Database : MEDLINE
Search on : Vasculitis [Words]
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[PMID]: 23560992
[Au] Autor:Gigante A; Gasperini ML; Barbano B; Liberatori M; Sardo L; Jovanovic T; Giannakakis K; Cianci R; Amoroso A
[Ad] Address:Department of Nephrology, Sapienza University of Rome , Rome , Italy.
[Ti] Title:Gabexate Mesylate as Treatment in the Course of ANCA-Negative Microscopic Polyangiitis.
[So] Source:Ren Fail;35(5):721-4, 2013.
[Is] ISSN:1525-6049
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Patients with small vessel vasculitis present fluctuating antineutrophil cytoplasmic antibodies (ANCA) levels to the point that positive ANCA may be missed even if only up to 10% of patients with microscopic polyangiitis (MPA) are ANCA-negative. The first-line treatment of MPA is the association of steroids and cyclophosphamide, especially in the presence of a rapidly progressive glomerulonephritis. Plasmapheresis, intravenous immunoglobulins, and tumor necrosis factor inhibitors have been proposed as alternative to standard therapy. Disseminated intravascular coagulation (DIC) is a possible event in the course of small vessel vasculitis. Gabexate mesylate is a protease inhibitor able to suppress endothelial cell injury, and it may be administered to treat DIC related to different diseases. In ANCA-associated vasculitis, cytokines play a key role in promoting endothelial damage. DIC-related thrombocytopenia may be misinterpreted as drug-induced because of the immunosuppressive properties of cyclophosphamide. Two cases of ANCA-positive MPA associated with DIC and treated with gabexate are reported in the literature with improvement of both hematological disorder and renal function. Our patient presented a rapidly progressive glomerulonephritis, and the renal biopsy showed MPA, in the absence of ANCA. After two weeks of steroid treatment, our patient developed a DIC. This case represents the first report of ANCA-negative MPA managed with gabexate, which showed improvement of coagulation disorders and kidney function. In conclusion, the anti-inflammatory properties of gabexate could be helpful in MPA at increased bleeding risk when immunosuppressive treatment is contraindicated, even in ANCA-negative vasculitis.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1305
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.3109/0886022X.2013.780620

  2 / 30028 MEDLINE  
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[PMID]: 23642042
[Au] Autor:Janssens P; Arnaud L; Galicier L; Mathian A; Hie M; Sene D; Haroche J; Veyssier-Belot C; Huynh-Charlier I; Grenier PA; Piette JC; Amoura Z
[Ad] Address:Department of internal medicine, French reference centre for Systemic Lupus Erythematosus, AP-HP, Hôpital Pitié-Salpêtrière, F-75013, Paris, France. laurent.arnaud@psl.aphp.fr.
[Ti] Title:Lupus enteritis: from clinical findings to therapeutic management.
[So] Source:Orphanet J Rare Dis;8:67, 2013.
[Is] ISSN:1750-1172
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Lupus enteritis is a rare and poorly understood cause of abdominal pain in patients with systemic lupus erythematosus (SLE). In this study, we report a series of 7 new patients with this rare condition who were referred to French tertiary care centers and perform a systematic literature review of SLE cases fulfilling the revised ACR criteria, with evidence for small bowel involvement, excluding those with infectious enteritis. We describe the characteristics of 143 previously published and 7 new cases. Clinical symptoms mostly included abdominal pain (97%), vomiting (42%), diarrhea (32%) and fever (20%). Laboratory features mostly reflected lupus activity: low complement levels (88%), anemia (52%), leukocytopenia or lymphocytopenia (40%) and thrombocytopenia (21%). Median CRP level was 2.0 mg/dL (range 0-8.2 mg/dL). Proteinuria was present in 47% of cases. Imaging studies revealed bowel wall edema (95%), ascites (78%), the characteristic target sign (71%), mesenteric abnormalities (71%) and bowel dilatation (24%). Only 9 patients (6%) had histologically confirmed vasculitis. All patients received corticosteroids as a first-line therapy, with additional immunosuppressants administered either from the initial episode or only in case of relapse (recurrence rate: 25%). Seven percent developed intestinal necrosis or perforation, yielding a mortality rate of 2.7%. Altogether, lupus enteritis is a poorly known cause of abdominal pain in SLE patients, with distinct clinical and therapeutic features. The disease may evolve to intestinal necrosis and perforation if untreated. Adding with this an excellent steroid responsiveness, timely diagnosis becomes primordial for the adequate management of this rare entity.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1305
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1186/1750-1172-8-67

