Ischemic Cerebellar Stroke in a 4-Year-Old Boy After Chickenpox: An Atypical Vascular Involvement

Objective. To determine active TGF-β1 (aTGF-β1) levels in serum, skin, and peripheral blood mononuclear cell (PBMC) culture supernatants and to understand their associations with clinical parameters in systemic sclerosis (SSc) patients.Methods. We evaluated serum samples from 56 SSc patients and 24 healthy controls (HC). In 20 SSc patients, we quantified spontaneous or anti-CD3/CD28 stimulated production of aTGF-β1 by PBMC. The aTGF-β1 levels were measured by ELISA. Skin biopsies were obtained from 13 SSc patients and six HC, and TGFB1 expression was analyzed by RT-PCR.Results. TGF-β1 serum levels were significantly higher in SSc patients than in HC (p< 0.0001). Patients with increased TGF-β1 serum levels were more likely to have diffuse subset (p= 0.02), digital ulcers (p= 0.02), lung fibrosis (p< 0.0001), positive antitopoisomerase I (p= 0.03), and higher modified Rodnan score (p= 0.046). Most of our culture supernatant samples had undetectable levels of TGF-β1. No significant difference in TGFB1 expression was observed in the SSc skin compared with HC skin.Conclusion. Raised active TGF-β1 serum levels and their association with clinical manifestations in scleroderma patients suggest that this cytokine could be a marker of fibrotic and vascular involvement in SSc.

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Vascular Involvements in Cholangiocarcinoma: Tips and Tricks

Cancers ◽

10.3390/cancers13153735 ◽

2021 ◽

Vol 13 (15) ◽

pp. 3735

Author(s):

Roberta Angelico ◽

Bruno Sensi ◽

Alessandro Parente ◽

Leandro Siragusa ◽

Carlo Gazia ◽

...

Keyword(s):

Portal Vein ◽

Hepatic Artery ◽

Vena Cava ◽

High Volume ◽

Vascular Reconstruction ◽

Ex Situ ◽

Vascular Involvement ◽

Hepatic Veins ◽

Hepatic Artery Resection ◽

Artery Resection

Cholangiocarcinoma (CCA) is an aggressive malignancy of the biliary tract. To date, surgical treatment remains the only hope for definitive cure of CCA patients. Involvement of major vascular structures was traditionally considered a contraindication for resection. Nowadays, selected cases of CCA with vascular involvement can be successfully approached. Intrahepatic CCA often involves the major hepatic veins or the inferior vena cava and might necessitate complete vascular exclusion, in situ hypothermic perfusion, ex situ surgery and reconstruction with autologous, heterologous or synthetic grafts. Hilar CCA more frequently involves the portal vein and hepatic artery. Resection and reconstruction of the portal vein is now considered a relatively safe and beneficial technique, and it is accepted as a standard option either with direct anastomosis or jump grafts. However, hepatic artery resection remains controversial; despite accumulating positive reports, the procedure remains technically challenging with increased rates of morbidity. When arterial reconstruction is not possible, arterio-portal shunting may offer salvage, while sometimes an efficient collateral system could bypass the need for arterial reconstructions. Keys to achieve success are represented by accurate selection of patients in high-volume referral centres, adequate technical skills and eclectic knowledge of the various possibilities for vascular reconstruction.

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CAT FINDINGS IN A CEREBELLAR STROKE RESULTING IN PALATAL MYOCLONUS

The Medical Journal of Australia ◽

10.5694/j.1326-5377.1977.tb130814.x ◽

1977 ◽

Vol 1 (13) ◽

pp. 462-462

Keyword(s):

Palatal Myoclonus ◽

Cerebellar Stroke

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From Cerebellar Apoplexy in 1849 to Cerebellar Stroke in the 2020s: Robert Dunn’s Contribution

The Cerebellum ◽

10.1007/s12311-021-01240-z ◽

2021 ◽

Author(s):

Mario Manto ◽

Lazaros C. Triarhou

Keyword(s):

Cerebellar Stroke

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Characterization and clinical implications of peripheral arterial involvement in transthyretin cardiac amyloidosis cardiomyopathy

European Heart Journal ◽

10.1093/ehjci/ehaa946.3247 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

K Stamatelopoulos ◽

D Delialis ◽

D Bampatsias ◽

M.E Tselegkidi ◽

I Petropoulos ◽

...

