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The Use and Pitfalls of Intracranial Vessel Wall Imaging: How We Do It.

Vascularites - jeu, 11/01/2018 - 00:50
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The Use and Pitfalls of Intracranial Vessel Wall Imaging: How We Do It.

Radiology. 2018 Jan;286(1):12-28

Authors: Lindenholz A, van der Kolk AG, Zwanenburg JJM, Hendrikse J

Abstract
Intracranial vessel wall magnetic resonance (MR) imaging has gained much attention in the past decade and has become part of state-of-the-art MR imaging protocols to assist in diagnosing the cause of ischemic stroke. With intracranial vessel wall imaging, vessel wall characteristics have tentatively been described for atherosclerosis, vasculitis, dissections, Moyamoya disease, and aneurysms. With the increasing demand and subsequently increased use of intracranial vessel wall imaging in clinical practice, radiologists should be aware of the choices in imaging parameters and how they affect image quality, the clinical indications, methods of assessment, and limitations in the interpretation of these images. In this How I do It article, the authors will discuss the technical requirements and considerations for vessel wall image acquisition in general, describe their own vessel wall imaging protocol at 3 T and 7 T, show a step-by-step basic assessment of intracranial vessel wall imaging as performed at their institution-including commonly encountered artifacts and pitfalls-and summarize the commonly reported imaging characteristics of various intracranial vessel wall diseases for direct clinical applicability. Finally, future technical and clinical considerations for full implementation of intracranial vessel wall imaging in clinical practice, including the need for histologic validation and acquisition time reduction, will be discussed.

PMID: 29261469 [PubMed - indexed for MEDLINE]

Atypical acute retinal necrosis accompanied by Terson's syndrome: a case report.

Vascularites - dim, 07/01/2018 - 23:25
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Atypical acute retinal necrosis accompanied by Terson's syndrome: a case report.

BMC Ophthalmol. 2017 Dec 19;17(1):255

Authors: Lee JY, Kim DY, Lee HJ, Jeong JH, Park SP, Kim JY

Abstract
BACKGROUND: Acute retinal necrosis (ARN) has characterized by panuveitis, vitritis, severe vaso-occlusive vasculitis, and diffuse necrotizing retinitis. There are no case reports on atypical ARN combined with Terson's syndrome. Herein, we report a case of ARN with atypical clinical features combined with Terson's syndrome that we successfully treated by intravitreal ganciclovir injection.
CASE PRESENTATION: A 64-year-old man visited our eye clinic with a complaint of decreased visual acuity in his right eye. At the initial visit, his best corrected visual acuity was 20/125 in the right eye. Slit-lamp examination demonstrated mild hyperemia, keratic precipitates, and anterior chamber inflammatory reaction. Fundus examination revealed multiple diffuse white-yellowish infiltrations in the peripheral retina combined with dot hemorrhages. Ultra-wide-field fluorescence angiography showed obstructive arteritis with peripheral non-perfusion and leakage from the retinal vessels. As a result of the PCR analysis, varicella zoster virus DNA was identified in the aqueous humor. Under the diagnosis with VZV-mediated ARN, we started with intravenous acyclovir and oral prednisolone. After 3 days of the above treatment, the anterior chamber inflammation and vitreous opacity were increased. On fundus examination, multiple whitish infiltrations were increased. In addition, newly developed vitreous and peripapillary hemorrhages were detected. On the T2 brain magnetic resonance imaging (MRI) demonstrated a sub-acute or old hemorrhagic infarction in the right occipital lobe, and contrast-enhancing lesions in the right basal ganglia. The spinal tapping was performed in the department of neurology in our hospital at the time when the patient complained of headache, and intracranial pressure was 31 mmHg. Under the diagnosis of ARN with Terson's syndrome, we started intravitreal ganciclovir (2 mg/0.5 ml) injections. After 5 intravitreal ganciclovir injections over a period of 8 months, the diffuse whitish infiltrating retinal lesions combined with dot hemorrhage were decreased. The vitreous and peripapillary hemorrhage was significantly reduced. There was no recurrence in the patient's right eye, in which his visual acuity had improved to 20/60.
CONCLUSIONS: In the event of a poor response to traditional treatment such as intravenous acyclovir, intravitreal ganciclovir may have a role as an adjunctive therapy in patients of VZV associated ARN combined with Terson's syndrome.

