Systemic Vasculitis Disease Research Articles

The risk of cancer in pediatric-onset immune-mediated inflammatory diseases – A nationwide study

Background and objectivesAdult-onset immune-mediated inflammatory disease (IMID) increases the risk of several cancers. However, data on pediatric-onset IMID (pIMID) remains scarce. We estimated the long-term cancer risk in pIMID and the association between medical treatment and specific cancers. MethodsWe used the nationwide Danish health registers to identify pIMID patients diagnosed from Jan 1, 1980 to Dec 31, 2018. Patients were matched with ten reference individuals based on age, sex, and residence. The primary exposure was pIMID, including autoimmune hepatitis, primary sclerosing cholangitis, Crohn's disease, ulcerative colitis, juvenile idiopathic arthritis, systemic lupus erythematosus, vasculitis, and connective tissue disease. Secondary exposures were immunomodulators and tumor necrosis factor-α antagonists (anti-TNFα). The primary outcome was cancer. Estimates are presented as hazard ratios adjusted for family income at diagnosis (AHR). ResultsWe included 12,664 pIMID patients and 109,274 reference individuals. Median follow-up time was 10.6 (interquartile range: 5.4–17.7) years for patients and 10.2 (interquartile range: 5.2–17.3) years for reference individuals. Patients with pIMID had a twofold higher cancer risk (AHR 2.2 [95 % confidence interval (CI): 1.8–2.6]) compared with reference individuals. Thiopurine treatment was associated with a higher risk of lymphoma (AHR 6.1 [95%CI: 2.2–16.8]) and skin cancer (AHR 6.1 [95%CI: 2.4–15.4]). Anti-TNFα treatment was associated with a higher risk of lymphoma (AHR 4.9 [95%CI: 1.1–22.6]). ConclusionsWe found an increased cancer risk in patients with pIMID followed into adulthood. Additionally, thiopurines and anti-TNFα were associated with increased lymphoma and skin cancer risks. This highlights the importance of individualized immunotherapy and cancer surveillance.

An Update on Noninfectious Retinal Vasculitis.

Retinal vasculitis (RV) signifies the inflammation of various retinal vessels. Noninfectious RV differs from infectious RV with regard to its pathogenesis and treatment. It can have varied clinical presentations and may be associated with systemic vasculitic diseases. Noninfectious RV can be caused due to type-III hypersensitivity reactions, increased expression of intracellular adhesion molecules, and genetic susceptibility. Noninfectious RV is primarily classified on the basis of the type of retinal vessels involved. It can be further classified as an occlusive or nonocclusive. RV can be a major association of systemic diseases like Behcet's disease, sarcoidosis and systemic lupus erythematosus. Newer modalities, like ultra-widefield fundus fluorescein angiography, can help in the management of RV. Effective treatment of noninfectious RV requires anti-inflammatory and immunosuppressive therapy. The patients may require treatment with high-dose corticosteroids and biological agents. Anti-vascular endothelial growth factor injections and laser photocoagulation may be indicated to treat the occlusive disease. Prompt treatment may prevent complications like vitreous hemorrhage, neovascular glaucoma, and tractional retinal detachment. The treatment more often requires a multidisciplinary approach. This review provides a comprehensive update on the various causes of noninfectious RV, including both systemic and isolated ocular conditions. It also details various complications and management strategies for this condition.

Serum sulfatide level is associated with severe systemic vasculitis with kidney involvement.

Sulfatides are a type of sulfated glycosphingolipid that are secreted with lipoproteins into the serum. These molecules are involved in the inflammatory pathway of vessels in addition to coagulation and platelet aggregation. Previous studies have proposed that sulfatides play a pivotal role in regulating inflammation-related disorders. Systemic vasculitis (SV) diseases are generally caused by autoimmune diseases and often involve kidney vasculitis, which may lead to rapidly progressive kidney dysfunction and end-stage kidney disease. Our earlier pilot study revealed that the level of serum sulfatides (SSs) was significantly decreased in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), a representative disease-causing SV with kidney involvement (SVKI), especially in patients exhibiting active crescentic findings on kidney biopsy. To further explore the clinical significance of an association between SS and SVKI, we analyzed and compared the SS level of patients with various SVKI diseases in this retrospective cohort study. Among patients admitted to our hospital between 2008 and 2021, we ultimately enrolled 26 patients with IgA vasculitis (IgAV), 62 patients with AAV, and 10 patients with anti-glomerular basement membrane disease (GBM) as examples of SVKI diseases, as well as 50 patients with IgA nephropathy (IgAN) and 23 donors for living kidney transplantation as controls. The mean ± standard deviation SS level in the donor, IgAN, IgAV, AAV, and GBM groups was 8.26 ± 1.72, 8.01 ± 2.21, 6.01 ± 1.73, 5.37 ± 1.97, and 2.73 ± 0.99 nmol/mL, respectively. Analysis of patients in the SVKI disease group showed that those with the crescentic class kidney biopsy finding exhibited a significantly lower SS level than did those with other class biopsy features. Additionally, the SS level had a higher detection ability for SVKI patients with crescentic class kidney biopsy findings (area under the receiver operating characteristic curve 0.90, 95% confidence interval 0.82-0.99) than did several other predictor candidates. Our results indicate that the SS level is decreased in more severe SVKI diseases and may be associated with active glomerular lesions in SVKI kidney biopsy samples.

