Il existe au cours du lupus érythémateux systémique (LES) une surmortalité cardiovasculaire (CV) par une athérosclérose majeure et précoce. Les facteurs de risque CV traditionnels jouent un rôle ...important, mais n’expliquent pas totalement cette majoration du risque CV.
Rapporter les incidences de mortalité CV et d’accidents vasculaires ainsi que la prévalence de l’athérosclérose infraclinique au cours du lupus. Les différents facteurs de risque impliqués dans cette athérosclérose sont inhérents, en plus des facteurs de risque CV traditionnels, à la présentation de la maladie et aux thérapeutiques utilisées. Les rôles de l’inflammation chronique et de l’auto-immunité sont également détaillés.
Les accidents CV et l’athérosclérose infraclinique sont très fréquents au cours du lupus systémique. Les patients lupiques ont 5 à 6 fois plus de risque de développer un infarctus du myocarde, et ce risque est particulièrement marqué chez les femmes de 35 à 44ans où il dépasse 50. L’athérosclérose infraclinique est également surreprésentée et près d’un tiers des patients ont des plaques carotidiennes et/ou des calcifications coronaires. Les facteurs de risque classiques (HTA, diabète, hyperlipidémie, obésité, tabac) n’expliquent qu’une partie de cet excès de risque CV, car 50 % des patients avec athérosclérose avancée n’ont aucun de ces facteurs de risque. Certains facteurs spécifiques du lupus apparaissent comme significativement associés à ce phénomène : un âge avancé lors du diagnostic, la présence d’une atteinte rénale, l’ancienneté et l’activité de la maladie. Il existe également au cours du lupus une dyslipidémie fréquente, majorée par les poussées inflammatoires, associant hypertriglycéridémie, baisse du HDL-cholestérol avec production d’un HDL pro-inflammatoire athérogène. L’auto-immunité pourrait avoir une part de responsabilité, mais les données en faveur de cette hypothèse sont relativement pauvres. D’autres anomalies communes à la pathogénie de l’athérosclérose et du lupus sont rapportées : augmentation du stress oxydatif, activation du complément et dépôt d’immuns complexes. Le rôle de la corticothérapie est complexe à interpréter, avec un effet délétère reconnu au plan métabolique mais une action potentiellement bénéfique dans le contrôle de l’inflammation, de l’activité de la maladie et de l’atteinte rénale. Les antipaludéens de synthèse ont un rôle athéro-protecteur émergeant, en plus de leurs bénéfices sur la maladie ; et l’utilisation d’immunosuppresseurs (cyclophosphamide et mycophénolate mofétil) semble s’accompagner d’un meilleur pronostic sur la progression de l’athérosclérose. Enfin, et sur les dernières données, malgré leur intérêt prouvé en prévention primaire de l’athérosclérose dans une population générale ciblée, les statines données systématiquement n’ont pas démontré actuellement de bénéfice sur l’amélioration du risque CV au cours du LES.
Les mécanismes de l’athérosclérose au cours du lupus sont multiples et non encore totalement élucidés. Elle est en partie médiée par des facteurs inhérents au lupus lui-même, en plus des facteurs de risque CV traditionnels. Une stratégie de prévention précoce de cette athérosclérose devra viser ces différents paramètres.
Evidence from epidemiological studies demonstrates that patients with systemic lupus erythematosus (SLE) are at increased risk for the development of cardiovascular disease. Traditional cardiovascular risk factors’ play an important role in this phenomenon but do not account for the entire risk in lupus patients.
The incidence and prevalence of cardiovascular events and infraclinical atherosclerosis are reviewed. Combinations of traditional risk factors with lupus-specific and treatment-related variables are detailed.
Atherosclerosis is more prevalent and occurs prematurely in lupus patients. Relative risk of myocardial infarction is between 5 to 8 times greater that of general population, and may exceed 50 in women between 35 and 44 years old. SLE was also found as an independent risk factor for subclinical atherosclerosis, and more than one third of lupus patient show evidence of carotid plaques of coronary artery calcifications. Lupus patients have more frequent traditional risk factors compared with general population of similar age and sex. Besides the traditional risk factors, SLE specific risk factors have been identified among witch advanced age at diagnosis, current disease activity, duration of the disease and renal activity. Moreover, lipid abnormalities in patients with SLE are common and likely are one of the major causes of premature atherosclerosis in these patients; the dyslipoprotein associated increased triglycerides and depressed HDL-cholesterol with proinflammatory HDL production. Autoimmunity may have a part of responsibility, but data's in favour of this hypothesis are not strong. Other mechanisms such as vascular inflammation, oxidative stress, immune complexes and complement activation may also elicit endothelial damage and promote atherosclerosis are associated with the pathogenesis of both SLE and atherosclerosis. Steroids may have a true double-edged role with a pro-atherogenic risk regarding the exacerbation of metabolic risk factors and a “beneficial” anti-inflammatory role. It is becoming increasingly apparent that antimalarials treatment in SLE has an atheroprotective and a cardioprotective effect. The other immunosuppressive drugs may reduce progression of atherosclerosis and cardiovascular events but their precise role remains to be elucidated. Despite their role in primary prevention in target general population, for now, systematic prescription of statins does not show a great benefit in the cardiovascular risk in lupus patients.