PMID: 29258454 [PubMed - indexed for MEDLINE]

Multiple aneurysms and gastrointestinal involvement in Behcet's disease: A case report.

Vascularites - dim, 07/01/2018 - 23:25
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Multiple aneurysms and gastrointestinal involvement in Behcet's disease: A case report.

Medicine (Baltimore). 2017 Dec;96(49):e9131

Authors: Wang M, Sun W, Chen Z, Wang X, Lv J, Tan Q, Wang Y, Zhou J

Abstract
RATIONALE: Behcet's disease (BD) is a rare systemic vasculitis disorder that can involve vessels of any size.
PATIENT CONCERNS: A 60-year-old female had recurrent painful mouth ulcers about 30 years ago and recently presented with abdominal distension, conjunctival congestion, and chest pain in sequence.
DIAGNOSES: The patient was diagnosed with BD according to the International Criteria for Behcet's Disease (ICBD).
INTERVENTIONS: A therapy of glucocorticoids and cyclophosphamide was administered.
OUTCOMES: After a 3-month treatment, follow-up examination showed a depression of the inflammation and a slight decrease of the arterial aneurysms.
LESSONS: BD patients may get a delayed diagnosis because of the long duration between the first and subsequent manifestations. Suspect patients should be followed-up and the diagnosis of BD should be considered when multiple tissues or organs are involved.

PMID: 29245353 [PubMed - indexed for MEDLINE]

Ten-year progress of coronary artery lesions prior to Behçet disease diagnosis: A case report and care-compliant article.

Vascularites - dim, 07/01/2018 - 23:25
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Ten-year progress of coronary artery lesions prior to Behçet disease diagnosis: A case report and care-compliant article.

Medicine (Baltimore). 2017 Dec;96(49):e9102

Authors: Ma W, Liang Y, Zhu J

Abstract
INTRODUCTION: Behçet disease is a multisystemic chronic inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. However, particularly part of patients would present cardiovascular involvements and vascular lesions could be the presenting sign of Behçet disease preceding classical symptoms. We presented a middle aged male patient, in whom abdominal aorta pseudoaneurysm was as the first leading sign to reveal Behçet disease, and with his coronary artery lesions progress through a 10-year period before Behçet disease was diagnosed.
CONCLUSIONS: Coronary artery involvement of Behçet disease warrants more attention and investigation; repeated in-stent stenosis, aggressive progress, and elevated inflammation markers should be regard with more care earlier in clinical practice.

PMID: 29245339 [PubMed - indexed for MEDLINE]

Successful treatment of erythema induratum with topical application of antituberculous drugs: A case report.

Vascularites - dim, 07/01/2018 - 23:25
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Successful treatment of erythema induratum with topical application of antituberculous drugs: A case report.

Medicine (Baltimore). 2017 Dec;96(49):e9010

Authors: Mei X, Zhao J

Abstract
RATIONALE: Erythema induratum, a chronic recurrent lobular panniculitis with vasculitis, is strongly associated with Mycobacterium tuberculosis infection. The recommended drugs include isoniazid, rifampicin, and pyrazinamide, which are typically administered in combination (orally or intravenously). Till date, there are no reports about topical application of anti-tuberculous (anti-TB) drugs for treatment of erythema induratum.
PATIENT CONCERNS: Herein, we present the case of a 73-year-old woman with recurrent ulceration, scarring and obvious pain in her lower legs.
DIAGNOSES: She was diagnosed of erythema induratum.
INTERVENTIONS: Topical anti-TB treatment (3.75% isoniazid twice a day) was necessitated by the development of severe gastrointestinal upset and significant reduction in platelets after oral treatment with isoniazid and rifampicin.
OUTCOMES: The skin lesions showed improvement at one month and remitted mostly at two months. After 6 months, the skin lesions have subsided and no obvious side effects were observed.
LESSONS: Our experience may help expand the therapeutic regimens for cutaneous tuberculosis, and provide physicians with alternative options for management of tuberculosis.