  3 / 30028 MEDLINE  
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[PMID]: 23475760
[Au] Autor:Gotkine M; Vaknin-Dembinsky A
[Ad] Address:Department of Neurology, Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, Hebrew University Hadassah Medical School, Kiryat Hadassah, Jerusalem, Israel, marc@gotkine.com.
[Ti] Title:Central nervous system vasculitis.
[So] Source:Curr Treat Options Neurol;15(3):367-74, 2013 Jun.
[Is] ISSN:1092-8480
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OPINION STATEMENT: A patient presenting with suspected primary central nervous system (CNS) vasculitis is often difficult to manage. Once the diagnosis is suspected, the clinical course may already be severe and warrant timely aggressive treatment with potentially toxic drugs. Definitive diagnosis is often delayed, requiring specialized tests that are only possible in certain centers and sometimes prove to be inconclusive. The need to make a confident diagnosis before starting powerful and potentially toxic immunosuppression, often conflicts with the desire to initiate timely treatment aimed at preventing irreversible neurological dysfunction. The heterogeneity and rarity of this condition is a major obstacle to conducting properly designed, double blinded, placebo-controlled studies. There is no Class I evidence for any form of treatment in these situations, and management guidelines represent expert opinion, based primarily on trials investigating therapies affecting systemic conditions with a CNS vasculitic component. Cyclophosphamide is still the main immunosuppressive drug used during active disease in combination with high dose steroids. Rituximab may be an equally efficacious, less toxic alternative, but its use is still limited due to its cost.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1305
[Da] Date of entry for processing:130513
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1007/s11940-013-0227-8

  4 / 30028 MEDLINE  
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[PMID]: 23212754
[Au] Autor:Wolf J; Schmitt V; Palm F; Grau AJ; Bergner R
[Ad] Address:Department of Neurology, Klinikum der Stadt Ludwigshafen, Bremserstraße 79, 67063 Ludwigshafen, Germany. wolfj@klilu.de
[Ti] Title:Peripheral neuropathy as initial manifestation of primary systemic vasculitides.
[So] Source:J Neurol;260(4):1061-70, 2013 Apr.
[Is] ISSN:1432-1459
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Peripheral neuropathies are well-known complications of primary systemic vasculitides. In rare cases, peripheral neuropathies are among the first symptoms of these diseases. In this prospective study, 89 consecutive adult patients with newly diagnosed primary systemic vasculitis were screened, of whom 22 patients (25 %, 12 men, ten women, mean age, 59 years, range, 26-82 years) suffered from peripheral neuropathy due to systemic vasculitis at initial presentation. Peripheral neuropathy was most frequent in newly diagnosed patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome, 12 out of 20 patients, 60 %) and polyarteritis nodosa (three out of six patients, 50 %), and less common in patients with granulomatosis with polyangiitis (six out of 47 patients, 13 %) and microscopic polyangiitis (one out of 16 patients, 6 %). Multiplex mononeuropathy was more frequent (n = 13, 59 %) than symmetric polyneuropathy (n = 9, 41 %). The nerves commonly affected were the peroneal nerve, followed by the sural, posterior tibial, and median nerves. Treatment options were chosen according to current guidelines of the national neurological and rheumatologic societies, with initial corticosteroid monotherapy for patients with a mild disease form and a combination of corticosteroids and intravenously pulsed cyclophosphamide for patients with a more extended organ involvement. During follow-up (mean, 34 months, range, 12-112 months), new neurological complications were rare (9 %): One patient suffered from a cerebral infarct while another patient sustained epileptic seizures. Two patients (9 %) died from sepsis (after 60 months) or severe gastrointestinal bleeding (after 13 months). The degree of neurological disability measured by the functional disability score (described by Prineas) improved in 20 of 22 patients after 12 months of therapy.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1304
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1007/s00415-012-6760-7

  5 / 30028 MEDLINE  
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[PMID]: 23552597
[Au] Autor:Alokaily FA; Alghamdi M; Almalki AS; Alhussaini H
[Ad] Address:Department of Medicine, Division of Rheumatology, Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia. alokaily@yahoo.com
[Ti] Title:Aspirin induced leukocytoclastic vasculitis, lower gastrointestinal hemorrhage and acute renal failure (mimicking systemic vasculitis).
[So] Source:Saudi Med J;34(4):420-3, 2013 Apr.
[Is] ISSN:0379-5284
[Cp] Country of publication:Saudi Arabia
[La] Language:eng
[Ab] Abstract:This is a case of a 60-year-old gentleman who presented with an extensive cutaneous vasculitic rash (leukocytoclastic) with lower gastrointestinal bleeding following a small dose of aspirin (81 mg). The aspirin was stopped immediately. Three weeks later, he had acute renal failure, which was initially thought to be secondary to systemic vasculitis, but proved to be acute renal tubular necrosis as a delayed reaction to aspirin.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1304
[Js] Journal subset:IM
[St] Status:In-Process