Mechanisms of atherosclerosis in SLE remain elusive. It is partially explained by the interaction of traditional cardiovascular risk factors, lupus-specific factors and therapy specially corticosteroids. Management strategies of lupus should include early all those items.
Systemic lupus erythematosus (SLE), a multisystem autoimmune disease with protean manifestations, occurs with neuropsychiatric manifestations in </=60% of patients. Myelitis is a rare but serious ...complication of SLE. The diagnosis of myelitis may be difficult, but magnetic resonance imaging is generally very useful in assessing the extension and severity of lupus-related myelitis. We present the case of a 22-year-old woman with SLE since age 18 who presented with bilateral leg paresis of 7 days' duration.
Abstract
Background/Aims
Behçet’s disease (BD) is a relapsing vasculitis that can affect vessels of all sizes and kinds. Intracardiac thrombosis (ICT) is a rare but serious complication which often ...presents with vascular lesions. Yet, the link between these manifestations is not fully clarified. Our study aims to describe the profile, therapeutic management, and outcomes of BD patients with ICT. Secondly, it aims to shed some light on the relationship between ICT and vascular involvement in BD.
Methods
We retrospectively conducted a descriptive and monocentric study, including 531 Behçet’s patients admitted to our department between 2010 and 2022. All patients selected fulfilled the criteria of the International Study Group of Behçet’s Disease.
Results
Twelve patients were enrolled (2%). The sex ratio (M:F) was 9:1 and the mean age was 29 ± 6 years (20-41). ICT occurred as the revealing form of BD (50%), or after a median of 4 years since the disease onset (50%). The main symptoms were dyspnea (58%), chest pain (58%), hemoptysis (41%), and dry cough (33%). Concurrent BD flares were mainly mucocutaneous (80%). Inflammatory parameters were elevated with a median ESR of 48 mm/h and CRP of 52 mg/L. High levels of BNP and troponin were noted in 58% and 16% of the cases. Hemostasis parameters were within the normal range. On transthoracic echocardiogram, ICT was located in the right (100%), and left cavities (20%), with an extension to the inferior vena cava in half of the cases (50%). Multiple thrombi were visualized (n = 4). The associated cardiac lesions were tricuspid insufficiency (50%), pericardial effusion (41%), mitral insufficiency (16%), endomyocardial fibrosis (8%), myocarditis (8%), and ischemic coronaropathy (8%). Pulmonary hypertension (58%) and right dilatation (50%) were common. Concomitant pulmonary artery involvement was frequent (83%), consisting of thrombosis (70%) and aneurysms (50%). Six patients had a past history of lower extremity DVT, either as a single episode (n = 4) or recurrent episodes (n = 2). Vascular thrombosis was also found in the superior vena cava (n = 2), suprahepatic veins (n = 2), internal jugular vein (n = 1), pulmonary veins (n = 1), cerebral vein (n = 1), superior mesenteric vein (n = 1), and femoral artery (n = 1). Patients were treated with colchicine (100%), glucocorticoids (100%), and immunosuppressants (92%), mainly cyclophosphamide (n = 11), and a TNF-alpha inhibitor (n = 2). Six patients were under curative anticoagulation. A good response to therapy was observed in all patients. One case of relapse was reported. No deaths were recorded.
Conclusion
ICT usually occurs during the first years of BD onset in young male patients presenting with vascular lesions, especially of the pulmonary arteries and vena cava. We thus believe that, unlike common thrombotic diseases, ICT in BD is a direct consequence of the underlying vasculitis. The use of immunosuppressants, with or without anticoagulants, is generally associated with a favorable outcome.
Disclosure
S. Chadli: None. H. Khibri: None. S. Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.
Abstract
Background/Aims
Behçet’s syndrome (BS) is a multi-systemic vasculitis highly prevalent in a broad range stretching from the Mediterranean region to eastern Asia. The vascular involvement, ...present in up to 40% of the cases, is characterised by a high tendency towards the venous system and thrombosis. Although pulmonary artery aneurysms (PAA) are considered to be rare (less than 5% in cohorts), they are a quasi-pathognomonic sign of BS and play a major role in patient’s morbidity and long-term mortality. Our work aims to depict the clinical profile, imaging features, therapeutic management, and outcomes of BS patients presenting with PAA.
