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2.
Rheumatology (Oxford) ; 59(Supplement_5): v29-v38, 2020 Dec 05.
Artigo em Inglês | MEDLINE | ID: mdl-33280012

RESUMO

SLE is a chronic autoimmune rheumatic disorder of high heterogeneity in clinical presentation, treatment response and prognosis. Long-term outcomes in SLE have been dramatically improved over the past decades, however, increased morbidity and mortality, especially among young individuals, still exists. Unmet needs include residual disease activity and frequent flares, glucocorticoid treatment dependency and toxicity, comorbidity burden, reduced health-related quality of life, health disparities and damage. The main determinants of long-term outcomes in SLE are age, sex, race/ethnicity, genetic profile, environmental factors including smoking, disease activity, major organ involvement such as lupus nephritis and CNS involvement, comorbidities including cardiovascular disease and serious infections, coexistence with APS, treatment adherence, socio-economic factors and access to care. In this review we discuss trends in long-term outcomes in SLE over the years and major contributors such as genetic, disease-related, treatment, comorbidity, socio-economic and other factors.

3.
Artigo em Inglês | MEDLINE | ID: mdl-33295627

RESUMO

OBJECTIVES: Predicting serious infections (SI) in patients with rheumatoid arthritis (RA) is crucial for the implementation of appropriate preventive measures. Here we aimed to identify risk factors for SI and to validate the RA Observation of Biologic Therapy (RABBIT) risk score in real-life settings. METHODS: A multi-centre, prospective, RA cohort study in Greece. Demographics, disease characteristics, treatments and comorbidities were documented at first evaluation and one year later. The incidence of SI was recorded and compared with the expected SI rate using the RABBIT risk score. RESULTS: A total of 1557 RA patients were included. During follow-up, 38 SI were recorded [incidence rate ratio (IRR): 2.3/100 patient-years]. Patients who developed SI had longer disease duration, higher HAQ at first evaluation and were more likely to have a history of previous SI, chronic lung disease, cardiovascular disease and chronic kidney disease. By multivariate analysis, longer disease duration (IRR: 1.05; 95% CI: 1.005, 1.1), history of previous SI (IRR: 4.15; 95% CI: 1.7, 10.1), diabetes (IRR: 2.55; 95% CI: 1.06, 6.14), chronic lung disease (IRR: 3.14; 95% CI: 1.35, 7.27) and daily prednisolone dose ≥10 mg (IRR: 4.77; 95% CI: 1.47, 15.5) were independent risk factors for SI. Using the RABBIT risk score in 1359 patients, the expected SI incidence rate was 1.71/100 patient-years, not different from the observed (1.91/100 patient-years; P = 0.97). CONCLUSION: In this large real-life, prospective study of RA patients, the incidence of SI was 2.3/100 patient-years. Longer disease duration, history of previous SI, comorbidities and high glucocorticoid dose were independently associated with SI. The RABBIT score accurately predicted SI in our cohort.

4.
J Rheumatol ; 2020 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-33259328

RESUMO

OBJECTIVE: APS ACTION Registry studies long-term outcomes in persistently antiphospholipid antibody (aPL)-positive patients. Our primary objective was to determine whether clinically meaningful aPL profiles at baseline remain stable over time. Our secondary objectives were to determine a) whether baseline characteristics differ between patients with stable and unstable aPL profiles, and b) predictors of unstable aPL profiles over time. METHODS: Clinically meaningful aPL profile was defined as positive lupus anticoagulant (LA) test and/or anticardiolipin (aCL)/anti-ß2 glycoprotein-I (aß2GPI) IgG/M ≥40 U. Stable aPL profile was defined as a clinically meaningful aPL profile in at least two-thirds of follow-up measurements. Generalized linear mixed models with logit link were used for primary objective analysis. RESULTS: Of 472 patients with clinically meaningful aPL profile at baseline (median follow up: 5.1 years), 366/472 (78%) patients had stable aPL profiles over time, 54 (11%) unstable; and 52 (11%) inconclusive. Time did not significantly affect odds of maintaining a clinically meaningful aPL profile at follow-up in univariate (p=0.906) and multivariable analysis (p=0.790). Baseline triple aPL positivity decreased (Odds Ratio [OR] 0.25, 95% Confidence Interval [CI] 0.10-0.64, p=0.004) and isolated LA test positivity increased (OR 3.3, 95% CI 1.53-7.13, p=0.002) the odds of an unstable aPL profile over time. CONCLUSION: Approximately 80% of our international cohort patients with clinically meaningful aPL profile at baseline maintain such at a median follow-up of five years; triple aPL-positivity increase the odds of a stable aPL profile. These results will guide future validation studies of stored blood samples through APS ACTION Core Laboratories.