Keyword(s):

Risk Factors ◽

Clinical Significance ◽

Vascular Involvement ◽

Control Group ◽

Peripheral Vascular ◽

Cvd Risk Factors ◽

Wave Reflections ◽

Cvd Risk ◽

Arterial Involvement ◽

Peripheral Arterial

Abstract Background The sporadic form of transthyretin amyloidosis cardiomyopathy (ATTR-CM) is underdiagnosed but its prevalence is increasing due to the aging population. Given the poor prognosis of ATTR-CM understanding the underlying pathophysiologic mechanisms of the disease is imperative in order to improve strategies for early diagnosis and risk stratification and to develop new effective therapeutic options. ATTR-CM is associated with hypotension and there is preliminary experimental evidence of vascular involvement but its presence and clinical significance remains unknown. Purpose To characterize peripheral arterial involvement and explore its clinical role in ATTR-CM. Methods We consecutively recruited 28 previously untreated patients with newly diagnosed ATTR-CM and 34 elderly controls >70 years old, without ATTR-CM or heart failure. In both groups, flow-mediated dilatation (FMD) and intima-media thickness (IMT) in the carotid arteries were measured by high-resolution ultrasonography as markers of peripheral vascular reactivity and of subclinical atherosclerosis, respectively. Carotid-femoral pulse wave velocity (PWV) was measured as a marker of arterial stiffness. Aortic blood pressure (BP) and augmentation index (AI) using applanation tonometry were measured as markers of arterial wave reflections, peripheral arterial resistance and central hemodynamics. Echocardiography was performed in all ATTR patients. All cardiovascular (CV) measurements were performed before administration of any ATTR-specific therapy. Results ATTR patients were older and had lower prevalence of hypertension and male gender (p<0.05 for all) than the control group. Aortic and peripheral BP (p=0.016–0.088) and AI (p=0.003) were lower in ATTR patients. IMT in the common (cc) and internal carotid (ic) as well as in the carotid bulb (cb) were significantly higher in ATTR patients (p=0.001–0.042). After multivariable adjustment for traditional CV disease (CVD) risk factors, the ATTR group was independently associated with AI and IMT in cc, cb and ic (p<0.05 for all). In a subgroup of subjects with similar age between groups (n=13 and n=33 and 74.5±2.9 vs. 75.6±3.6 years, for ATTR vs. controls, respectively) differences in AI and cbIMT remained significant. Interestingly, AI was strongly and inversely associated with interventricular wall thickness (IVwt) in ATTR patients (spearman rho=−0.651, p=0.001). After adjustment for traditional CVD risk factors this association remained significant. Conclusion ATTR-CM is associated with lower aortic wave reflections, which correlate with more advanced structural cardiac disease, as assessed by IVwt. Further, ATTR-CM patients present accelerated subclinical carotid atherosclerosis as compared to elderly control subjects. These findings suggest that in ATTR-CM there is disease-specific peripheral vascular involvement in parallel to cardiac involvement. The clinical significance of these findings merits further investigation. Funding Acknowledgement Type of funding source: None

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AB0608 CARDIAC VESSELS CALCIFICATION IN A COHORT OF SYSTEMIC SCLEROSIS PATIENTS: POSSIBLE ROLE IN VASCULOPATHY AND HEART ABNORMALITIES.

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5746 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1600.2-1600

Author(s):

S. Sciacca ◽

C. Rotondo ◽

A. Corrado ◽

L. Giardullo ◽

S. Stefania ◽

...

