketing drug safety: example of the WHO databaseFUNDAMENTAL & CLINICAL PHARMACOLOGY, Issue 2 2008A. Bate Abstract After market launch, new information on adverse effects of medicinal products is almost exclusively first highlighted by spontaneous reporting. As data sets of spontaneous reports have become larger, and computational capability has increased, quantitative methods have been increasingly applied to such data sets. The screening of such data sets is an application of knowledge discovery in databases (KDD). Effective KDD is an iterative and interactive process made up of the following steps: developing an understanding of an application domain, creating a target data set, data cleaning and pre-processing, data reduction and projection, choosing the data mining task, choosing the data mining algorithm, data mining, interpretation of results and consolidating and using acquired knowledge. The process of KDD as it applies to the analysis of spontaneous reports can be exemplified by its routine use on the 3.5 million suspected adverse drug reaction (ADR) reports in the WHO ADR database. Examples of new adverse effects first highlighted by the KDD process on WHO data include topiramate glaucoma, infliximab vasculitis and the association of selective serotonin reuptake inhibitors (SSRIs) and neonatal convulsions. The KDD process has already improved our ability to highlight previously unsuspected ADRs for clinical review in spontaneous reporting, and we anticipate that such techniques will be increasingly used in the successful screening of other healthcare data sets such as patient records in the future. [source] Henoch,Schonlein purpura as a complication of a myelodysplastic syndromeGERIATRICS & GERONTOLOGY INTERNATIONAL, Issue 1 2006Jacob Feldman Henoch,Schonlein purpura (HSP) is considered as a small blood vessel systemic vasculitis. We describe a 78-year-old female, known to suffer from a myelodysplastic syndrome (MDS), who developed HSP with renal involvement. The ensuing decline in kidney function progressed to the point where the patient required dialysis. Surprisingly, renal biopsy did not show crescentic glomerulonephritis. MDS, essentially a hematological disorder of the elderly, has been associated with various autoimmune diseases including vasculitis, predominantly cutaneous. Our patient, however, is only the third reported in whom the combination of MDS with HSP was found. The occurrence of HSP in our patient with underlying MDS may represent a paraneoplastic phenomenon. [source] Posterior Ischemic Optic Neuropathy Associated With MigraineHEADACHE, Issue 7 2008Rod Foroozan MD Posterior ischemic optic neuropathy (PION) is an uncommon form of optic nerve ischemia that results from damage to the intraorbital, intracanalicular, or intracranial optic nerve. It has been reported perioperatively, in association with systemic vasculitis, and in the nonsurgical setting with no identifiable cause. Review of the literature reveals only 2 patients with PION associated with migraine in a single report. We report a patient who developed PION in the setting of a migraine headache without any other identifiable risk factors. [source] Tongue Necrosis in Temporal ArteritisHEADACHE, Issue 8 2007Maria Goicochea MD Temporal arteritis is a form of systemic vasculitis that involves branches of the carotid artery. Clinical features are headache, visual loss, ophthalmoplegia, jaw claudication, temporal headache, with tenderness and thickening on the affected temporal artery. We present 3 cases of tongue necrosis due to this granulomatous arteritis. Ischemic necrosis of the tongue is unusual and appears to be an association between its occurrence and high dose steroid tapering. [source] Migraine and Raynaud Phenomenon: Possible Late Complications of Kawasaki DiseaseHEADACHE, Issue 3 2002Cris S. Constantinescu MD Migraine and Raynaud phenomenon often coexist and may reflect similar vascular reactions. Both have been associated with vascular endothelial cell dysfunction. Kawasaki disease is a systemic vasculitis of unknown etiology that affects children and may lead to the formation of coronary artery aneurysms. Endothelial cell dysfunction has been demonstrated late in Kawasaki disease and is not restricted to coronary vessels. We report the case of a patient who developed typical migraine with aura and Raynaud phenomenon at the age of 14, 12 years after onset of Kawasaki disease. His migraine responded well to pizotifen, and both migraine and Raynaud phenomenon improved after initiation of treatment with valproic acid. We postulate that both migraine and Raynaud phenomenon in this case represent late consequences of Kawasaki disease and result from extracoronary endothelial dysfunction. [source] Epidemiology of primary systemic vasculitis in the Australian Capital Territory and south-eastern New South WalesINTERNAL MEDICINE JOURNAL, Issue 11 2008A. S. Ormerod Abstract Background:, The aim of the study was to determine the epidemiology of primary systemic vasculitis in the Australian Capital Territory and the surrounding rural region between 1995 and 2005. Methods:, Cases were ascertained by a medical record search according to international consensus classification criteria. For antineutrophil cytoplasmic antibody-associated vasculitides, ascertainment was corroborated by a search of all positive antineutrophil cytoplasmic antibody serology during the study period. Denominators were obtained from region-specific census data collected during the study period. Prevalence, incidence and patient characteristics for primary systemic vasculitides were determined for two 5-year periods, 1995,1999 and 2000,2004. Results:, We identified 41 cases of primary systemic vasculitides (Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg,Strauss syndrome or polyarteritis nodosa) between 1995 and 1999 and 67 between 2000 and 2004, giving prevalences of 95/million (95% confidence interval (CI) 76.9,116.1) and 148/million (95%CI 125.1,173.9), respectively. Annual incidence was similar in both periods (approximately 17/year per million adult population). Disease-specific incidences (per million per year) for each of the two periods were 8.8 and 8.4 for WG, 2.3 and 5.0 for MPA, 2.3 and 2.2 for Churg,Strauss syndrome and 2.3 and 1.1 for polyarteritis nodosa. The rural incidence of MPA was 13.9 (95%CI 7.7,23.5) compared with 1.6 (95%CI 0.2,7.2) in the city and there was a trend towards a higher incidence of WG in rural than urban areas. Conclusion:, The overall incidence of primary systemic vasculitides is similar to that reported from other developed countries. WG is more common in south-eastern Australia than in southern Europe, whereas MPA is less common. There was a trend towards higher incidence of antineutrophil cytoplasmic antibody-associated vasculitides in rural than urban areas. [source] Warfarin-induced allergic interstitial nephritis and leucocytoclastic vasculitisINTERNAL MEDICINE JOURNAL, Issue 4 2008K. G. Kapoor Abstract Warfarin sodium has been associated with leucocytoclastic vasculitis and has once been associated with allergic interstitial nephritis. Hypersensitivity to warfarin sodium simultaneously resulting in allergic interstitial nephritis and leucocytoclastic vasculitis has not yet been previously reported. We present a 48-year-old man who was on warfarin sodium for 2 months and presented with acute renal failure and reddish purplish macules on his hypogastric regions and lower extremities bilaterally. Kidney biopsy showed allergic interstitial nephritis and punch skin biopsy showed leucocytoclastic vasculitis. Both biopsies also showed high eosinophil count, highly suggestive of a drug-induced reaction. After a negative comprehensive work-up and the absence of other recent medication changes, our patient was determined to have allergic interstitial nephritis and leucocytoclastic vasculitis secondary to warfarin sodium. [source] Systemic lupus erythematosus with vasculitis confined to the coronary arteriesINTERNAL MEDICINE JOURNAL, Issue 2 2000W. A. C. DOUGLAS No abstract is available for this article. [source] | |||||||||||||||||||||||||||||||||||||||||||