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NCBI: db=pubmed; Term=vasculitis AND ((English[lang] OR French[lang]) AND adult[MeSH] AND "last 90 days"[PDat])
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Diagnosis, Management, and Outcome of Aortitis at a Single Center.

mer, 01/11/2017 - 14:26
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Diagnosis, Management, and Outcome of Aortitis at a Single Center.

Vasc Endovascular Surg. 2017 Oct;51(7):470-479

Authors: Skeik N, Ostertag-Hill CA, Garberich RF, Alden PB, Alexander JQ, Cragg AH, Manunga JM, Stephenson EJ, Titus JM, Sullivan TM

Abstract
BACKGROUND: Aortitis is a rare condition with inflammatory or infectious etiology that can be difficult to diagnose due to the highly variable clinical presentation and nonspecific symptoms. However, current literature on the diagnosis, management, and prognosis of aortitis is extremely scarce.
METHODS: We retrospectively reviewed all patients' charts who were diagnosed with giant cell arteritis, Takayasu arteritis, or noninfectious aortitis presenting at a single center between January 1, 2009, and April 17, 2015. Data collected included demographics, medical history, comorbidities, laboratory and imaging data, management, and outcome.
RESULTS: Among the included 15 patients presenting with aortitis at our center, 53% were diagnosed with Takayasu arteritis, 33% with idiopathic inflammatory aortitis, and 13% with giant cell arteritis. All patients received steroid treatment, 67% received adjunctive immunosuppressants or immunomodulators, and 33% underwent interventional procedures. Based on clinical presentation and laboratory and imaging findings at the last follow-up visit for each patient, 67% showed improvement, 27% had no change in disease activity, and 7% had a progression of the disease.
CONCLUSIONS: Takayasu arteritis was found to be more common than idiopathic inflammatory aortitis and giant cell arteritis among our 15 cases diagnosed with aortitis. All patients received medical therapy and 33% received interventional procedures, leading to 67% improvement of disease activity or related complications. This article also offers a comprehensive review of the diagnosis, management, and outcome of aortitis, supplementing the very limited literature on this disease.

PMID: 28859604 [PubMed - indexed for MEDLINE]

[Acute pancreatitis as the presenting feature of an IgA vasculitis: An unusual presentation].

mer, 01/11/2017 - 14:26
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[Acute pancreatitis as the presenting feature of an IgA vasculitis: An unusual presentation].

Rev Med Interne. 2017 Oct;38(10):691-694

Authors: Fertitta L, Noel N, Ackermann F, Lerolle N, Benoist S, Rocher L, Lambotte O

Abstract
INTRODUCTION: IgA vasculitis is a systemic small vessel leukocytoclastic vasculitis characterized by skin purpura, arthritis, abdominal pain and nephritis. Most of the abdominal complications are due to edema and hemorrhage in the small bowel wall, but rarely to acute secondary pancreatitis.
CASE REPORT: Here, we report a 53-year-old woman who presented with acute pancreatitis and, secondarily, developed skin purpura and arthritis at the seventh day of the clinical onset. Biological tests and computed tomographic scan allowed to rule out another cause of pancreatitis and IgA vasculitis was diagnosed as its etiology. The outcome was favorable without any relapse on glucocorticoids.
CONCLUSION: Despite its rarity, pancreatitis is a potential life-threatening complication of IgA vasculitis in which the role of glucocorticoids and immunosuppressive drugs remains uncertain. A prompt elimination of other usual pancreatitis etiologies is mandatory to improve the management of the patients.

PMID: 28215926 [PubMed - indexed for MEDLINE]

Case 32-2017. A 64-Year-Old Man with Dyspnea, Wheezing, Headache, Cough, and Night Sweats.

dim, 29/10/2017 - 12:01
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Case 32-2017. A 64-Year-Old Man with Dyspnea, Wheezing, Headache, Cough, and Night Sweats.

N Engl J Med. 2017 10 19;377(16):1569-1578

Authors: Palamara K, Nagarur A, Fintelmann FJ, Kohler MJ, Cortazar FB

PMID: 29045211 [PubMed - indexed for MEDLINE]

Added Value of Vessel Wall Magnetic Resonance Imaging for Differentiation of Nonocclusive Intracranial Vasculopathies.

dim, 29/10/2017 - 12:01
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Added Value of Vessel Wall Magnetic Resonance Imaging for Differentiation of Nonocclusive Intracranial Vasculopathies.