Keyword(s):

Uric Acid ◽

Systemic Sclerosis ◽

Disease Duration ◽

Vascular Complications ◽

Hypovitaminosis D ◽

Older Age ◽

Vascular Involvement ◽

Cvd Risk ◽

1 John ◽

The Impact

Background:Cardiovascular disease is the leading cause of morbidity and mortality worldwide. Myocardial calcifications have been related with cardiovascular diseases (CVD) such as focal wall motion abnormalities and arrhythmias. The impact of vascular calcifications is under investigation in order to define the risk of cardiovascular events. The relationship between cardiac calcification and systemic sclerosis (SSc) has not been investigated.Objectives:The aim of the study is to evaluate the frequency of different patterns of cardiac calcification in SSc patients, and to correlate them to other CVD risk factors.Methods:We analyzed thoracic-CT scanners of 35 SSc patients (88% female, aged 47,8 ys ±12,9, disease duration 12,8 ys ±9) to determine the location and extension of vascular and cardiac calcification. All recruited patients fulfilled the 2013 ACR-EULAR classification criteria for SSc. No one patients had renal failure, cardiomyopathy, myocarditis, history of cardiac surgery or radiotherapy.Results:We found myocardial vessels calcifications (MCv) in 37% SSc patients, aortic wall calcifications (ACw)in 60% SSc patients, cardiac valve calcifications (VC) in 28% SSc patient and heart wall calcifications (HCw) in 20%.The SSc patients with almost one calcification had older age (65±9,8 ys vs 50±8,8 ys; p=0,0001) and higher values of circulating NTproBNP (336,9±351,9 vs 144,2±107,8; p=0,04) compared to those without.In particular, the SSc patients with MCv had and uric acid (5,3 ±1,5 vs 4,1 ±1,3; p=0,05), higher rate of PAH (25% vs 0%; p=0,037), arrhythmia (38,5% vs 9%; p=0,036) and higher prevalence of CENP-B antibodies(46% vs 4%; p=0,01) compared to patients without MCv.Patients with HCw had lower C reactive protein (0,16 ±0,10 vs 0,7±0,7; p=0,008) compared to those without HCw. No differences in the rate of heart and vascular complications of SSc were observed.The SSc patients with ACw had higher frequency of arrhythmia (33% vs 0%; p=0,016) and longer disease duration (15,5 y ±9,9 vs 8,8 ±5,8; p=0,03).The SSc patients with VC had higher rate of PAH (33%vs0%; p=0,003) and uric acid (6±0,5vs3,8±1,2 p=0,0001).Regression analysis excluded any association with gender, BMI, systemic arterial hypertension, steroid therapy, hypovitaminosis D or smoke habit. No cardiovascular event was recorded in one year of observation.Conclusion:All patterns of calcifications may be related mostly with the older age. Myocardial vessels calcifications have been found in a high percentage of SSc patients and in particular in those with PAH and positive for anti CENP-B. Furthermore, myocardial vessels calcifications could be associated to the higher occurrence of arrhythmia. More studied are needed to assess the importance of vascular calcification as a part of the vascular involvement in SSc.References:[1]John W. Nance Jr. MD. Myocardial calcifications: Pathophysiology, etiologies, differential diagnoses, and imaging findings. Journal of Cardiovascular Computed Tomography 9 (2015) 58 e 67.[2]Pagkopoulou E, Poutakidou M. Cardiovascular risk in systemic sclerosis: Micro- and Macro-vascular involvement. Indian J Rheumatol 2017;12, Suppl S1:211-7[3]Plastiras SC, Toumanidis ST. Systemic sclerosis: the heart of the matter. Hellenic J Cardiol. 2012;53(4):287–300.Disclosure of Interests:None declared

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Potential Biomarkers in Systemic Sclerosis: A Literature Review and Update

Journal of Clinical Medicine ◽

10.3390/jcm9113388 ◽

2020 ◽

Vol 9 (11) ◽

pp. 3388

Author(s):

Akira Utsunomiya ◽

Noritaka Oyama ◽

Minoru Hasegawa

Keyword(s):