PMID: 29245283 [PubMed - indexed for MEDLINE]

Transcriptional profiling of PBMCs unravels B cell mediated immunopathogenic imprints of HCV vasculitis.

Vascularites - dim, 07/01/2018 - 23:25
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Transcriptional profiling of PBMCs unravels B cell mediated immunopathogenic imprints of HCV vasculitis.

PLoS One. 2017;12(12):e0188314

Authors: Comstock E, Kim CW, Murphy A, Emmanuel B, Zhang X, Sneller M, Poonia B, Kottilil S

Abstract
B cell depletion therapy using rituximab has been shown to be effective in achieving remission in patients with HCV-mixed cryoglobulinemic (MC) vasculitis. Previously, we have demonstrated abnormalities in peripheral immune cells involving neutrophils, chemotaxis, and innate immune activation among patients with HCV-MC vasculitis when compared to HCV patients without vasculitis. In this study, we evaluated the effect of B cell depletion therapy on transcriptional profiles of peripheral blood mononuclear cells before and after riruximab therapy, in order to unravel the pathogenic mechanism involved in HCV-MC vasculitis induced by abnormal B cell proliferation. DNA microarray analysis was performed using RNA from PBMCs from seven patients with HCV-MC vasculitis and seven normal volunteers. DNA was hybridized to Affymetrix U133A chips. After normalization, differentially expressed gene list with treatment was generated using partitional clustering. RT-PCR, flow cytometry, and enzyme immunoassay (EIA) was used to validate DNA microarray findings. Differentially expressed genes included B cells and non-B cell genes. Validation of genes using purified cell subsets demonstrated distinct effect of B cell depletion therapy on non-B cells, such as monocytes, T cells, and NK cells. Notably, B lymphocyte stimulator (BLyS) levels were persistently elevated in patients who subsequently relapsed. In conclusion, pathogenesis of HCV-MC vasculitis is mediated by abnormal proliferation of B cells, driven by BLyS, leading to significant effects on non-B cells in mediating symptomatology. Future therapeutics using a combination approach of B cell depletion and proliferation may be desired to achieve long-term remission.

PMID: 29228031 [PubMed - indexed for MEDLINE]

In vivo visualization of braid-like appearance in Kawasaki disease: insights from multi-modality imaging.

Vascularites - dim, 07/01/2018 - 23:25
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In vivo visualization of braid-like appearance in Kawasaki disease: insights from multi-modality imaging.

Int J Cardiovasc Imaging. 2017 Dec;33(12):1891-1893

Authors: Nakano H, Kataoka Y, Yasuda S

Abstract
A 42-year-old gentleman with Kawasaki disease was referred for silent cardiac ischemia at inferior myocardial region. Coronary angiography showed braid-like appearance in the right coronary artery. Several channels at the segment with braid-like appearance were visualized by multi-slice computed tomography and intravascular ultrasound. Interestingly in particular, optical coherence tomography visualized the presence of multiple micro-vessels which were not detectable on other modalities. This case further elucidates distinct morphological features of braid-like lesion which potentially influences blood supply and causing cardiac ischemia.

PMID: 28667489 [PubMed - indexed for MEDLINE]

Location of skin lesions in Henoch-Schönlein purpura and its association with significant renal involvement.

Vascularites - lun, 01/01/2018 - 20:55
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Location of skin lesions in Henoch-Schönlein purpura and its association with significant renal involvement.

