Audrey Sultan

Giant-cell arteritis: concordance study between aortic CT angiography and FDG-PET/CT in detection of large-vessel involvement

PurposeThe purpose of our study was to assess the concordance of aortic CT angiography (CTA) and FDG-PET/CT in the detection of large-vessel involvement at diagnosis in patients with giant-cell arteritis (GCA).MethodsWe created a multicenter cohort of patients with GCA diagnosed between 2010 and 2015, and who underwent both FDG-PET/CT and aortic CTA before or in the first ten days following treatment introduction. Eight vascular segments were studied on each procedure. We calculated concordance between both imaging techniques in a per-patient and a per-segment analysis, using Cohen’s kappa concordance index.ResultsWe included 28 patients (21/7 women/men, median age 67 [56–82]). Nineteen patients had large-vessel involvement on PET/CT and 18 of these patients also presented positive findings on CTA. In a per-segment analysis, a median of 5 [1–7] and 3 [1–6] vascular territories were involved on positive PET/CT and CTA, respectively (pxa0=xa00.03). In qualitative analysis, i.e., positivity of the procedure suggesting a large-vessel involvement, the concordance rate between both procedures was 0.85 [0.64–1]. In quantitative analysis, i.e., per-segment analysis in both procedures, the global concordance rate was 0.64 [0.54–0.75]. Using FDG-PET/CT as a reference, CTA showed excellent sensitivity (95%) and specificity (100%) in a per-patient analysis. In a per-segment analysis, sensitivity and specificity were 61% and 97.9%, respectively.ConclusionsCTA and FDG-PET/CT were both able to detect large-vessel involvement in GCA with comparable results in a per-patient analysis. However, PET/CT showed higher performance in a per-segment analysis, especially in the detection of inflammation of the aorta’s branches.

Large-vessel involvement and aortic dilation in giant-cell arteritis. A multicenter study of 549 patients

OBJECTIVESnLarge-vessel involvement (LVI) can occur in giant-cell arteritis (GCA) and may represent a distinct disease subgroup with a higher risk for aortic dilation. This study aimed to better characterize the presentation and evolution of LVI in patients with GCA.nnnPATIENTS AND METHODSnA retrospective multicenter study enrolled 248 GCA patients with LVI and 301 GCA patients without LVI on imaging. Factors associated with aortic dilation were identified in a multivariable model.nnnRESULTSnThe patients with LVI were younger (p<0.0001), more likely to be women (p=0.01), and showed fewer cephalic symptoms (p<0.0001) and polymyalgia rheumatica (p=0.001) but more extracranial vascular symptoms (p=0.05) than the patients without LVI. Glucocorticoids (GC) management did not differ between the two groups, but the GC discontinuation rate was lower in the patients with LVI (p=0.0003). Repeated aortic imaging procedures were performed at 19months [range: 5-162months] and 17months [range: 6-168months] after diagnosis in 154 patients with LVI and 123 patients without LVI, respectively, of whom 21% and 7%, respectively, presented new aortic dilations (p=0.0008). In the patients with LVI, aortic dilation occurred on an aorta segment shown to be inflammatory on previous imaging in 94% of patients. In the multivariate analysis, LVI was the strongest predictor of aortic dilation (hazard ratio: 3.16 [range: 1.34-7.48], p=0.009).nnnCONCLUSIONSnLVI represents a distinct disease pattern of GCA with an increased risk of aortic dilation. Control of the aortic morphology during follow-up is required.

Glutathione S Transferases Polymorphisms Are Independent Prognostic Factors in Lupus Nephritis Treated with Cyclophosphamide.

Objective To investigate association between genetic polymorphisms of GST, CYP and renal outcome or occurrence of adverse drug reactions (ADRs) in lupus nephritis (LN) treated with cyclophosphamide (CYC). CYC, as a pro-drug, requires bioactivation through multiple hepatic cytochrome P450s and glutathione S transferases (GST). Methods We carried out a multicentric retrospective study including 70 patients with proliferative LN treated with CYC. Patients were genotyped for polymorphisms of the CYP2B6, CYP2C19, GSTP1, GSTM1 and GSTT1 genes. Complete remission (CR) was defined as proteinuria ≤0.33g/day and serum creatinine ≤124 µmol/l. Partial remission (PR) was defined as proteinuria ≤1.5g/day with a 50% decrease of the baseline proteinuria value and serum creatinine no greater than 25% above baseline. Results Most patients were women (84%) and 77% were Caucasian. The mean age at LN diagnosis was 41 ± 10 years. The frequency of patients carrying the GST null genotype GSTT1-, GSTM1-, and the Ile→105Val GSTP1 genotype were respectively 38%, 60% and 44%. In multivariate analysis, the Ile→105Val GSTP1 genotype was an independent factor of poor renal outcome (achievement of CR or PR) (OR = 5.01 95% CI [1.02–24.51]) and the sole factor that influenced occurrence of ADRs was the GSTM1 null genotype (OR = 3.34 95% CI [1.064–10.58]). No association between polymorphisms of cytochrome P450s gene and efficacy or ADRs was observed. Conclusion This study suggests that GST polymorphisms highly impact renal outcome and occurrence of ADRs related to CYC in LN patients.