Methods
We conducted a retrospective, descriptive and monocentric study including 531 BS patients admitted to the internal medicine department between 2000 and 2021. All patients fulfilled the International Study Group Criteria for Behçet’s disease. We enrolled all cases with documented PAA on CT angiography of the chest (CTA chest).
Results
Among 531 BS patients, 51 had PAA (10%). The mean age was 36 ± 11 20-69 and the sex ratio (M:F) was 4:1. Cardiovascular risk factors comprised chronic tobacco consumption (13.5%) and high blood pressure (9.6%). PAA occurred after a median of 5.5 years following the disease onset. Patients were either asymptomatic (78%) or presented with hemoptysis (6%), dry cough (6%), chest pain (4%), and dyspnea (2%). On CTA chest, PAA were commonly multiple (78%), proximal (46%), and bilateral (29%). Concomitant arterial manifestations were observed in 25% of the patients, consisting of peripheral arteries aneurysms (17%), pulmonary artery thrombosis (7.7%), cerebral artery thrombosis (4%), and aortic aneurysms (2%). Venous thrombosis was also simultaneously found in 21% of the cases, observed in the superior (9.6%) and inferior vena cava (9.6%), cerebral veins (7.7%), and lower extremity veins (6%). On echocardiography, right intracardiac thrombosis was visualised (17%). The other extravascular involvements were mainly mucocutaneous (73%), ocular (46%), articular (29%), and neurological (10%). Our patients were treated with colchicine (100%) and an initial pulse of methylprednisolone (15mg/kg/day for three days), followed by oral prednisolone (1 mg/kg/day), gradually tapered (94%). The immunosuppressive therapy administered consisted of cyclophosphamide (38.5%), azathioprine (31%), and infliximab, a TNF alpha inhibitor (2%). Curative anticoagulation was prescribed in 21% of the cases. Only one patient underwent surgery. Relapse and death were recorded in respectively 15% and 6% of the cases.
Conclusion
PAA are a very unique feature of BS, with specific characteristics related to their inflammatory pathogenesis. We highly recommend evoking the diagnosis of BD in presence of PAA, especially in young male patients presenting with other vascular lesions and intracardiac thrombosis. The prompt initiation of anti-inflammatory and immunosuppressive therapy is of paramount importance to reduce the risk of massive hemorrhage from PAA rupture.
Disclosure
S. Chadli: None. H. Khibri: None. S. Fari Firdaous: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.
Abstract
Background/Aims
Amyloidosis is a broad spectrum of heterogeneous diseases, characterized by the accumulation and extracellular deposit of misfolded proteins, resulting in organs’ thickening ...and dysfunction. Cardiac amyloidosis (CA) is common and represents an important key factor in patients’ prognosis. Our study aims to depict the profile of patients with CA, so as present their therapeutic management and outcomes.
Methods
We retrospectively carried out a descriptive study between 2015 and 2022, including all patients admitted to our department for systemic amyloidosis (SA) with confirmed cardiac involvement on echocardiography (TTE) and/or cardiac magnetic resonance (CMR).
Results
31 patients were enrolled. The sex ratio (M/F) was 0.93 and the mean age was 62 ± 15 years (26-86). Past history included multiple myeloma (n = 7), monoclonal gammapathy of undetermined significance (n = 1), T lymphoma (n = 1), and systemic amyloidosis (n = 1). Cardiovascular risk factors comprised high blood pressure (25%), type 2 diabetes (15%), dyslipidemia (9%), and smoking (8%). Patients presented after a mean of 12 ± 4 months since the onset, with peripheral edema (68%) and other signs of right (53%) and global heart failure (19%). Concomitant extracardiac involvements were mainly renal (55%), mucocutaneous (20%), and neurological (16%). Electrocardiogram showed low-voltage (41%), poor R-wave progression (23%), atrial fibrillation (10%), atrioventricular block (6%), right bundle branch block (6%), left bundle branch block (6%), junctional tachycardia (6%), and left ventricular hypertrophy (LVH) (6%). On TTE, the features observed were the altered longitudinal global strain (93%) with a bull’s eye pattern (90%), LVH (90%), valvular and septal hypertrophy (88%), sparkling myocardium (65%), pericardial effusion (54%), diastolic dysfunction (37%), dilated cavities (35%), pulmonary hypertension (29%), and aortic stenosis (3%). Systolic function was mostly conserved (73%), at a mean of 53 ± 10%. The most recurrent signs on CMR were: late enhancement (90%), LVH (80%), and abnormalities in myocardium nulling (25%). Cardiac biomarkers included a Troponin of 0.35 ng/ml 0.13; 506 and BNP of 4895 pg/ml 1588; 13 224. BS showed positive cardiac fixation (grade 2 or 3) in six cases, confirming ATTR-CA. Peripheral biopsies, serum and urine immunofixation, free light chains assays, and medullar investigations demonstrated AL in 23 patients. Two cases of AA were confirmed on immunohistochemistry, complicating sarcoidosis (n = 1) and ankylosing spondylitis (n = 1). Patients were treated with chemotherapy (n = 11), Tafamidis (n = 3), and Infliximab (n = 2). The outcomes documented were complete remission (n = 1), partial remission (n = 3), and death (n = 9) with a median survival of 7 months.