Stroke. 2017 Nov;48(11):3026-3033

Authors: Mossa-Basha M, Shibata DK, Hallam DK, de Havenon A, Hippe DS, Becker KJ, Tirschwell DL, Hatsukami T, Balu N, Yuan C

Abstract
BACKGROUND AND PURPOSE: Our goal is to determine the added value of intracranial vessel wall magnetic resonance imaging (IVWI) in differentiating nonocclusive vasculopathies compared with luminal imaging alone.
METHODS: We retrospectively reviewed images from patients with both luminal and IVWI to identify cases with clinically defined intracranial vasculopathies: atherosclerosis (intracranial atherosclerotic disease), reversible cerebral vasoconstriction syndrome, and inflammatory vasculopathy. Two neuroradiologists blinded to clinical data reviewed the luminal imaging of defined luminal stenoses/irregularities and evaluated the pattern of involvement to make a presumed diagnosis with diagnostic confidence. Six weeks later, the 2 raters rereviewed the luminal imaging in addition to IVWI for the pattern of wall involvement, presence and pattern of postcontrast enhancement, and presumed diagnosis and confidence. Analysis was performed on per-lesion and per-patient bases.
RESULTS: Thirty intracranial atherosclerotic disease, 12 inflammatory vasculopathies, and 12 reversible cerebral vasoconstriction syndrome patients with 201 lesions (90 intracranial atherosclerotic disease, 64 reversible cerebral vasoconstriction syndrome, and 47 inflammatory vasculopathy lesions) were included. For both per-lesion and per-patient analyses, there was significant diagnostic accuracy improvement with luminal imaging+IVWI when compared with luminal imaging alone (per-lesion: 88.8% versus 36.1%; P<0.001 and per-patient: 96.3% versus 43.5%; P<0.001, respectively). There was substantial interrater diagnostic agreement for luminal imaging+IVWI (κ=0.72) and only slight agreement for luminal imaging (κ=0.04). Although there was a significant correlation for both luminal and IVWI pattern of wall involvement with diagnosis, there was a stronger correlation for IVWI finding of lesion eccentricity and intracranial atherosclerotic disease diagnosis than for luminal imaging (κ=0.69 versus 0.18; P<0.001).
CONCLUSIONS: IVWI can significantly improve the differentiation of nonocclusive intracranial vasculopathies when combined with traditional luminal imaging modalities.

PMID: 29030476 [PubMed - indexed for MEDLINE]

Methotrexate versus cyclophosphamide for remission maintenance in ANCA-associated vasculitis: A randomised trial.

lun, 23/10/2017 - 10:48
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Methotrexate versus cyclophosphamide for remission maintenance in ANCA-associated vasculitis: A randomised trial.

PLoS One. 2017;12(10):e0185880

Authors: Maritati F, Alberici F, Oliva E, Urban ML, Palmisano A, Santarsia F, Andrulli S, Pavone L, Pesci A, Grasselli C, Santi R, Tumiati B, Manenti L, Buzio C, Vaglio A

Abstract
OBJECTIVES: The treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is based on remission-induction and remission-maintenance. Methotrexate is a widely used immunosuppressant but only a few studies explored its role for maintenance in AAV. This trial investigated the efficacy and safety of methotrexate as maintenance therapy for AAV.
METHODS: In this single-centre, open-label, randomised trial we compared methotrexate and cyclophosphamide for maintenance in AAV. We enrolled patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), the latter with poor-prognosis factors and/or peripheral neuropathy. Remission was induced with cyclophosphamide. At remission, the patients were randomised to receive methotrexate or to continue with cyclophosphamide for 12 months; after treatment, they were followed for another 12 months. The primary end-point was relapse; secondary end-points included renal outcomes and treatment-related toxicity.
RESULTS: Of the 94 enrolled patients, 23 were excluded during remission-induction or did not achieve remission; the remaining 71 were randomised to cyclophosphamide (n = 33) or methotrexate (n = 38). Relapse frequencies at months 12 and 24 after randomisation were not different between the two groups (p = 1.00 and 1.00). Relapse-free survival was also comparable (log-rank test p = 0.99). No differences in relapses were detected between the two treatments when GPA+MPA and EGPA were analysed separately. There were no differences in eGFR at months 12 and 24; proteinuria declined significantly (from diagnosis to month 24) only in the cyclophosphamide group (p = 0.0007). No significant differences in adverse event frequencies were observed.
CONCLUSIONS: MTX may be effective and safe for remission-maintenance in AAV.
TRIAL REGISTRATION: clinicaltrials.gov NCT00751517.

PMID: 29016646 [PubMed - indexed for MEDLINE]

Aspergillus Galactomannan for Diagnosing Invasive Aspergillosis.

lun, 23/10/2017 - 10:48
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Aspergillus Galactomannan for Diagnosing Invasive Aspergillosis.

JAMA. 2017 Sep 26;318(12):1175-1176

Authors: Miceli MH, Kauffman CA

PMID: 28973595 [PubMed - indexed for MEDLINE]

Clinical and imaging features of spinal cord type of neuro Behçet disease: A case report and systematic review.

sam, 14/10/2017 - 04:13
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Clinical and imaging features of spinal cord type of neuro Behçet disease: A case report and systematic review.