J Am Acad Dermatol. 2018 Jan;78(1):115-120

Authors: St John J, Vedak P, Garza-Mayers AC, Hoang MP, Nigwekar SU, Kroshinsky D

Abstract
BACKGROUND: Henoch-Schönlein purpura (HSP) is a small vessel IgA-predominant vasculitis.
OBJECTIVE: To describe adult patients with HSP and determine if the distribution of skin lesions (ie, purpura above the waist or purpura below the waist only), is a predictor of significant renal involvement at the time of the skin biopsy and the months following.
METHODS: A retrospective study on renal function from 72 adult patients with skin-biopsy proven HSP. Longitudinal renal data were analyzed after HSP diagnosis by using baseline renal function for comparison.
RESULTS: Statistical analysis adjusted for sex, age, and baseline creatinine revealed a trend between HSP lesions only on the upper and lower extremities and long-term renal involvement (4.767, P = .067). Moreover, in another analysis adjusted for age and baseline creatinine, lesions located only on the upper and lower extremities significantly increased the odds of having long-term significant renal involvement (6.55, P = .049) in men.
LIMITATIONS: This retrospective study used patient information that was subject to selection bias.
CONCLUSION: In patients with HSP, skin lesion distribution on the extremities might be predictive of significant long-term renal involvement and might be critical for risk stratification and development of personalized diagnostics and therapeutics.

PMID: 29241772 [PubMed - indexed for MEDLINE]

Development of a predictive model for temporal artery biopsies.

Vascularites - ven, 29/12/2017 - 20:11
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Development of a predictive model for temporal artery biopsies.

Can J Ophthalmol. 2017 Dec;52(6):599-605

Authors: Weis E, Toren A, Jordan D, Patel V, Gilberg S

Abstract
OBJECTIVE: Temporal artery biopsy is a critical, relatively safe, and reliable test in the diagnosis of temporal arteritis. Yet, a clarification of the pre-test probabilities may provide clarity on which patients with suspected giant cell arteritis would benefit from this invasive diagnostic procedure.
DESIGN: A prospective case series PARTICIPANTS: A consecutive case series of patients referred to the Ophthalmology service for temporal artery biopsy.
METHODS: All subjects underwent standardized serum testing, and signs and symptoms assessment. Predictive models were created and evaluated.
RESULTS: 119 patients were analyzed. This exploratory study found that a simple model including platelet count, erythrocyte sedimentation rate, and c-reactive protein was able to define a subset of patients with a pre-test probability of a positive biopsy of 0% or 100%. 40% (95% confidence interval 31%-49%) of patients fell into this category.
CONCLUSIONS: Utilizing a simple clinically applicable predictive model of the pretest probability of a temporal artery biopsy in patients with suspected giant cell arteritis, up to 31%-49% of temporal artery biopsies may be avoided. This study was a single site exploratory study with data-driven thresholds - therefore these results need to be validated with an independent sample prior to clinical use.

PMID: 29217029 [PubMed - indexed for MEDLINE]

Early anti-VEGF treatment for hemorrhagic occlusive retinal vasculitis as a complication of cataract surgery.

Vascularites - ven, 29/12/2017 - 20:11
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Early anti-VEGF treatment for hemorrhagic occlusive retinal vasculitis as a complication of cataract surgery.

BMC Ophthalmol. 2017 Dec 06;17(1):238

Authors: Andreanos K, Petrou P, Kymionis G, Papaconstantinou D, Georgalas I

Abstract
BACKGROUND: We report a case of hemorrhagic occlusive retinal vasculitis (HORV) after prophylactic intracameral vancomycin use during an uneventful cataract surgery treated with early anti-VEGF treatment.
CASE PRESENTATION: A 51-year-old female underwent uneventful cataract surgery with prophylactic intracameral vancomycin during the procedure. On the seventh post-operative-day, she presented with sudden painful, visual loss. Fundus examination revealed peripheral hemorrhagic retinal vasculitis. She received anti-VEGF therapy to prevent further vision loss and retinal neovascularization due to extensive retinal ischemia. At the 6-month follow-up visit, visual acuity was 20/20 with no sign of neovascularization.
CONCLUSIONS: Postoperative HORV is a devastating condition that can occur after otherwise uncomplicated cataract surgery. The nature of this rare condition remains unknown. Early anti-VEGF administration seems to demonstrate favorable results.