Conclusion
Our study highlights the grim prognosis still carried by CA patients. Although increased awareness is noticed among physicians, the disease is often discovered at a late stage or even overlooked. Further understanding of CA and prompt screening of at-risk patients are thus crucial for early diagnosis and management, in order to improve patients' outcomes.
Disclosure
S. Chadli: None. M. Maamar: None. H. Khibri: None. N. Moatassim: None. W. Ammouri: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.
Les étiologies des méningites sont surtout infectieuses. Les causes non infectieuses, sont souvent de diagnostic difficile et incertain, tel est le cas de la maladie de Behçet. Nous rapportons deux ...observations de patients ayant présenté des épisodes récurrents de méningite. Dans un premier temps, la suspicion d'une étiologie infectieuse a conduit à introduire un traitement anti-infectieux probabiliste. La découverte à posteriori d'une aphtose bipolaire a permis de poser le diagnostic de maladie de Behçet. Une corticothérapie s'est révélée efficace. La maladie de Behçet doit toujours faire partie des diagnostics différentiels des méningites récurrentes.
Abstract
Background/Aims
Behçet’s syndrome (BS) is a complex inflammatory disorder frequently observed in the so-called “Silk Road”. Venous thrombosis (VT) is common in BS and known to be a ...predictive factor of future severe manifestations of the disease. Our work aims to describe the clinical and imaging characteristics of VT in BS, in order to shed the light on its specific features for early diagnosis and management of these patients.
Methods
We conducted a retrospective, descriptive and monocentric study, including 200 BS patients admitted to our internal medicine department between 2014 and 2021. All patients fulfilled the International Study Group criteria for Behçet’s disease.
Results
VT was found in 62 patients (31%). The mean age was 29 ± 10.8 (18-61) and the sex ratio (M:F) was 7.85:1. Family history of BS was documented in 5% of the cases. VT was either the revealing manifestation of the syndrome (56%) or occurred after a median of 3.5 years (1; 6.5) following the disease onset. Four patients had superficial venous thrombosis (SVT) mostly affecting the superficial femoral vein (98%). All patients had deep vein thrombosis (DVT), visualised in the lower extremities (58%), superior vena cava (21%), inferior vena cava (16%), cerebral veins (13%), and suprahepatic veins (5%). Atypical sites of DVT occurred in the renal (n = 1), axillary (n = 1), central retinal (n = 1), and dorsal penile (n = 1) veins. DVT was observed in one vein (55%), two veins (27%), or multiple (3 to 7) veins (16%). Thrombi sizes ranged from 10 to 40 mm, being willingly extended (55%) and strongly adherent to the vessel walls (95%). Concomitant arterial lesions were found in 34% of the patients, mainly in the pulmonary arteries (57%). They consisted of aneurysms (52%), thrombosis (43%), stenosis (17%), and pseudoaneurysms (9%). Cardiac lesions were also observed (11%), including right intracardiac thrombosis (n = 4), coronary artery thrombosis (n = 2), pericarditis (n = 1), and myocarditis (n = 1). The other extravascular involvements associated were mucocutaneous (100%), ocular (42%), neurological (29%), articular (24%), and digestive (23%). Fever was reported in 4 cases. All patients were treated with colchicine and glucocorticoids. Immunosuppressive therapy was prescribed in 75% of the cases: cyclophosphamide (n = 31), azathioprine (n = 12), and TNF alpha inhibitor (n = 3). Curative anticoagulation was also administered (98%). Two patients underwent surgery: thrombectomy (n = 1) and embolectomy (n = 1). Relapse occurred in 27% of the patients. No death was recorded.
Conclusion
VT is widely considered to be the hallmark of BS as well as a major risk factor for future severe vascular and cardiac involvements. Due to its inflammatory pathogenesis, it has specific clinical and imaging features. Better knowledge of these characteristics could help early establish the diagnosis of BS, thus improving patient outcomes.
Disclosure
S. Chadli: None. H. Khibri: None. R. Benachour: None. S. Firdaous Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.
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