Medicine (Baltimore). 2017 Oct;96(40):e7958

Authors: Liu HM, Dong C, Zhang YZ, Tian YY, Chen HX, Zhang S, Li N, Gu P

Abstract
RATIONAL: To investigate the clinical and MRI characteristics of spinal cord nerve Behçet's disease.
PATIENT CONCERNS: One patient with spinal cord nerve Behçet's disease was admitted to our hospital at October 20, 2015.
DIAGNOSE: Spinal cord nerve Behçet's disease.
INTERVENTIONS: Retrospective analysis was performed on such case as well as 16 cases of spinal cord nerve Behçet's disease reported in China or abroad.
OUTCOMES: Seventeen cases of spinal cord type of neuro Behçet's disease include 13 men and 4 women, with an average age of onset of 34.8 years old. The mean time from Behçet's disease symptoms to spinal cord involvement were 10.8 years. The initial symptom in one case was spinal cord injury, and another 4 cases had a recurrence course. The most common performance of spinal cord injury was sensory disturbance (82.4%), following by weakness (76.5%), sphincter or sexual dysfunction (58.8%), and pain in back, backside of neck or lower chest (29.4%). The number of cells was slightly increased or the protein level was increased in cerebrospinal fluid test. And the water channel protein antibody and oligoclonal band of serum levels were all negative. The spinal cord injury involved more than 3 vertebral bodies in 10 cases, and involved more than half of spinal cord in sagittal plane in 8 cases. In acute stage, shock therapy with large dose of glucocorticoid was generally applied both in China and abroad.
LESSONS: The clinical features of spinal cord nerve Behçet's disease were various, making it easily misdiagnosed. Longitudinal extensive transverse myelitis performs as a characteristic manifestation.

PMID: 28984755 [PubMed - indexed for MEDLINE]

Variability in quantitative analysis of atherosclerotic plaque inflammation using 18F-FDG PET/CT.

sam, 14/10/2017 - 04:13
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Variability in quantitative analysis of atherosclerotic plaque inflammation using 18F-FDG PET/CT.

PLoS One. 2017;12(8):e0181847

Authors: Lensen KDF, van Sijl AM, Voskuyl AE, van der Laken CJ, Heymans MW, Comans EFI, Nurmohamed MT, Smulders YM, Boellaard R

Abstract
BACKGROUND: 18F-FDG-PET(/CT) is increasingly used in studies aiming at quantifying atherosclerotic plaque inflammation. Considerable methodological variability exists. The effect of data acquisition and image analysis parameters on quantitative uptake measures, such as standardized uptake value (SUV) and target-to-background ratio (TBR) has not been investigated extensively.
OBJECTIVE: The goal of this study was to explore the effect of several data acquisition and image analysis parameters on quantification of vascular wall 18F-FDG uptake measures, in order to increase awareness of potential variability.
METHODS: Three whole-body emission scans and a low-dose CT scan were acquired 38, 60 and 90 minutes after injection of 18F-FDG in six rheumatoid arthritis patients with high cardiovascular risk profiles.Data acquisition (1 and 2) and image analysis (3, 4 and 5) parameters comprised:1. 18F-FDG uptake time, 2. SUV normalisation, 3. drawing regions/volumes of interest (ROI's/VOI's) according to: a. hot-spot (HS), b. whole-segment (WS) and c. most-diseased segment (MDS), 4. Background activity, 5. Image matrix/voxel size.Intraclass correlation coefficients (ICC's) and Bland Altman plots were used to assess agreement between these techniques and between observers. A linear mixed model was used to determine the association between uptake time and continuous outcome variables.
RESULTS: 1. Significantly higher TBRmax values were found at 90 minutes (1,57 95%CI 1,35-1,80) compared to 38 minutes (1,30 95%CI 1,21-1,39) (P = 0,024) 2. Normalising SUV for BW, LBM and BSA significantly influences average SUVmax (2,25 (±0,60) vs 1,67 (±0,37) vs 0,058 (±0,013)). 3. Intraclass correlation coefficients were high in all vascular segments when SUVmax HS was compared to SUVmax WS. SUVmax HS was consistently higher than SUVmax MDS in all vascular segments. 4. Blood pool activity significantly decreases in all (venous and arterial) segments over time, but does not differ between segments. 5. Image matrix/voxel size does not influence SUVmax.
CONCLUSION: Quantitative measures of vascular wall 18F-FDG uptake are affected mainly by changes in data acquisition parameters. Standardization of methodology needs to be considered when studying atherosclerosis and/or vasculitis.

PMID: 28800625 [PubMed - indexed for MEDLINE]

Clinical impact of a targeted next-generation sequencing gene panel for autoinflammation and vasculitis.

sam, 14/10/2017 - 04:13
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Clinical impact of a targeted next-generation sequencing gene panel for autoinflammation and vasculitis.