PMID: 29212481 [PubMed - indexed for MEDLINE]

Subglottic Stenosis in Granulomatosis With Polyangiitis: The Role of Laryngotracheal Resection.

Vascularites - ven, 29/12/2017 - 20:11
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Subglottic Stenosis in Granulomatosis With Polyangiitis: The Role of Laryngotracheal Resection.

Ann Thorac Surg. 2018 Jan;105(1):249-253

Authors: Costantino CL, Niles JL, Wright CD, Mathisen DJ, Muniappan A

Abstract
BACKGROUND: Granulomatosis with polyangiitis (GPA) is associated with development of subglottic stenosis in about one-fourth of all patients. Although endoscopic management is the primary treatment method for tracheobronchial stenosis, some patients have refractory disease, and tracheostomy is required. It is unclear if laryngotracheal resection and reconstruction (LTRR) can be safely performed in patients with GPA.
METHODS: A retrospective review was performed of 11 patients with GPA undergoing LTRR.
RESULTS: Eleven female patients with GPA and a median age of 47 years underwent LTRR. Six patients were diagnosed with GPA after LTRR and had not received any induction immunosuppression regimen. Five patients had received induction immunosuppression regimen and were in clinical remission before LTRR. LTRR was performed with a protective tracheostomy in 3 patients, which was eventually removed in all. There were no major complications and no postoperative deaths. One patient (9%) failed surgical management and had replacement of a permanent tracheostomy 4 months after LTRR. Six patients (55%) required additional tracheal dilations after LTRR. Ten patients (91%) had durable control of symptoms and freedom from tracheostomy with a median follow-up of 9.7 years. Two patients (18%) experienced subsequent lower airway stenoses.
CONCLUSIONS: Surgical treatment of subglottic stenosis in highly selected patients with GPA is effective and associated with minimal morbidity. Although long-term outcomes are encouraging, additional procedures may be necessary, and patients are at risk of experiencing lower airway disease.

PMID: 29100647 [PubMed - indexed for MEDLINE]

Bilateral Arteritic Anterior Ischemic Optic Neuropathy Associated with Giant Cell Arteritis in Korea.

Vascularites - ven, 29/12/2017 - 20:11
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Bilateral Arteritic Anterior Ischemic Optic Neuropathy Associated with Giant Cell Arteritis in Korea.

Korean J Ophthalmol. 2017 10;31(5):466-467

Authors: Yoon HJ, Park SW, Lee HK, Choi YD, Heo H

PMID: 28994270 [PubMed - indexed for MEDLINE]

Alarming Internal Carotid Artery Aneurysm Eroding the Sphenoid Sinus.

Vascularites - ven, 29/12/2017 - 20:11
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Alarming Internal Carotid Artery Aneurysm Eroding the Sphenoid Sinus.

World Neurosurg. 2017 Dec;108:985.e13-985.e14

Authors: Brasiliense LBC, Dumont TM

Abstract
In rare instances, extradural internal carotid artery aneurysms can present with massive epistaxis, which is often a fatal event. We present the case of a young female with an aneurysm eroding through the sphenoid sinus and whose timely diagnosis resulted in treatment of the aneurysm with coil embolization and satisfactory outcome.

PMID: 28974410 [PubMed - indexed for MEDLINE]

Kidney Injury Molecule-1 Level is Associated with the Severity of Renal Interstitial Injury and Prognosis in Adult Henoch-Schönlein Purpura Nephritis.