Systemic Sclerosis ◽

Brain Natriuretic Peptide ◽

Natriuretic Peptide ◽

Surfactant Protein ◽

Vascular Involvement ◽

Surfactant Protein D ◽

Plasma Cytokines ◽

Precise Diagnosis ◽

Pulmonary Arterial ◽

Chronic Autoimmune Disease

Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by dysregulation of the immune system, vascular damage, and fibrosis of the skin and internal organs. Patients with SSc show a heterogeneous phenotype and a range of clinical courses. Therefore, biomarkers that are helpful for precise diagnosis, prediction of clinical course, and evaluation of the therapeutic responsiveness of disease are required in clinical practice. SSc-specific autoantibodies are currently used for diagnosis and prediction of clinical features, as other biomarkers have not yet been fully vetted. Krebs von den Lungen-6 (KL-6), surfactant protein-D (SP-D), and CCL18 have been considered as serum biomarkers of SSc-related interstitial lung disease. Moreover, levels of circulating brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) can provide diagnostic information and indicate the severity of pulmonary arterial hypertension. Assessment of several serum/plasma cytokines, chemokines, growth factors, adhesion molecules, and other molecules may also reflect the activity or progression of fibrosis and vascular involvement in affected organs. Recently, microRNAs have also been implicated as possible circulating indicators of SSc. In this review, we focus on several potential SSc biomarkers and discuss their clinical utility.

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P158 Severity of hand vascular involvement as assessed by MRI Digital Artery Volume Index (DAVIX©) predicts worsening of clinical parameters and patient reported outcomes in scleroderma

Rheumatology ◽

10.1093/rheumatology/keaa111.153 ◽

2020 ◽

Vol 59 (Supplement_2) ◽

Author(s):

Fiammetta Danzo ◽

Klodian Gjeloshi ◽

Giovanni Lettieri ◽

Giuseppina Abignano ◽

Mark Hinton ◽

...

Keyword(s):

Disease Activity ◽

Health Assessment Questionnaire ◽

Patient Reported Outcomes ◽

Health Assessment ◽

Volume Index ◽

Vascular Involvement ◽

Clinical Parameters ◽

Digital Artery ◽

Patient Reported ◽

Assessment Questionnaire

Abstract Background Neointima proliferation is a key pathologic feature of systemic sclerosis (SSc), causing arterial vessel narrowing and being the recognised culprit pathological lesion in digital ulcers (DUs), pulmonary artery hypertension and renal crisis. Nevertheless, there are no validated imaging techniques to assess the severity of vascular involvement in SSc. We have previously shown digital artery volume index (DAVIX©) assessed with time of right MRI angiography, is a reliable measure of neointima proliferation in the hands. The purpose of our study was to identify the value of DAVIX© in predicting worsening of patient reported outcomes (PROs) and clinical parameters in SSc. Methods Cross-sectional data were available for 91 patients and complete 12 months follow-up data for 68 patients. Data collected included: modfied Rodnan skin score (mRSS), pulmonary function tests (PFTs), echocardiography, nailfold capillaroscopy, Health Assessment Questionnaire Disability Index (HAQ-DI), and Scleroderma Health Assessment Questionnaire (sHAQ). DAVIX© of the dominant hand was calculated as the % mean of the 4 fingers, employing MeVisLab software. Following analysis of distribution, Spearman or Pearson test were used to determine correlation coefficients, as appropriate (Prism 7). Results 56/68 were female and median of disease duration was 4 years (IQR 1.91-9). As previously reported DAVIX© correlated with the presence of DUs (p = 0.0093). Considering all patients, DAVIX© correlated with mRSS (r=-0.258, p = 0.017), DLCO% (r = 0.338, p = 0.008) and the pattern of capillaroscopy (r=-0.388, p = 0.001). In patients with DUs, DAVIX© showed a stronger correlation with DLCO% (r = 0.786, p = 0.048). Most importantly, DAVIX© predicted the worsening of HAQ-DI (r=-0.295, p = 0.029), sHAQ (r =-0.333, p = 0.029) and VAS pain (r=-0.269, p = 0.038) independently of the presence of DUs. Conclusion The quantitative assessment of neointima proliferation in the hand by DAVIX© is a useful imaging biomarker of vascular disease activity. The value of DAVIX© in predicting the worsening of PROs and clinical parameters in overall patients, may offer insights on the role of vascular disease activity in the global progression of SSc. The validation of our data in an independent cohort and the sensitivity to change over time of DAVIX© may aid to the implementation of hand MRI as imaging outcome measure of vascular severity in SSc. Disclosures F. Danzo None. K. Gjeloshi None. G. Lettieri None. G. Abignano None. M. Hinton None. A. Dean None. G. Cuomo None. O. Kubassova None. F. del Galdo None.

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