5.
Mediterr J Rheumatol ; 31(Suppl 2): 288-294, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33196007

RESUMO

Background: The COVID-19 pandemic is associated with emotional distress and significant disruptions in health-care services. These are key players in the development of nocebo phenomena. We aimed to investigate nocebo-prone behaviour in patients with autoimmune rheumatic diseases (ARD) amid the COVID-19 pandemic-associated lockdown. Methods: Consecutive patients were telephone-interviewed during the COVID-19 pandemic in Greece. Clinical and socioeconomic characteristics (eg, level of education) were recorded. For nocebo behaviour, a four-item validated questionnaire (Q-No, cut-off score>15), was used. Results were compared with pre-COVID-19 Q-No scores collected from patients followed-up in our department. Results: Nocebo behaviour was detected in 51/500 (10.2%) individuals. In patients with nocebo behaviour, use of anti-hypertensives was less common (17.6% vs 31.8%, p=0.04), but a higher level of education was more common (58.8% vs 35.9%, p=0.002), compared with patients with Q-No score ≤15; the latter retained statistical significance in multivariate regression analysis (p=0.009, OR [95%CI]: 2.29, [1.23-4.25]). Total Q-No scores were higher in the COVID-19-period compared to the pre-COVID-19 era [median (range); 12 (4-20) vs 11 (4-20), p=0.02]. Among 78 patients with available Q-No questionnaires in the pre-COVID-19 era, 11 (14.1%) displayed nocebo behaviour, which increased to 16 (20.5%) amid the COVID-19 pandemic. Interim development of nocebo behaviour was also associated with higher educational level (p=0.049, OR: 3.65, 95%CI: 1.005-13.268). Conclusion: A considerable proportion of ARD patients manifested nocebo-prone behaviour during the COVID-19 pandemic, which was more common among those with high educational level.

6.
Clin Rheumatol ; 2020 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-33196982

RESUMO

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by arterial, venous, and/or small vessel thrombosis, pregnancy morbidity, and persistently elevated levels of antiphospholipid antibodies (aPL). Cardiovascular disease (CVD) in APS can present as heart valvular disease (HVD), macro-micro-coronary artery disease (CAD), myocardial dysfunction, cardiac thrombi, or pulmonary hypertension. Brain disease presents as stroke or transient ischemic attack (TIA) and less frequently as cerebral venous thrombosis, seizures, cognitive dysfunction, multiple sclerosis (MS)-like syndrome, or chorea. Infarcts and focal white matter hyperenhancement are the commonest brain (MRI) abnormalities, while myocardial ischemia/fibrosis, valvular stenosis/regurgitation, or cardiac thrombi are the main abnormalities detected by cardiovascular magnetic resonance. This review aims to present the existing evidence on brain/heart involvement and their interrelationship in APS and the role of brain/heart MRI in their evaluation. Embolic brain disease, due to HVD, CAD, and/or cardiac thrombus, or brain hypo-perfusion, due to myocardial dysfunction, are among the main brain/heart interactions in APS and they are considered determinants of morbidity and mortality. Currently, there is no evidence to support the use of combined brain/heart MRI in asymptomatic APS patients. Until more data will be available, this approach may be considered in APS patients at high risk for CVD/stroke, such as systemic lupus erythematosus with high-risk aPL profile or high scores in CVD risk prediction models; APS patients with HVD/thrombus, CAD, or heart failure; those with classic and non-criteria neurologic APS manifestations (seizures, cognitive dysfunction, MS-like syndrome); or with aggressive multi-organ disease. Key Points • Cardiovascular disease (CVD) in antiphospholipid syndrome (APS) can present as heart valvular disease (HVD), macro-micro-coronary artery disease (CAD), myocardial dysfunction, cardiac thrombi, or pulmonary hypertension. • Brain disease presents as stroke or transient ischemic attack (TIA), and less frequently as cerebral venous thrombosis, seizures, cognitive dysfunction, and multiple sclerosis (MS). • A combined brain/heart MRI may be considered in APS patients at high risk for CVD/stroke, such as systemic lupus erythematosus with high-risk aPL profile or high scores in CVD risks; APS patients with HVD/thrombus, CAD, or heart failure; those with classic and non-criteria neurologic APS manifestations (seizures, cognitive dysfunction, MS-like syndrome); or with aggressive multi-organ disease.