Vascularites - sam, 23/12/2017 - 17:39
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Kidney Injury Molecule-1 Level is Associated with the Severity of Renal Interstitial Injury and Prognosis in Adult Henoch-Schönlein Purpura Nephritis.

Arch Med Res. 2017 Jul;48(5):449-458

Authors: Zhang Y, Li A, Wen J, Zhen J, Hao Q, Zhang Y, Hu Z, Xiao X

Abstract
BACKGROUND AND AIMS: Kidney injury molecule-1 (KIM-1) was identified the most highly upregulated protein in chronic kidney diseases and prolonged KIM-1 expression may be maladaptive. The present study was aimed to investigate urinary, renal and plasma KIM-1 levels and to analyze association between KIM-1 levels with clinical and pathological indexes in adult Henoch-Schönlein purpura (HSP) patients.
METHODS: Twenty healthy individuals, 20 HSP patients without nephritis and 35 HSP patients with nephritis were recruited. Urinary and plasma KIM-1 levels were determined by ELISA and Luminex, respectively. Renal KIM-1 expression was evaluated by immunohistochemistry.
RESULTS: HSP patients with nephritis were characterized as elevated levels of urinary, renal and plasma KIM-1. Those with more severe tubular injury of renal biopsy tissues presented significantly higher urinary and renal KIM-1 levels compared to control and patients without nephritis. Urinary and renal levels of KIM-1 were positively correlated with blood urea nitrogen and proteinuria, while they were negatively correlated with eGFR at both baseline and after two years follow-up. Moreover, plasma KIM-1 levels were associated with blood urea nitrogen and proteinuria as well. Further univariate correlation analysis indicated urinary and renal KIM-1 levels were positively correlated with interstitial inflammation index and tubulointerstitial chronicity index. Only urinary KIM-1 levels were associated with interstitial inflammation index, tubulointerstitial chronicity index and extracapillary glomerular activity index, after logistic regression analysis. The area under the curve (AUC) for urinary KIM-1/Cr predicting progression of renal damage was significantly greater than the AUC for proteinuria.
CONCLUSIONS: This finding suggests that measurement of urinary and renal KIM-1 level may be helpful to evaluate severity of renal pathological damage and prognosis in adult HSP patients with nephritis.

PMID: 29122357 [PubMed - indexed for MEDLINE]

An unusual presentation of Roth spots in Cogan's syndrome.

Vascularites - sam, 23/12/2017 - 17:39
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An unusual presentation of Roth spots in Cogan's syndrome.

Can J Ophthalmol. 2017 10;52(5):e196-e197

Authors: Phee J, Kennedy A, Saunders N, Hughes E

PMID: 28985834 [PubMed - indexed for MEDLINE]

Complement regulatory proteins in kidneys of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis.

Vascularites - sam, 23/12/2017 - 17:39
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Complement regulatory proteins in kidneys of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis.

Clin Exp Immunol. 2018 Jan;191(1):116-124

Authors: Cheng L, Gou SJ, Qiu HY, Ma L, Fu P

Abstract
The complement system activation is involved in the development of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). The study aimed to investigate the expression of complement regulatory proteins (CRPs) CD46, CD55 and CD59 in kidneys of 51 AVV patients. The expression of CD46, CD55 and CD59 in kidneys was detected by immunohistochemistry and double immunofluorescence staining. The immunohistochemical examination revealed that expression of the three CRPs could be detected in the glomeruli and tubules of both AAV patients and normal controls. The expression levels of the three CRPs in glomeruli of patients with AAV were significantly lower than those of normal controls. The scores of CD46 and CD55 expression in the tubules of AAV patients were significantly lower than those of normal controls, while there was no significant difference between the scores of CD59 expression in tubules of AAV patients and those of normal controls. Among AAV patients, the expression level of CD46 in glomeruli correlated inversely with the proportion of normal glomeruli, while it correlated with tubular atrophy in renal interstitium (r = -0·305, P = 0·026; r = 0·330, P = 0·023, respectively). The expression levels of CD55 and CD59 in glomeruli correlated with the proportion of total crescents (r = 0·384, P = 0·006; r = 0·351, P = 0·011, respectively). Double immunofluorescence staining indicated that all three CRPs were expressed on endothelial cells, podocytes and mesangial cells in glomeruli. The expression levels of the three CRPs were dysregulated in kidneys of patients with AAV. The expression levels of CD46, CD55 and CD59 were associated with the severity of renal injury of AAV patients.