PLoS One. 2017;12(7):e0181874

Authors: Omoyinmi E, Standing A, Keylock A, Price-Kuehne F, Melo Gomes S, Rowczenio D, Nanthapisal S, Cullup T, Nyanhete R, Ashton E, Murphy C, Clarke M, Ahlfors H, Jenkins L, Gilmour K, Eleftheriou D, Lachmann HJ, Hawkins PN, Klein N, Brogan PA

Abstract
BACKGROUND: Monogenic autoinflammatory diseases (AID) are a rapidly expanding group of genetically diverse but phenotypically overlapping systemic inflammatory disorders associated with dysregulated innate immunity. They cause significant morbidity, mortality and economic burden. Here, we aimed to develop and evaluate the clinical impact of a NGS targeted gene panel, the "Vasculitis and Inflammation Panel" (VIP) for AID and vasculitis.
METHODS: The Agilent SureDesign tool was used to design 2 versions of VIP; VIP1 targeting 113 genes, and a later version, VIP2, targeting 166 genes. Captured and indexed libraries (QXT Target Enrichment System) prepared for 72 patients were sequenced as a multiplex of 16 samples on an Illumina MiSeq sequencer in 150bp paired-end mode. The cohort comprised 22 positive control DNA samples from patients with previously validated mutations in a variety of the genes; and 50 prospective samples from patients with suspected AID in whom previous Sanger based genetic screening had been non-diagnostic.
RESULTS: VIP was sensitive and specific at detecting all the different types of known mutations in 22 positive controls, including gene deletion, small INDELS, and somatic mosaicism with allele fraction as low as 3%. Six/50 patients (12%) with unclassified AID had at least one class 5 (clearly pathogenic) variant; and 11/50 (22%) had at least one likely pathogenic variant (class 4). Overall, testing with VIP resulted in a firm or strongly suspected molecular diagnosis in 16/50 patients (32%).
CONCLUSIONS: The high diagnostic yield and accuracy of this comprehensive targeted gene panel validate the use of broad NGS-based testing for patients with suspected AID.

PMID: 28750028 [PubMed - indexed for MEDLINE]

Febrile abdominal pain revealing Horton's disease.

sam, 14/10/2017 - 04:13
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Febrile abdominal pain revealing Horton's disease.

Am J Emerg Med. 2017 Oct;35(10):1583.e3-1583.e5

Authors: Chaudet A, Goujon JM, Ghazali AD

Abstract
BACKGROUND: Horton's disease is a systemic inflammatory vasculitis, usually found in persons over 50years old. It affects medium and large-sized arteries stemming from the external carotid, especially the superficial temporal arteries. It can affect extracranial large vessels but only rarely the aorta. Diagnosis of aortitis is difficult and its incidence is probably underestimated.
CASE PRESENTATION: A 68-year-old Caucasian woman consulted in an emergency department for febrile abdominal pain with inflammatory syndrome. Abdomen was soft with right-side flank sensitivity. A contrast-enhanced CT scan showed aortitis from the descending aorta to the iliac arteries without complication. Because of age, clinical presentation and aortitis, Horton disease was suspected. The temporal artery biopsy showed a histological aspect of degenerative endarteritis with intimal thickening and luminal stenosis. High-dose corticosteroid therapy was introduced which improved clinical conditions and resulted in the amendment of the pain.
DISCUSSION: In the present case, this patient had Horton's disease, based on 3 criteria of The American College of Rheumatology (age, temporal artery abnormalities and inflammatory syndrome) associated with aortitis. However, aortitis is a rare complication of Horton disease and is a major cause of mortality inasmuch as it can be complicated by aneurysm and dissection. It is unusual to diagnose Horton's disease from aortitis symptoms without complications. The aorta represents the most severe localization of Horton's disease. It should not be ignored in etiological hypotheses regarding febrile abdominal pain in the elderly. Corticosteroids should be started rapidly at high doses and temporal artery biopsy should be planned.

PMID: 28712642 [PubMed - indexed for MEDLINE]

Adverse Local Tissue Responses to Failed Temporomandibular Joint Implants.

sam, 14/10/2017 - 04:13
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Adverse Local Tissue Responses to Failed Temporomandibular Joint Implants.

J Oral Maxillofac Surg. 2017 Oct;75(10):2076-2084

Authors: Mercuri LG, Urban RM, Hall DJ, Mathew MT

Abstract
PURPOSE: The purpose of this study was to determine whether failed alloplastic temporomandibular joint replacement (TMJR) devices can elicit the aseptic lymphocyte-dominated vasculitis-associated lesion (ALVAL) reaction seen in some patients with metal-on-metal hip arthroplasties.
MATERIALS AND METHODS: This study involved analysis of paraffin-embedded sections of peri-implant tissue from failed TMJ implant cases obtained from 3 independent sources. Hematoxylin and eosin staining, conventional and polarized light microscopy, back-scattered electron imaging, and energy-dispersive x-ray analysis were used. Immunohistochemical methods were used to identify T and B lymphocytes and macrophages.
RESULTS: The total TMJR device specimens showed primary macrophage and lymphocytic responses similar to responses reported previously for failed total hip implants, including ALVAL. No chronic or acute inflammation was apparent in the failed hemiarthroplasty TMJR cases.
CONCLUSION: In this limited preliminary study, the local tissue responses to the failed TMJR implants showed similar primary macrophage and lymphocyte responses to previously reported failed metal-on-metal and metal-on-polyethylene orthopedic total joint replacement devices. No such local inflammatory responses were seen with the failed TMJR hemiarthroplasty devices.