7.
Clin Exp Rheumatol ; 2020 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-33200737

RESUMO

OBJECTIVES: Autologous haematopoietic stem cell transplantation (HSCT) has exhibited superior efficacy compared to conventional immunosuppressives in rapidly progressive diffuse systemic sclerosis (SSc) patients, albeit still of limited availability. We examined disease outcomes of conventionally-treated real-world inception patients eligible for HSCT, according to HSCT criteria used in the ASTIS and SCOT randomised trials, and compared them to the outcomes of participants in these trials. METHODS: Overall and event-free survival rates in our inception cohort were analysed at 4.5 and 7 years after HSCT criteria fulfilment and compared to those reported in HSCT and control arms of ASTIS and SCOT. RESULTS: Forty-five of our 142 inception cohort patients fulfilled HSCT criteria within 4 years from disease onset and had comparable baseline characteristics to SCOT/ASTIS patients. Four patients underwent HSCT. The remaining 41 were treated with conventional DMARDs: cyclophosphamide (n=24), mycophenolate mofetil (n=17), rituximab (n=2), tocilizumab (n=3), methotrexate (n=6) or combinations and their 10-year survival was 56% vs. 76% in those with diffuse SSc not fulfilling HSCT criteria. Their survival rates at the time endpoints of SCOT and ASTIS (4.5 and 7 years, respectively) were comparable to the conventionally-treated SCOT/ASTIS control groups. Extrapolating from SCOT/ASTIS results, if all our patients had undergone HSCT promptly, their overall and event-free survival rates could have increased from 73/51% to 83/72% at 4.5 years, and from 63/39% to 76/72% at 7 years, respectively. CONCLUSIONS: Wider availability and physician's early acknowledgement and referral of eligible patients for HSCT could significantly improve disease outcomes of rapidly progressive diffuse SSc patients.

9.
Lupus ; 29(12): 1571-1593, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33100166

RESUMO

Antiphospholipid syndrome (APS), an acquired autoimmune thrombophilia, is characterised by thrombosis and/or pregnancy morbidity in association with persistent antiphospholipid antibodies. The 16th International Congress on Antiphospholipid Antibodies Task Force on APS Treatment Trends reviewed the current status with regard to existing and novel treatment trends for APS, which is the focus of this Task Force report. The report addresses current treatments and developments since the last report, on the use of direct oral anticoagulants in patients with APS, antiplatelet agents, adjunctive therapies (hydroxychloroquine, statins and vitamin D), targeted treatment including rituximab, belimumab, and anti-TNF agents, complement inhibition and drugs based on peptides of beta-2-glycoprotein I. In addition, the report summarises potential new players, including coenzyme Q10, adenosine receptor agonists and adenosine potentiation. In each case, the report provides recommendations for clinicians, based on the current state of the art, and suggests a clinical research agenda. The initiation and development of appropriate clinical studies requires a focus on devising suitable outcome measures, including a disease activity index, an optimal damage index, and a specific quality of life index.