PMID: 28940198 [PubMed - indexed for MEDLINE]

Circulating Endothelial Markers in Retinal Vasculopathy With Cerebral Leukoencephalopathy and Systemic Manifestations.

Vascularites - mer, 20/12/2017 - 16:26
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Circulating Endothelial Markers in Retinal Vasculopathy With Cerebral Leukoencephalopathy and Systemic Manifestations.

Stroke. 2017 Dec;48(12):3301-3307

Authors: Pelzer N, Bijkerk R, Reinders MEJ, van Zonneveld AJ, Ferrari MD, van den Maagdenberg AMJM, Eikenboom J, Terwindt GM

Abstract
BACKGROUND AND PURPOSE: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a monogenic small vessel disease, caused by C-terminal truncating TREX1 mutations, that can be considered a model for stroke and vascular dementia. The pathophysiology of RVCL-S is largely unknown, but systemic endothelial involvement has been suggested, leading to pathology in the brain and other highly vascularized organs. Here, we investigated circulating endothelial markers to confirm endothelial involvement and identify biomarkers for disease activity.
METHODS: We measured circulating levels of von Willebrand factor (VWF) antigen, VWF propeptide, and angiopoietin-2 in members of 3 Dutch RVCL-S families and matched unrelated healthy controls. Stratified analyses based on symptomatology and age were performed.
RESULTS: We found elevated levels of VWF antigen, VWF propeptide, and angiopoietin-2 in TREX1 mutation carriers (n=31) compared with family members without a TREX1 mutation (n=33) and unrelated healthy controls (n=31; Kruskal-Wallis test P<0.001 for all comparisons). Effects were most pronounced in mutation carriers with clinical manifestations aged ≥40 years (Mann-Whitney U test P<0.001 for all comparisons). Compared with healthy controls, levels of VWF antigen (P=0.02) and angiopoietin-2 (P=0.04) were also elevated in mutation carriers aged <40 years. All 3 markers showed moderate correlations with markers of kidney and liver disease and inflammation (ie, systemic symptoms of RVCL-S).
CONCLUSIONS: Our results confirm an important role of the endothelium in RVCL-S pathophysiology. VWF antigen, VWF propeptide, and angiopoietin-2 might serve as early biomarkers of disease activity. Our findings might also help to understand the pathophysiology of common neurovascular disorders, such as stroke.

PMID: 29114091 [PubMed - indexed for MEDLINE]

An Unusual Cause of Leg Pain.

Vascularites - dim, 17/12/2017 - 13:30
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An Unusual Cause of Leg Pain.

N Engl J Med. 2017 12 07;377(23):2267-2272

Authors: Kelly NP, Gerhard-Herman M, Desai AS, Miller AL, Loscalzo J

PMID: 29211675 [PubMed - indexed for MEDLINE]

The value of 18F-FDG-PET/CT in identifying the cause of fever of unknown origin (FUO) and inflammation of unknown origin (IUO): data from a prospective study.

Vascularites - dim, 17/12/2017 - 13:30
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The value of 18F-FDG-PET/CT in identifying the cause of fever of unknown origin (FUO) and inflammation of unknown origin (IUO): data from a prospective study.