PMID: 28449848 [PubMed - indexed for MEDLINE]

Clinical characteristics of inflammatory ocular disease in anti-neutrophil cytoplasmic antibody associated vasculitis: a retrospective cohort study.

sam, 07/10/2017 - 22:25
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Clinical characteristics of inflammatory ocular disease in anti-neutrophil cytoplasmic antibody associated vasculitis: a retrospective cohort study.

Rheumatology (Oxford). 2017 Oct 01;56(10):1763-1770

Authors: Ungprasert P, Crowson CS, Cartin-Ceba R, Garrity JA, Smith WM, Specks U, Matteson EL, Makol A

Abstract
Objective: To characterize the clinical correlates and outcome of inflammatory ocular disease (IOD) among patients with ANCA-associated vasculitides (AAV).
Methods: Medical records of potential cases of AAV seen at Mayo Clinic from 2003 to 2013, inclusive, were reviewed to identify confirmed cases meeting the diagnosis of AAV using the Chapel Hill Consensus Conference 2012 descriptors. Records of confirmed cases of AAV were then further reviewed for IOD, and clinical characteristics, treatment and outcomes abstracted.
Results: A total of 1171 confirmed cases of AAV were identified of which 183 patients (mean age 49.0 years; 51% female; 95% Caucasian) had IOD. The most common manifestation of IOD was injection of the eye (57%) followed by eye pain (46%) and visual acuity loss (18%). Scleritis was the most common type of IOD (22%) followed by episcleritis (21%), orbital inflammation (18%), lacrimal duct stenosis (10%) and uveitis (9%). Oral glucocorticoids were used to treat IOD in the majority of patients (96%). CYC and rituximab were the most frequently used immunosuppressive agents (54 and 36%, respectively). Of those with orbital inflammation, 52% underwent therapeutic surgical intervention. Clinical remission of IOD was achieved in 91% of patients but relapses were seen in 23%. Significant visual acuity loss was observed in only six patients.
Conclusion: IOD is a common manifestation of AAV and seen in about 16% of patients with AAV. Scleritis, episcleritis and orbital inflammation are the most common subtypes. Most patients respond well to glucocorticoids and immunosuppression, but relapse of IOD is common.

PMID: 28957561 [PubMed - indexed for MEDLINE]

Long-Term Outcomes and Prognostic Factors of Complications in Takayasu Arteritis: A Multicenter Study of 318 Patients.

sam, 07/10/2017 - 22:25
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Long-Term Outcomes and Prognostic Factors of Complications in Takayasu Arteritis: A Multicenter Study of 318 Patients.

Circulation. 2017 Sep 19;136(12):1114-1122

Authors: Comarmond C, Biard L, Lambert M, Mekinian A, Ferfar Y, Kahn JE, Benhamou Y, Chiche L, Koskas F, Cluzel P, Hachulla E, Messas E, Resche-Rigon M, Cacoub P, Mirault T, Saadoun D, French Takayasu Network

Abstract
BACKGROUND: Because of the wide variation in the course of Takayasu arteritis (TA), predicting outcome is challenging. We assess long-term outcome and prognosis factors for vascular complications in patients with TA.
METHODS: A retrospective multicenter study of characteristics and outcomes of 318 patients with TA fulfilling American College of Rheumatology and Ishikawa criteria was analyzed. Factors associated with event-free survival, relapse-free survival, and incidences of vascular complications were assessed. Risk factors for vascular complications were identified in a multivariable model.
RESULTS: The median age at TA diagnosis was 36 [25-47] years, and 276 patients (86.8%) were women. After a median follow-up of 6.1 years, relapses were observed in 43%, vascular complications in 38%, and death in 5%. Progressive clinical course was observed in 45%, carotidodynia in 10%, and retinopathy in 4%. The 5- and 10-year event-free survival, relapse-free survival, and complication-free survival were 48.2% (42.2; 54.9) and 36.4% (30.3; 43.9), 58.6% (52.7; 65.1) and 47.7% (41.2; 55.1), and 69.9% (64.3; 76.0) and 53.7% (46.8; 61.7), respectively. Progressive disease course (P=0.018) and carotidynia (P=0.036) were independently associated with event-free survival. Male sex (P=0.048), elevated C-reactive protein (P=0.013), and carotidynia (P=0.003) were associated with relapse-free survival. Progressive disease course (P=0.017), thoracic aorta involvement (P=0.009), and retinopathy (P=0.002) were associated with complication-free survival.
CONCLUSIONS: This nationwide study shows that 50% of patients with TA will relapse and experience a vascular complication ≤10 years from diagnosis. We identified specific characteristics that identified those at highest risk for subsequent vascular complications.

PMID: 28701469 [PubMed - indexed for MEDLINE]

Successful Hybrid Neurovascular Surgery for a Ruptured Basilar Artery Dissecting Aneurysm in a Patient with Jeopardized Collateral Circulation Associated with Aortitis Syndrome.

sam, 07/10/2017 - 22:25
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Successful Hybrid Neurovascular Surgery for a Ruptured Basilar Artery Dissecting Aneurysm in a Patient with Jeopardized Collateral Circulation Associated with Aortitis Syndrome.