10.
Sci Rep ; 10(1): 16648, 2020 10 06.
Artigo em Inglês | MEDLINE | ID: mdl-33024230

RESUMO

Systemic Lupus Erythematosus (SLE) is the prototype of autoimmune diseases, characterized by extensive gene expression perturbations in peripheral blood immune cells. Circumstantial evidence suggests that these perturbations may be due to altered epigenetic profiles and chromatin accessibility but the relationship between transcriptional deregulation and genome organization remains largely unstudied. In this work we propose a genomic approach that leverages patterns of gene coexpression from genome-wide transcriptome profiles in order to identify statistically robust Domains of Co-ordinated gene Expression (DCEs). Application of this method on a large transcriptome profiling dataset of 148 SLE patients and 52 healthy individuals enabled the identification of significant disease-associated alterations in gene co-regulation patterns, which also correlate with SLE activity status. Low disease activity patient genomes are characterized by extensive fragmentation leading to overall fewer DCEs of smaller size. High disease activity genomes display extensive redistribution of co-expression domains with expanded and newly-appearing (emerged) DCEs. The dynamics of domain fragmentation and redistribution are associated with SLE clinical endophenotypes, with genes of the interferon pathway being highly enriched in DCEs that become disrupted and with functions associated to more generalized symptoms, being located in domains that emerge anew in high disease activity genomes. Our results suggest strong links between the SLE phenotype and the underlying genome structure and underline an important role for genome organization in shaping gene expression in SLE.

11.
Artigo em Inglês | MEDLINE | ID: mdl-33027516

RESUMO

OBJECTIVE: Amyloid-beta1-40 (Aß40) is a pro-inflammatory peptide under investigation as a novel biomarker of vascular inflammation, endothelial dysfunction and atherothrombosis in the general population. Herein we tested the hypothesis that Aß40 is deregulated in APS, a systemic autoimmune disease characterized by a thrombo-inflammatory state. METHODS: Between January 2016 and July 2017, we consecutively recruited 80 regularly followed thrombotic APS patients (44 primary, 36 SLE/APS) and 80 age- and sex-matched controls. Plasma Aß40 levels were measured using ELISA and APS-related clinical and laboratory characteristics were recorded. The adjusted Global Anti-Phospholipid Syndrome Score (aGAPSS), a validated risk score in APS, was calculated as a comparator to Aß40 performance to detect arterial thrombotic APS-related events. RESULTS: Higher Aß40 levels were significantly associated with the presence of APS [odds ratio (OR) 1.024 per 1 pg/ml (95% CI 1.007, 1.041)] after adjustment for cardiovascular risk factors (CVRFs), including smoking, arterial hypertension, dyslipidaemia and BMI, and for estimated glomerular filtration rate (eGFR). Among APS patients, increased high-sensitivity CRP (hs-CRP) serum levels was the only independent determinant of Aß40 levels. Importantly, Aß40 levels above the optimal receiver operating characteristics (ROC)-derived cut-off value were independently associated with recurrent arterial events [OR 4.93 (95% CI 1.31, 18.51)] after adjustment for age, sex, CVRFs, hs-CRP and high anti-ß2 glycoprotein I IgG titres. Finally, by ROC curve analysis, Aß40 provided incremental additive value over the aGAPSS by significantly improving its discrimination ability for recurrent arterial thromboses. CONCLUSION: In APS, Aß40 plasma levels are elevated and associated with an adverse thrombo-inflammatory profile. The pathophysiological and prognostic role of Aß40 in APS merits further investigation.

13.
Ther Adv Musculoskelet Dis ; 12: 1759720X20949088, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33062067

RESUMO

Objective: Our aim was to evaluate the effect of adalimumab on work productivity measures, overall activity impairment, and sleep quality in patients with active moderate to severe rheumatoid arthritis (RA), psoriatic arthritis (PsA), or ankylosing spondylitis (AS) treated in routine care settings in Greece and determine factors associated with work impairment and sleep disturbance. Methods: Patients with active moderate to severe RA (n = 184), PsA (n = 166), and AS (n = 150) were enrolled in this 24-month, prospective, observational study at 80 hospital outpatient clinics and private practices throughout Greece. Patients received adalimumab alone or in combination with standard antirheumatic therapies according to routine care. Work productivity and sleep were assessed through two patient-reported outcome measures: the Work Productivity and Activity Impairment-General Health questionnaire and the Medical Outcomes Study Sleep Scale (MOS-SS). Pearson correlation coefficients were estimated to assess the association of work impairment and sleep disturbances with disease activity scores. Results: In the overall population, adalimumab significantly lowered absenteeism [mean (95% confidence interval) reduction, 18.9% (13.3-24.5%); n = 100]; presenteeism [40.0% (33.8-46.3%); n = 98], overall work productivity impairment [46.8% (40.4-53.2%); n = 94], activity impairment [47.0% (44.3-49.6); n = 421], and the MOS-SS sleep problems index [31.6 (29.5-34.1); n = 421] after 24-month treatment (p < 0.001). Significant improvements were also noted across the RA, PsA, and AS subpopulations (p < 0.05). Improvements in overall work impairment and sleep disturbance positively correlated with improvements in disease activity measures. Conclusion: Adalimumab improves work productivity and sleep problems while lowering disease activity in patients with moderate to severe RA, PsA, and AS managed in real-world settings.