Is it relevant to screen young women hospitalized in psychiatric department for neuropsychiatric systemic lupus erythematosus (NPSLE)?: A prospective study of 100 psychiatric inpatients.

AbstractOn the basis that diagnosis of neuropsychiatric systemic lupus erythematosus (NPSLE) is sometimes difficult and systemic lupus erythematosus (SLE) can present with isolated psychiatric symptoms, we initiated a survey in a psychiatric department to screen for NPSLE in young female inpatients.We prospectively studied consecutive young female patients referred to the department of psychiatry. Antinuclear antibodies (ANA), anti-deoxyribonucleic acid (DNA), and antiextractable soluble nuclear antigens (ENA) in the serum of patients were screened. In case of positive anti-DNA or anti-ENA, the patient was referred to the department of internal medicine.One hundred patients were enrolled, mean age 33.1u200a±u200a8.4 years. Most patients presented underlying psychiatric disorders: depression (46%), schizophrenia (13%), anxiety disorder (6%), and personality disorder (10%). A quarter of the cohort did not display underlying psychiatric disorders before hospitalization. Positive ANA ≥1:160 were found in 32 of the 100 patients tested (32%). No patients presented anti-DNA antibodies. One patient had positive anti-sjogrëns syndrome related antigen A (SSA), but did not present any features of SLE or Sjögren syndrome.Thus, systematic screening of SLE is not relevant in young women hospitalized in psychiatric department. However, clinicians should keep in mind that SLE can present with pure psychiatric symptoms.

Factors associated with relapse and dependence to glucocorticoids in giant-cell arteritis

Introduction To identify characteristics and factors associated with relapse and glucocorticoid (GC) dependence in a large cohort of consecutive patients with giant-cell arteritis (GCA). Patients and methods We retrospectively analyzed 326 consecutive patients with GCA diagnosed between 2000 and 2016 and followed them for at least 12 months. Factors associated with relapse were identified in a Cox proportional hazards model. Factors associated with GC dependence were identified in a multivariate logistic regression analysis. Results The 326 patients (73% women; median age at diagnosis: 74 [48–92] years) were followed up for 62 [12–262] months. During follow-up, 171 (52%) patients relapsed, including 113 (35%) who developed a GC dependence. Relapsing patients more frequently showed a past history of stroke (Pxa0=xa00.01) and large-vessel involvement (LVI) on imaging (Pxa0=xa00.01) than patients without relapse. Therapeutic strategy did not differ among relapsing and non-relapsing patients. GC-dependent patients were younger (Pxa0=xa00.0001) and more frequently showed a past history of stroke (Pxa0=xa00.004) and LVI on imaging (Pxa0=xa00.005) than patients without GC-dependent disease. In multivariate analyses, LVI was an independent predictive factor of relapse [hazards ratio (HR): 1.49 (95% IC: 1.002–2.12), Pxa0=xa00.04] and GC dependence [odds ratio (OR): 2.19 (95% IC: 1.19–4.05), Pxa0=xa00.01]. Conversely, stroke was a protective factor against relapse [HR: 0.21 (95% CI: 0.03–0.68), Pxa0=xa00.005] and GC dependence disease [OR: 0.10 (95% CI: 0.001–0.31), Pxa0=xa00.0005]. Conclusion LVI was an independent predictive factor of relapse and GC dependence. Conversely, a previous stroke was protective against both unfavorable treatment outcomes. Further prospective studies are needed to confirm these findings and determine if patients with LVI require a different treatment.