World Neurosurg. 2017 Sep;105:1034.e7-1034.e10

Authors: Yamamoto K, Mori T, Watanabe T, Iwata T, Gondo G, Tanaka M, Tanaka S

Abstract
BACKGROUND: Dissecting aneurysms of the basilar artery (BA) are rare lesions, and the management of them has been controversial and challenging. The treatment becomes much more difficult when the patient has a disorder such as aortitis syndrome, which makes cerebral arteries occluded and tortuous. We describe a case of a ruptured BA dissecting aneurysm associated with aortitis syndrome, successfully treated with hybrid neurovascular surgery.
CASE DESCRIPTION: A 64-year-old woman with a medical history of aortitis syndrome developed a severe headache and was diagnosed with a subarachnoid hemorrhage from a BA dissecting aneurysm. Her right common carotid artery and right vertebral artery (VA) were occluded because of aortitis syndrome. Blood flow in the right middle cerebral artery (MCA) was mainly supplied by the right posterior communicating artery. It was essential to preserve blood flow in the BA to prevent a cerebral infarction in the area of the right MCA. Stent-assisted coil embolization was considered the most appropriate treatment. The occluded right VA was thought to be more appropriate for introduction of an intravascular stent than the left VA. The patient underwent open surgery to expose the right VA. An intravascular stent was placed through the exposed right VA, and intra-aneurysmal coils were introduced from the left VA. The aneurysm was successfully treated with a hybrid technique.
CONCLUSIONS: To our knowledge, this is the first report of hybrid neurovascular surgery for a BA dissecting aneurysm. A ruptured BA dissecting aneurysm in a patient with aortitis syndrome was successfully treated with hybrid neurovascular surgery.

PMID: 28602923 [PubMed - indexed for MEDLINE]

Maintenance therapy is associated with better long-term outcomes in adult patients with primary angiitis of the central nervous system.

sam, 07/10/2017 - 22:25
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Maintenance therapy is associated with better long-term outcomes in adult patients with primary angiitis of the central nervous system.

Rheumatology (Oxford). 2017 Oct 01;56(10):1684-1693

Authors: de Boysson H, Parienti JJ, Arquizan C, Boulouis G, Gaillard N, Régent A, Néel A, Detante O, Touzé E, Aouba A, Bienvenu B, Guillevin L, Naggara O, Zuber M, Pagnoux C

Abstract
Objective: We aimed to analyse the effect of maintenance therapy after induction on the outcomes of adult patients with primary angiitis of the CNS (PACNS).
Methods: We analysed long-term outcomes (relapse, survival and functional status) of patients enrolled in the French multicentre PACNS cohort who achieved remission after induction treatment and with ⩾12 months' follow-up, according to whether or not they received maintenance therapy. Good outcome was defined as relapse-free survival and good functional status (modified Rankin scale ⩽ 2) at last follow-up.
Results: Ninety-seven patients [46 (47%) female, median age: 46 (18-78) years at diagnosis] were followed up for a median of 55 (5-198) months. Induction treatment consisted of glucocorticoids in 95 (98%) patients, combined with an immunosuppressant in 80 (83%) patients, mostly CYC. Maintenance therapy was prescribed in 48 (49%) patients, following CYC in 42 of them. Maintenance therapy was started 4 (3-18) months after glucocorticoid initiation. At last follow-up, good outcomes were observed in 32 (67%) patients who had received maintenance therapy vs 10 (20%) who had not (P < 0.0001). Thirty-two (33%) patients experienced relapse [10 (22%) had received maintenance therapy while 22 (45%) had not, P = 0.01]; four subsequently died from relapse. In the multivariate analysis, maintenance therapy was the only independent predictor of good outcome [odds ratio (OR) = 7.8 (95% CI: 3.21, 20.36), P < 0.0001].
Conclusion: The results of this long-term follow-up study suggest that maintenance therapy in adults with PACNS is associated with better functional outcomes and lower relapse rates. Further studies are needed to confirm these findings.

PMID: 28340158 [PubMed - indexed for MEDLINE]

The Role of Human Immunodeficiency Virus-Associated Vasculopathy in the Etiology of Stroke.

mer, 04/10/2017 - 19:38
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The Role of Human Immunodeficiency Virus-Associated Vasculopathy in the Etiology of Stroke.

J Infect Dis. 2017 Sep 01;216(5):545-553

Authors: Benjamin LA, Allain TJ, Mzinganjira H, Connor MD, Smith C, Lucas S, Joekes E, Kampondeni S, Chetcuti K, Turnbull I, Hopkins M, Kamiza S, Corbett EL, Heyderman RS, Solomon T