14.
Artigo em Inglês | MEDLINE | ID: mdl-32986935

RESUMO

OBJECTIVE: To describe baseline characteristics of antiphospholipid antibody (aPL)-positive patients, overall and by clinical and laboratory subtypes, enrolled in an international registry. METHODS: AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking Registry includes persistently aPL-positive adults. We evaluated baseline sociodemographic and aPL-related (APS classification criteria and "non-criteria") characteristics of patients overall and in subgroups (aPL-positive without APS, APS overall, thrombotic APS [TAPS] only, obstetric APS [OAPS] only, and both TAPS/OAPS). We assessed baseline characteristics of patients tested for three aPL (lupus anticoagulant test [LA], anticardiolipin antibody [aCL], and anti-ß2 -Glycoprotein-I [aß2 GPI]) by aPL profiles (LA only, single, double, and triple aPL positivity). RESULTS: Of 804 aPL-positive patients (mean age: 45 ± 13y; female: 74%; white 68%; other systemic autoimmune diseases: 36%), 80% were classified as APS (55% TAPS, 9% OAPS, and 15% TAPS/OAPS). In the overall cohort, 71% had vascular thrombosis, 50% with pregnancy history had obstetric morbidity, and 56% had at least one non-criteria manifestation. Among those with three aPL tested (n: 660), 42% were triple aPL positive. While single, double and triple aPL positive subgroups had similar frequencies of vascular, obstetric, and non-criteria events, these events were lowest in the single aPL subgroup consisting of aCL or aß2 GPI only. CONCLUSION: Our study demonstrates the heterogeneity of aPL-related clinical manifestations and laboratory profiles in a multicenter, international cohort. Within single aPL-positivity, LA may be a major contributor to clinical events. Future prospective analyses, using standardized core laboratory aPL tests, will help clarify aPL risk profiles and improve risk stratification.

15.
RMD Open ; 6(3)2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32978302

RESUMO

OBJECTIVES: Depression and anxiety are linked bi-directionally with inflammatory rheumatic diseases (IRDs) activity, which in turn, depends on subjective patient reported outcomes that can be distorted by comorbid mood disorders. We tested the hypothesis that introduction and/or switching of biologic agents for IRDs are associated with treatment for depression and/or anxiety, by analysing real-world data. METHODS: Using a country-wide electronic prescription database (10 012 604 registered, 99% population coverage), we captured almost all patients with rheumatoid arthritis (n=12 002), psoriatic arthritis (n=5465) and ankylosing spondylitis (n=6423) who received biologic disease modifying anti-rheumatic drugs (bDMARDs) during a 2-year period (8/2016-7/2018). Concomitant antidepressant/anxiolytic medication use was documented in patients who started or switched bDMARDs and compared with those who remained on conventional synthetic (cs)DMARDs or the same bDMARD, respectively, by multivariate regression analysis. RESULTS: Two-year data analysis on 42 815 patients revealed that bDMARD introduction was associated with both antidepressant [OR: 1.248, 95% CI 1.153 to 1.350, p<0.0001] and anxiolytic medication use [OR: 1.178, 95% CI 1.099 to 1.263, p<0.0001]. Moreover, bDMARD switching was also associated with antidepressant [OR: 1.502, 95% CI 1.370 to 1.646, p<0.0001] and anxiolytic medication use [OR: 1.161, 95% CI 1.067 to 1.264, p=0.001]. Notably, all these associations were independent of age, gender, underlying disease diagnosis and concomitant glucocorticoid or csDMARD medication use. CONCLUSION: In real-world settings, both introduction and switching of bDMARDs in patients with IRDs were associated with the presence of mood disorders. Although a causal relationship is uncertain, the impact of depression and anxiety should always be considered by physicians facing the decision to introduce or switch bDMARDs in patients with active IRDs.