Impact de la présentation clinique initiale et de l’imagerie des gros vaisseaux sur les complications cardiovasculaires précoces et tardives dans l’artérite à cellules géantes

Introduction L’arterite a cellules geantes (ACG) est une maladie proteiforme dont la presentation crânienne typique est la forme la plus frequente. Toutefois, d’autres formes plus rares de la maladie existent et sont moins bien connues. Les complications vasculaires aigues de la maladie sont dominees par les atteintes ophtalmologiques ischemiques, tandis que les complications aortiques dominent les complications vasculaires plus tardives. L’atteinte des gros vaisseaux serait egalement associee a un risque accru de mortalite cardiovasculaire. Dans cette etude, nous avons voulu decrire les differentes formes cliniques de la maladie au sein d’une grande cohorte de patients consecutivement suivis, et evaluer leur evolution en particulier en termes de complications cardiovasculaires. Patients et methodes Au sein d’une cohorte reunissant tous les patients consecutivement suivis dans les services de medecine interne des CHU de Caen et de Limoges, nous avons selectionne tous les patients dont le diagnostic d’ACG etait histologiquement prouve ou documente sur une imagerie des gros vaisseaux. Nous distinguions 4xa0formes differentes de la maladie. La premiere incluait la presentation crânienne typique isolee, sans atteinte extracrânienne. Les patients avec une atteinte crânienne et une atteinte asymptomatique des gros vaisseaux a l’imagerie etaient egalement inclus dans ce groupe. Le second groupe incluait tous les patients avec une atteinte symptomatique des gros vaisseaux, avec ou sans atteinte crânienne. Le troisieme groupe comptait tous les patients avec une presentation silencieuse de la maladie, c’est-a-dire asymptomatique mais avec une fievre et/ou un syndrome inflammatoire biologique. Le dernier groupe incluait les patients avec une PPR isolee mais avec une biopsie d’artere temporale positive et/ou une atteinte des gros vaisseaux a l’imagerie. Resultats Nous avons inclus 693xa0patients (âge median au diagnosticxa0: 75 [48–94] ans, 486 [70xa0%] femmes). La forme crânienne isolee atteignait 80xa0% (nxa0=xa0554) des patients, tandis que l’atteinte symptomatique des gros vaisseaux, la forme silencieuse et les PPR avec demonstration d’une vascularite associee concernaient 9xa0% (nxa0=xa063), 9xa0% (nxa0=xa061) et 2xa0% (nxa0=xa015) des patients, respectivement. Sur les 310xa0patients qui beneficiaient d’une imagerie des gros vaisseaux au diagnostic, 173 (56xa0%) avaient des signes de vascularites des gros vaisseaux, dont 63xa0etaient symptomatiques (deuxieme groupe). Les 173xa0patients avec une atteinte des gros vaisseaux rechutaient plus (pxa0=xa00,0002), etaient plus souvent corticodependants (pxa0=xa00,003) et faisaient plus de complications cardiovasculaires tardives (pxa0 Conclusion Parmi les differentes presentations cliniques identifiees dans cette cohorte, l’existence d’une atteinte des gros vaisseaux, particulierement lorsqu’elle est symptomatique, est associee a une evolution moins favorable, avec un risque eleve de complications cardiovasculaires.

Interest of immunodeficiency screening in adult after admission in medical intensive care unit for severe infection, a retrospective and a prospective study: the Intensive Care Unit and Primary and Secondary Immunodeficiency (ICUSPID) study

BackgroundPrimary immunodeficiency (PID) in adults is rare and mostly revealed by infections.Material and MethodsAdults without predisposing factors who were admitted to an intensive care unit (ICU) for infection were screened for PID.ResultsSix PID cases were diagnosed, mostly revealed by encapsulated bacterial infections.ConclusionInvestigation of PID after ICU discharge should be considered to improve early detection.

Giant cell arteritis presenting as isolated inflammatory response and/or fever of unknown origin: a case-control study

The objective of this study was to determine the proportion and characteristics of patients with giant cell arteritis (GCA) who present with isolated inflammatory response and/or fever of unknown origin (IFUO). Using a cohort of 693 consecutive patients in two centers with evidence of GCA on biopsy and/or imaging, we compared the characteristics and outcomes of patients with IFUO at diagnosis to a control group made up of the remaining patients with GCA. Sixty-one (9%) patients initially presented with IFUO. GCA diagnosis was proven by biopsy in 50 (82%) patients and/or imaging in 23 out of 39 (59%) patients who underwent large-vessel imaging. At diagnosis, patients with IFUO were younger (pu2009=u20090.008), had longer time to diagnosis (pu2009=u20090.001), and showed more intense inflammatory response, i.e., had higher levels of C-reactive protein (pu2009=u20090.02) and lower hemoglobin levels (pu2009=u20090.0001) than control patients. However, the therapeutic regimen did not differ between the two groups. Similarly, during a median follow-up period of 50 [0–279] months, the total rate of cardiovascular events, including ischemic cranial complications and overall outcomes, including relapse, glucocorticoids-dependence and death rates did not differ between the two groups. Five (16%) patients with initial IFUO exhibited cranial symptoms at relapse. Giant cell arteritis presenting with isolated inflammatory response and/or fever of unknown origin is a well-defined demographic and clinical pattern affecting nearly 10% of patients. This clinical form is not associated with a particular prognosis but remains a challenging diagnosis.