  6 / 30028 MEDLINE  
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[PMID]: 22928813
[Au] Autor:Farah M; Levin A; Kiaii M; Vickars L; Werb R
[Ad] Address:Divisions of Nephrology, University of British Columbia, Vancouver, Canada.
[Ti] Title:Combination hemodialysis and centrifugal therapeutic plasma exchange: 18 years of Canadian experience.
[So] Source:Hemodial Int;17(2):256-65, 2013 Apr.
[Is] ISSN:1542-4758
[Cp] Country of publication:Canada
[La] Language:eng
[Ab] Abstract:Hemodialysis (HD) and therapeutic plasma exchange (TPE) are extracorporeal treatments that may both be required in the same patient. When provided separately, 7-8 hours of therapy time is required. Simultaneous administration of both therapies can reduce time and personnel requirements. We report our 18-year institutional experience with combination HD and centrifugal TPE therapy. During combination therapy, the TPE circuit is attached to the HD circuit through an extension blood line connected to the HD venous return line, allowing simultaneous operation of both circuits. The HD circuit is anticoagulated with heparin and the TPE circuit with regional citrate. Blood flow rates through the HD circuit can reach 350 mL/min with plasma removal rates in the TPE circuit up to 60 mL/min. Ninety-two patients received a total of 621 treatments between December 1993 and July 2011. All treatments were completed within 4 hours. No major treatment-related adverse events occurred and less than 10% of treatments were complicated by minor events. Main indications for treatment were ANCA (anti-neutrophilic cytoplasmic antibody) vasculitis (n = 25), Goodpasture's/antiglomerular basement membrane disease (n = 24), adult thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (n = 24), and acute antibody-mediated renal transplant rejection (n = 8). Overall rates of renal recovery, in-hospital mortality, and overall mortality at 18-year follow-up were 45% (41/ 92), 2% (2/92), and 21% (19/ 92), respectively, compatible with published literature. Combination HD and TPE is safe, efficient, and requires less human resources and time than conventional sequential therapy. It should be considered in patients whose treatment regimen includes HD and TPE.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1304
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1111/j.1542-4758.2012.00737.x

  7 / 30028 MEDLINE  
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[PMID]: 22954107
[Au] Autor:Saraf R; Limaye U
[Ad] Address:Department of Radiology, Division of Interventional Neuroradiology, KEM Hospital, Mumbai, Maharashtra, India.
[Ti] Title:Ruptured intracranial tubercular infectious aneurysm secondary to a tuberculoma and its endovascular management.
[So] Source:Br J Neurosurg;27(2):243-5, 2013 Apr.
[Is] ISSN:1360-046X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Tuberculosis remains to be an endemic infectious disease in developing countries. With the increasing incidence of HIV and AIDS, there is further increase in the incidence of tuberculosis. Although CNS involvement by tuberculosis is seen in all age groups, there is a predilection for younger patients. Central nervous system tuberculosis may present as tuberculoma, cerebral abscess or tuberculous meningitis (TBM). Vasculitis secondary to TBM can cause infarcts and rarely aneurysm formation. In TBM there is a thick, gelatinous exudate around the sylvian fissures, basal cisterns. There is a border zone reaction occurring in the surrounding brain tissue. Inflammatory changes occur in the vessel wall of the arteries bathed in the exudate leading to narrowing of the lumen or occlusion by thrombus formation. The vessels at the base of the brain are most severely affected, including the internal carotid artery, proximal middle cerebral artery and perforating vessels of the basal ganglion. In these cases, the infection probably spreads from the adventitia towards the internal elastic lamina, weakening the vessel wall, with subsequent formation of an infectious aneurysm. Intracranial tuberculomas are space-occupying masses of granulomatous tissue that result from haematogenous spread from a distant focus of tuberculous infection. In endemic regions, tuberculomas account for as many as 50% of all intracranial space-occupying lesions. Inflammation in the vessels surrounding the tuberculoma may lead to formation of aneurysms. This case report illustrates an unusual case of intracranial tuberculomas complicated by intralesional haemorrhage due to an infective tubercular aneurysm in its vicinity. The endovascular treatment of these infectious aneurysms is safe, effective and durable. To the best of our knowledge, this is the first case report of a tuberculoma having intracranial haemorrhage on anti-tubercular treatment due to an infectious aneurysm developing in an artery in the vicinity of the tuberculoma and managed end.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1304
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.3109/02688697.2012.717986