18.
Curr Heart Fail Rep ; 17(5): 171-180, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32812180

RESUMO

PURPOSE OF REVIEW: Autoimmune rheumatic diseases (ARDs) affect 8% of the population and approximately 78% of patients are women. Myocardial disease in ARDs is the endpoint of various pathophysiologic mechanisms including atherosclerosis, valvular disease, systemic, myocardial, and/or vascular inflammation, as well as myocardial ischemia and replacement/diffuse fibrosis. RECENT FINDINGS: The increased risk of CVD in ARDs leads to excess comorbidity not fully explained by traditional cardiovascular risk factors. It seems that the chronic inflammatory status typically seen in ARDs, promotes both the development of myocardial inflammation/fibrosis and the acceleration of atherosclerosis. CMR (cardio-vascular magnetic resonance) is the ideal imaging modality for the evaluation of cardiac involvement in patients with ARDs, as it can simultaneously assess cardiac function and characterize myocardial tissues with regard to oedema and fibrosis. Due to its high spatial resolution, CMR is capable of identifying various disease entities such as myocardial oedema /inflammation, subendocardial vasculitis and myocardial fibrosis, that are often missed by other imaging modalities, notably at an early stage of development. Although generally accepted guidelines about the application of CMR in ARDs have not yet been formulated, according to our experience and the available published literature, we recommend CMR in ARD patientS with new-onset heart failure (HF), arrhythmia, for treatment evaluation/change or if there is any mismatch between patient symptoms and routine non-invasive evaluation.

19.
Curr Rheumatol Rep ; 22(9): 51, 2020 07 22.
Artigo em Inglês | MEDLINE | ID: mdl-32699944

RESUMO

PURPOSE OF THE REVIEW: To review the available evidence on the management of a variety of non-criteria manifestations in antiphospholipid syndrome (APS), including valvular disease, alveolar hemorrhage, thrombocytopenia, hemolytic anemia, APS nephropathy, skin ulcers, livedo reticularis, cognitive dysfunction, and epilepsy. RECENT FINDINGS: Current treatment relies on low-level evidence and mainly on expert consensus due to the rarity and the heterogeneity of non-criteria APS manifestations and the diversity in management approaches. Conventional anticoagulation and/or antiplatelet APS treatment do not adequately control most of non-criteria manifestations. Increasing knowledge about the contribution of inflammatory in addition to, or independently of, thrombotic mechanisms in non-criteria APS manifestations provides insight into the potential effect of novel therapies targeting B-cells, mammalian target of rapamycin, neutrophil, and complement or interferon pathways. Existing evidence is limited by lack of high-quality studies. Better understanding of the pathophysiology and clinical phenotypes of APS and well-designed prospective studies of homogenous populations are needed to provide evidence-based recommendations for the management of non-criteria APS manifestations.

20.
Clin Rheumatol ; 2020 Jul 24.
Artigo em Inglês | MEDLINE | ID: mdl-32710295

RESUMO

IgG4-related disease (IgG4-RD) can affect almost every tissue/organ. Vascular involvement in the setting of IgG4-RD is increasingly being recognized with most of the cases pertaining patients with aortitis and/or periaortitis with or without aneurysm formation. However, it is now evident that other vessels like iliac arteries, coronary arteries, carotids, and others can also be affected being often underdiagnosed. Vascular involvement is expressed as vessel wall thickening, while aneurysm formation is also occasionally seen. Although histopathological findings are the cornerstone in the diagnosis of IgG4-RD, imaging modalities like magnetic resonance imaging (MRI) and positron emission tomography (PET) are similarly important when vascular involvement occurs, helping in the mapping of the disease and in identifying other, more accessible to biopsy, affected organs. Inflammation markers like erythrocyte sedimentation rate and C-reactive protein have also been described in IgG4-RD patients with vascular involvement. Herein, we present a case of a middle-aged man with long-term high inflammation markers who eventually diagnosed with IgG4-RD after an ascending aorta aneurysm operation, while a subsequent comprehensive magnetic resonance angiography revealed also involvement of the abdominal aorta and the left subclavian and left common carotid. Moreover, we sought to review the current literature about medium- and large-vessel involvement, beyond the aorta, in patients with IgG4-RD.

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