Abstract
Background: Human immunodeficiency virus (HIV) infection is a recognized risk factor for stroke among young populations, but the exact mechanisms are poorly understood. We studied the clinical, radiologic, and histologic features of HIV-related ischemic stroke to gain insight into the disease mechanisms.
Methods: We conducted a prospective, in-depth analysis of adult ischemic stroke patients presenting to Queen Elizabeth Central Hospital, Blantyre, Malawi, in 2011.
Results: We recruited 64 HIV-infected and 107 HIV-uninfected patients. Those with HIV were significantly younger (P < .001) and less likely to have established vascular risk factors. Patients with HIV were more likely to have large artery disease (21% vs 10%; P < .001). The commonest etiology was HIV-associated vasculopathy (24 [38%]), followed by opportunistic infections (16 [25%]). Sixteen of 64 (25%) had a stroke soon after starting antiretroviral therapy (ART), suggesting an immune reconstitution-like syndrome. In this group, CD4+ T-lymphocyte count was low, despite a significantly lower HIV viral load in those recently started on treatment (P < .001).
Conclusions: HIV-associated vasculopathy and opportunistic infections are common causes of HIV-related ischemic stroke. Furthermore, subtypes of HIV-associated vasculopathy may manifest as a result of an immune reconstitution-like syndrome after starting ART. A better understanding of this mechanism may point toward new treatments.

PMID: 28931222 [PubMed - indexed for MEDLINE]

Gene therapy for Wiskott-Aldrich syndrome in a severely affected adult.

mer, 04/10/2017 - 19:38
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Gene therapy for Wiskott-Aldrich syndrome in a severely affected adult.

Blood. 2017 Sep 14;130(11):1327-1335

Authors: Morris EC, Fox T, Chakraverty R, Tendeiro R, Snell K, Rivat C, Grace S, Gilmour K, Workman S, Buckland K, Butler K, Chee R, Salama AD, Ibrahim H, Hara H, Duret C, Mavilio F, Male F, Bushman FD, Galy A, Burns SO, Gaspar HB, Thrasher AJ

Abstract
Until recently, hematopoietic stem cell transplantation was the only curative option for Wiskott-Aldrich syndrome (WAS). The first attempts at gene therapy for WAS using a ϒ-retroviral vector improved immunological parameters substantially but were complicated by acute leukemia as a result of insertional mutagenesis in a high proportion of patients. More recently, treatment of children with a state-of-the-art self-inactivating lentiviral vector (LV-w1.6 WASp) has resulted in significant clinical benefit without inducing selection of clones harboring integrations near oncogenes. Here, we describe a case of a presplenectomized 30-year-old patient with severe WAS manifesting as cutaneous vasculitis, inflammatory arthropathy, intermittent polyclonal lymphoproliferation, and significant chronic kidney disease and requiring long-term immunosuppressive treatment. Following reduced-intensity conditioning, there was rapid engraftment and expansion of a polyclonal pool of transgene-positive functional T cells and sustained gene marking in myeloid and B-cell lineages up to 20 months of observation. The patient was able to discontinue immunosuppression and exogenous immunoglobulin support, with improvement in vasculitic disease and proinflammatory markers. Autologous gene therapy using a lentiviral vector is a viable strategy for adult WAS patients with severe chronic disease complications and for whom an allogeneic procedure could present an unacceptable risk. This trial was registered at www.clinicaltrials.gov as #NCT01347242.

PMID: 28716862 [PubMed - indexed for MEDLINE]

Outcomes of endovascular recanalization versus autogenous venous bypass for thromboangiitis obliterans patients with critical limb ischemia due to tibioperoneal arterial occlusion.

mer, 04/10/2017 - 19:38
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Outcomes of endovascular recanalization versus autogenous venous bypass for thromboangiitis obliterans patients with critical limb ischemia due to tibioperoneal arterial occlusion.

J Vasc Surg. 2017 Oct;66(4):1133-1142.e1

Authors: Ye K, Shi H, Qin J, Yin M, Liu X, Li W, Jiang M, Lu X

Abstract
OBJECTIVE: This study sought to compare the outcomes of endovascular recanalization (ER) vs autogenous venous bypass (AVB) for tibioperoneal arterial occlusion in thromboangiitis obliterans patients with critical lower limb ischemia.
METHODS: A total of 90 limbs in 75 patients, successfully treated with ER (ER group, 35 patients, 43 limbs) and AVB (AVB group, 40 patients, 47 limbs) for tibioperoneal arterial occlusions from January 2009 to December 2015 in a single institution, were retrospectively analyzed. The characteristics and outcomes were compared between the two groups. The primary outcome was the amputation-free survival rate during follow-up; the secondary outcomes were patency rates, immediate failure, periprocedural complications, and rates of reintervention. Univariate and multivariate analysis to identify potentially significant predictors of amputation-free survival and primary patency in the whole study group was performed.
RESULTS: Not all patients in the ER group were bypass candidates because of unavailable runoff arteries or adequate conduit for bypass. Other baseline characteristics were similar between groups. Patients in the ER group had a much higher incidence of immediate failure (36.36% in femoropopliteal segment and 65.52% in tibioperoneal segment vs 14.89% in AVB bypass; P = .03 and < .001). Although patients in the ER group had a significantly higher rate of reintervention (62.79% vs 27.66%; P < .001) associated with a significantly lower primary patency rate (18.96% in femoropopliteal segment and 14.37% in tibioperoneal segment vs 60.41% in AVB bypass; P = .008 and < .001) and secondary patency rate (33.85% in femoropopliteal segment and 21.29% in tibioperoneal segment vs 68.78% in AVB bypass; P = .04 and .002) at 3 years, the amputation-free survival in the ER group and AVB group was similar at 1 year (92.9% vs 93.2%; P = .81) and 3 years (87.8% and 90.6%; P = .66). Univariate and multivariate analysis showed that the presence of gangrene was independently associated with major amputation (hazard ratio, 2.24; 95% confidence interval, 1.33-4.3; P = .02); however, the presence of active ulcer was the only risk factor for poorer primary patency during follow-up (hazard ratio, 1.86; 95% confidence interval, 0.55-5.6; P = .04).
CONCLUSIONS: ER is a valid strategy for limb salvage in thromboangiitis obliterans patients who are unsuitable for bypass, contributing an acceptable amputation-free survival as high as with AVB, even though it is associated with lower patency rates and a higher rate of reintervention.