  8 / 30028 MEDLINE  
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[PMID]: 23393320
[Au] Autor:Tan DS; Gan PY; O'Sullivan KM; Hammett MV; Summers SA; Ooi JD; Lundgren BA; Boyd RL; Scott HS; Kitching AR; Chidgey AP; Holdsworth SR
[Ad] Address:Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, 246 Clayton Road, Clayton, VIC 3168, Australia.
[Ti] Title:Thymic deletion and regulatory T cells prevent antimyeloperoxidase GN.
[So] Source:J Am Soc Nephrol;24(4):573-85, 2013 Mar.
[Is] ISSN:1533-3450
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Loss of tolerance to neutrophil myeloperoxidase (MPO) underlies the development of ANCA-associated vasculitis and GN, but the mechanisms underlying this loss of tolerance are poorly understood. Here, we assessed the role of the thymus in deletion of autoreactive anti-MPO T cells and the importance of peripheral regulatory T cells in maintaining tolerance to MPO and protecting from GN. Thymic expression of MPO mRNA predominantly localized to medullary thymic epithelial cells. To assess the role of MPO in forming the T cell repertoire and the role of the autoimmune regulator Aire in thymic MPO expression, we compared the effects of immunizing Mpo(-/-) mice, Aire(-/-) mice, and control littermates with MPO. Immunized Mpo(-/-) and Aire(-/-) mice developed significantly more proinflammatory cytokine-producing anti-MPO T cells and higher ANCA titers than control mice. When we triggered GN with a subnephritogenic dose of anti-glomerular basement membrane antibody, Aire(-/-) mice had more severe renal disease than Aire(+/+) mice, consistent with a role for Aire-dependent central deletion in establishing tolerance to MPO. Furthermore, depleting peripheral regulatory T cells in wild-type mice also led to more anti-MPO T cells, higher ANCA titers, and more severe GN after immunization with MPO. Taken together, these results suggest that Aire-dependent central deletion and regulatory T cell-mediated peripheral tolerance both play major roles in establishing and maintaining tolerance to MPO, thereby protecting against the development of anti-MPO GN.
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1304
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1681/ASN.2012090898

  9 / 30028 MEDLINE  
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[PMID]: 23528592
[Ti] Title:[Diagnosis of isolated angiitis of the central nervous system].
[So] Source:Zh Nevrol Psikhiatr Im S S Korsakova;113(2 Pt 2):32-42, 2013.
[Is] ISSN:1997-7298
[Cp] Country of publication:Russia (Federation)
[La] Language:rus
[Ab] Abstract:We have analyzed clinical presentations, neuroimaging data, blood and cerebrospinal fluid diagnostic tests in isolated angiitis of the central nervous system on the basis of data of recent publications and 22 our own cases. Due to the insufficient diagnostic value of invasive methods including biopsy of leptomeningeal and intracerebral vessels, we considered practical approaches to the diagnosis of cerebral vasculitis and ways of its optimization. We suggested the stratification of clinical, neuroimaging and laboratory criteria for the evaluation of their sensitivity and specificity and for the development of diagnostic algorithms.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1303
[Js] Journal subset:IM
[St] Status:In-Process

  10 / 30028 MEDLINE  
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[PMID]: 22696739
[Au] Autor:Ando K; Fujiya M; Sugiyama R; Nata T; Nomura Y; Ueno N; Kashima S; Ishikawa C; Inaba Y; Ito T; Moriichi K; Okamoto K; Ikuta K; Watari J; Mizukami Y; Kohgo Y
[Ad] Address:Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan.
[Ti] Title:Atypical tumour-like involvement of the colon in Henoch-Schonlein purpura successfully treated with the administration of factor XIII.
[So] Source:BMJ Case Rep;2011, 2011.
[Is] ISSN:1757-790X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Henoch-Schönlein purpura (HSP) is a type of systemic vasculitis of the small vessels, which frequently involves the skin, kidney and gastrointestinal tract. While the typical intestinal features of HSP include diffuse mucosal redness, small ring-like petechiae and haemorrhagic erosions, tumour-like lesions are rarely observed. The current study presents a rare case of HSP with an intestinal tumour-like lesion in the caecum. The intestinal lesion caused fresh melaena, and was completely resolved with the administration of factor XIII as described in previously reported cases. It is important to immediately undergo proper treatment for improving tumour-like lesions which may cause severe complications, such as excessive haemorrhage and stricture.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1206
[Js] Journal subset:IM
[St] Status:In-Process


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