Artérite à cellules géantes : comparaison entre la tomographie par émission de positons et l’angioscanner aortique dans la détection des atteintes des gros vaisseaux

Introduction L’angioscanner aortique et la tomographie par emission de positons (TEP) ont tous deux demontre leur bonne sensibilite dans la detection des atteintes des gros vaisseaux lies a l’arterite a cellules geantes (ACG). L’objectif de notre etude est d’evaluer la concordance des deux examens dans la detection de ces atteintes au diagnostic. Patients et methodes A partir d’une cohorte multicentrique de patients atteints d’ACG diagnostiques entre 2010xa0et 2015, et suivis dans 3xa0services de medecine interne, nous avons selectionne les patients qui ont beneficie d’un angioscanner aortique et d’une TEP avant ou dans les 10xa0premiers jours suivant l’introduction de la corticotherapie. Huit territoires vasculaires etaient analyses sur les deux examens. La concordance entre les deux techniques d’imagerie etait calculee par patients et par territoires vasculaires en utilisant l’indicex de concordance kappa de Cohen. Resultats Nous avons inclus 28xa0patients (21/7xa0femmes/hommes, âge medianxa0: 67 [56–82] ans). Dix-neuf patients avaient une atteinte des gros vaisseaux a la TEP et 18xa0d’entre eux avaient egalement une telle atteinte sur l’angioscanner aortique. L’analyse par territoires vasculaires montrait une mediane de 5 (1–7) et 3 (1–6) segments vasculaires atteints sur les TEP et angioscanners positifs, respectivement ( p xa0=xa00,03). L’analyse qualitative, c’est a dire positivite de l’imagerie suggerant une atteinte des gros vaisseaux, montrait que le taux de concordance entre les deux examens etait de 0,85 (0,64–1). L’analyse quantitative, c’est-a-dire, une analyse par territoires vasculaires sur les deux examens, montrait un taux de concordance de 0,64 (0,54–0,75). En utilisant la TEP comme examen de reference, l’angioscanner aortique montrait une excellente sensibilite (95xa0%) et specificite (100xa0%) dans une analyse par patients. Dans une analyse par territoires vasculaires, la sensibilite et specificite de l’angioscanner etaient respectivement de 61xa0% et 98xa0%. L’angioscanner perdait en sensibilite par rapport a la TEP sur la detection de l’inflammation des branches aortiques. Conclusion L’angioscanner aortique et la TEP peuvent tous deux detecter l’atteinte des gros vaisseaux avec des resultats comparables dans une analyse par patients. En revanche, la TEP montrait des performances plus importantes dans une analyse par territoires vasculaires, principalement dans la detection de l’inflammation des branches aortiques.

Ehlers-Danlos Syndrome, Hypermobility Type: Impact of Somatosensory Orthoses on Postural Control (A Pilot Study)

Elhers-Danlos syndrome (EDS) is the clinical manifestation of connective tissue disorders, and comprises several clinical forms with no specific symptoms and selective medical examinations which result in a delay in diagnosis of about 10 years. The EDS hypermobility type (hEDS) is characterized by generalized joint hypermobility, variable skin hyperextensibility and impaired proprioception. Since somatosensory processing and multisensory integration are crucial for both perception and action, we put forth the hypothesis that somatosensory deficits in hEDS patients may lead, among other clinical symptoms, to misperception of verticality and postural instability. Therefore, the purpose of this study was twofold: (i) to assess the impact of somatosensory deficit on subjective visual vertical (SVV) and postural stability; and (ii) to quantify the effect of wearing somatosensory orthoses (i.e., compressive garments and insoles) on postural stability. Six hEDS patients and six age- and gender-matched controls underwent a SVV (sitting, standing, lying on the right side) evaluation and a postural control evaluation on a force platform (Synapsys), with or without visual information (eyes open (EO)/eyes closed (EC)). These two latter conditions performed either without orthoses, or with compression garments (CG), or insoles, or both. Results showed that patients did not exhibit a substantial perceived tilt of the visual vertical in the direction of the body tilt (Aubert effect) as did the control subjects. Interestingly, such differential effects were only apparent when the rod was initially positioned to the left of the vertical axis (opposite the longitudinal body axis). In addition, patients showed greater postural instability (sway area) than the controls. The removal of vision exacerbated this instability, especially in the mediolateral (ML) direction. The wearing of orthoses improved postural stability, especially in the eyes-closed condition, with a particularly marked effect in the anteroposterior (AP) direction. Hence, this study suggests that hEDS is associated with changes in the relative contributions of somatosensory and vestibular inputs to verticality perception. Moreover, postural control impairment was offset, at least partially, by wearing somatosensory orthoses.