PMID: 28697938 [PubMed - indexed for MEDLINE]

Safety and Efficacy of Warfarin Therapy in Kawasaki Disease.

mer, 04/10/2017 - 19:38
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Safety and Efficacy of Warfarin Therapy in Kawasaki Disease.

J Pediatr. 2017 Oct;189:61-65

Authors: Baker AL, Vanderpluym C, Gauvreau KA, Fulton DR, de Ferranti SD, Friedman KG, Murray JM, Brown LD, Almond CS, Evans-Langhorst M, Newburger JW

Abstract
OBJECTIVE: To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications.
STUDY DESIGN: We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and <6; and INRs <1.5.
RESULTS: In 9 patients (5 male), median age 14.4 years (range 7.1-22.8 years), INR testing was prescribed weekly to monthly and was done by home monitor (n = 5) or laboratory (n = 3) or combined (1). Median length of warfarin therapy was 7.2 years (2.3-13.3 years). Goal INR was 2.0-3.0 (n = 6) or 2.5-3.5 (n = 3), based on CAA size and history of CAA thrombosis. All patients were treated with aspirin; 1 was on dual antiplatelet therapy and warfarin. The median time in therapeutic range was 59% (37%-85%), and median percentage of INRs in range was 68% (52%-87%). INR >6 occurred in 3 patients (4 events); INRs ≥5 <6 in 7 patients (12 events); and INR <1.5 in 5 patients (28 events). The incidence of major bleeding events and clinically relevant nonmajor bleeding events were each 4.3 per 100 patient-years (95% CI 0.9-12.6). New asymptomatic coronary thrombosis was detected by imaging in 2 patients.
CONCLUSIONS: Bleeding and clotting complications are common in patients with Kawasaki disease on warfarin and aspirin, with INRs in range only two-thirds of the time. Future studies should evaluate the use of direct oral anticoagulants in children as an alternative to warfarin.

PMID: 28552449 [PubMed - indexed for MEDLINE]

Randomised controlled trial of prolonged treatment in the remission phase of ANCA-associated vasculitis.

mer, 04/10/2017 - 19:38
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Randomised controlled trial of prolonged treatment in the remission phase of ANCA-associated vasculitis.

Ann Rheum Dis. 2017 Oct;76(10):1662-1668

Authors: Karras A, Pagnoux C, Haubitz M, Groot K, Puechal X, Tervaert JWC, Segelmark M, Guillevin L, Jayne D, European Vasculitis Society

Abstract
OBJECTIVES: A prospective randomised trial to compare two different durations of maintenance immunosuppressive therapy for the prevention of relapse in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV).
METHODS: Patients with AAV were recruited 18-24 months after diagnosis if they were in stable remission after cyclophosphamide/prednisolone-based induction followed by azathioprine/prednisolone maintenance therapy. They were randomised (1:1) to receive continued azathioprine/prednisolone to 48 months from diagnosis (continuation group) or to withdraw azathioprine/prednisolone by 24 months (withdrawal group). The primary endpoint was the relapse risk, from randomisation to 48 months from diagnosis.
RESULTS: One hundred and seventeen patients were randomised and 110 remained to the trial end. At entry, median serum creatinine was 116 μmol/L (range 58-372), 53% were ANCA positive. The percentage of patients presenting with relapse was higher in the withdrawal than in the continuation treatment group (63% vs 22%, p<0.0001, OR 5.96, 95% CI 2.58 to 13.77). ANCA positivity at randomisation was associated with relapse risk (51% vs 29%, p=0.017, OR 2.57, 95% CI 1.16 to 5.68). Renal function, ANCA specificity, vasculitis type and age were not predictive of relapse. Severe adverse events were more frequent in the continuation than withdrawal groups (nine vs three events), but the continuation group had better renal outcome (0 vs 4 cases of end-stage renal disease), with no difference in patient survival.
CONCLUSIONS: Prolonged remission maintenance therapy with azathioprine/prednisolone, beyond 24 months after diagnosis reduces relapse risk out to 48 months and improves renal survival in AAV.
TRIAL REGISTRATION NUMBER: ISRCTN13739474.

PMID: 28546260 [PubMed - indexed for MEDLINE]

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