Ann Rheum Dis. 2018 Jan;77(1):70-77

Authors: Schönau V, Vogel K, Englbrecht M, Wacker J, Schmidt D, Manger B, Kuwert T, Schett G

Abstract
BACKGROUND: Fever of unknown origin (FUO) and inflammation of unknown origin (IUO) are diagnostically challenging conditions. Diagnosis of underlying disease may be improved by 18F-fluorodesoxyglucose positron emission tomography (18F-FDG-PET).
METHODS: Prospective study to test diagnostic utility of 18F-FDG-PET/CT in a large cohort of patients with FUO or IUO and to define parameters that increase the likelihood of diagnostic 18F-FDG-PET/CT. Patients with FUO or IUO received 18F-FDG-PET/CT scanning in addition to standard diagnostic work-up. 18F-FDG-PET/CT results were classified as helpful or non-helpful in establishing final diagnosis. Binary logistic regression was used to identify clinical parameters associated with a diagnostic 18F-FDG-PET/CT.
RESULTS: 240 patients were enrolled, 72 with FUO, 142 with IUO and 26 had FUO or IUO previously (exFUO/IUO). Diagnosis was established in 190 patients (79.2%). The leading diagnoses were adult-onset Still's disease (15.3%) in the FUO group, large vessel vasculitis (21.1%) and polymyalgia rheumatica (18.3%) in the IUO group and IgG4-related disease (15.4%) in the exFUO/IUO group. In 136 patients (56.7% of all patients and 71.6% of patients with a diagnosis), 18F-FDG-PET/CT was positive and helpful in finding the diagnosis. Predictive markers for a diagnostic 18F-FDG-PET/CT were age over 50 years (p=0.019), C-reactive protein (CRP) level over 30 mg/L (p=0.002) and absence of fever (p=0.001).
CONCLUSION: 18F-FDG-PET/CT scanning is helpful in ascertaining the correct diagnosis in more than 50% of the cases presenting with FUO and IUO. Absence of intermittent fever, higher age and elevated CRP level increase the likelihood for a diagnostic 18F-FDG-PET/CT.

PMID: 28928271 [PubMed - indexed for MEDLINE]

Optical coherence tomography angiography in acute arteritic and non-arteritic anterior ischemic optic neuropathy.

Vascularites - dim, 17/12/2017 - 13:30
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Optical coherence tomography angiography in acute arteritic and non-arteritic anterior ischemic optic neuropathy.

Graefes Arch Clin Exp Ophthalmol. 2017 Nov;255(11):2255-2261

Authors: Balducci N, Morara M, Veronese C, Barboni P, Casadei NL, Savini G, Parisi V, Sadun AA, Ciardella A

Abstract
PURPOSE: The purpose of our study was to describe the feature of acute non-arteritic or arteritic anterior ischemic optic neuropathy (NA-AION and A-AION) using optical coherence tomography angiography (OCT-A) and to compare it with fluorescein angiography (FA) and indocyanine green angiography (ICGA).
METHODS: In this retrospective, observational case-control study four NA-AION patients and one A-AION patient were examined by FA, ICGA and OCT-A within 2 weeks from disease presentation. The characteristics of the images were analyzed. Optic nerve head (ONH) and radial peripapillary capillaries (RPC) vessel densities (VDs) were compared between NA-AION and controls.
RESULTS: In two of four NA-AION cases and in the A-AION patient, OCT-A clearly identified the boundary of the ischemic area at the level of the optic nerve head, which was comparable to optic disc filling defects detected by FA. In the other two NA-AION cases, a generalized leakage from the disc was visible with FA, yet OCT-A still demonstrated sectorial peripapillary capillary network reduction. Both ONH and RPC VDs were reduced in NA-AION patients, when compared to controls.
CONCLUSIONS: OCT-A was able to identify microvascular defects and VD reduction in cases of acute optic disc edema due to NA-AION and A-AION. OCT-A provides additional information in ischemic conditions of the optic nerve head.

PMID: 28861697 [PubMed - indexed for MEDLINE]

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