Incidence and risk factors of nephritis in childhood Henoch-Schonlein purpura

Background Henoch-Schönlein Purpura (HSP) is the most common systemic vasculitis disease in children. It is characterized by involvement of the skin, joints, gastrointestinal tract, and kidney. Kidney manifestations may progress to severe nephritis, even lead to end-stage kidney disease.
 Objective To identify the incidence and risk factors of nephritis in childhood HSP.
 Methods A retrospective cohort study was performed to evaluate clinical, demographic, laboratory, and therapeutic parameters of HSP patients aged 0-18 years between 2011-2019 at Dr. Cipto Mangunkusumo Hospital, Jakarta. Diagnoses of HSP were made according to the 2008 EULAR/PRES/PRINTO criteria. Wefollowed subjects’ medical records for at least 3 months after disease onset to observe incidence and risk factors of Henoch-Schönlein nephritis (HSN).Results There were 112 HSP patients (aged 2-17 years) included in this study. HSN was found in 40 out of 112 patients (35.7%). Nephritis developed within the first 4 weeks for a majority of cases. Multivariate analysis showed that persistent purpura (OR 3.306; 95%CI 1.315 to 8.315; P=0.011) and acute phase leukocytosis(OR 2.585; 95%CI 1.047 to 6.385; P=0.039) were significantly associated risk factors for HSN. We found that corticosteroid use did not reduce the risk of HSN. The accumulation of several risk factors was associated with the likelihood of developing HSN.
 Conclusion Persistent purpura and acute phase leukocytosis are independent risk factors for HSN. Therefore, blood tests are needed to estimate the risk of HSN. Early corticosteroid therapy do not reduce the risk of kidney impairment.

ITPKC polymorphism (rs7251246 T > C), coronary artery aneurysms, and thrombosis in patients with Kawasaki disease in a Southern Han Chinese population.

Kawasaki disease (KD) is a commonly acquired pediatric systemic vasculitis disease resulting in coronary artery aneurysm (CAA). The relationship between the ITPKC polymorphism (rs7251246) and the severity and susceptibility to KD in the Han Chinese population in Southern China remains unclear. We enrolled 262 children as controls and 221 children with KD (46 [20.8%] with intravenous immunoglobulin resistance and 82 [37.1%] with CAA). The relationship between the ITPKC rs7251246 polymorphism, KD susceptibility, and CAA formation was investigated. While the ITPKC rs7251246 T>C polymorphism was not significantly associated with KD susceptibility, it was significantly related to the CAA risk in children with KD [CC/CT vs. TT: adjusted odds ratio [OR] 2.089, 95% confidence interval [CI] 1.085-4.020]. Male children with the rs7251246 CT/TT genotype had a significantly lower risk of thrombosis [CT/TT vs. CC: adjusted OR 0.251, 95% CI 0.068-0.923]. Children with KD, especially those with CAA, had significantly downregulated ITPKC mRNA compared to healthy children. ITPKC mRNA levels were lower in children with CAA who developed thrombosis (P=0.039). In children with KD, the CC genotype showed lower mRNA levels of ITPKC (P=0.035). The ITPKC rs7251246 T>C polymorphism may be a risk factor for CAA and thrombosis in children with KD in the Han Chinese population, likely due to differences in mature mRNA levels caused by interference of RNA splicing. Dual antiplatelet therapy for thrombosis is recommended for male children with the rs7251246 CC genotype.

CHARACTERISTICS OF THE COURSE OF LEUCOCYTOCLASTIC VASCULITIS WITH SKIN LESIONS. ANALYSIS OF CLINICAL CASES

The article describes the features of the course of leukocytoclastic skin vasculitis with consideration of 3 clinical cases. Leukocytoclastic vasculitis of the skin (LCV) is an isolated cutaneous vasculitis without damage to internal organs. At present, vasculitis is considered a polyetiological disease. The most frequent causes of the development of vasculitis limited to the skin include various acute or chronic infections: bacterial, viral, and fungal. It can be caused by medications, and sometimes the cause cannot be found. When LVC is suspected, an extensive examination is usually required to determine whether the process is limited to the skin or is a manifestation of systemic vasculitis or other systemic diseases. Diagnosis and treatment of LCV is a complex problem in the practice of doctors of many specialties, such as rheumatologists, dermatologists, and therapists, family doctors, which requires close cooperation of specialists. We present 3 clinical cases of patients who were hospitalized in the clinic with symptoms of skin lesions and suspicion of LCV after taking medications. The paper describes various skin manifestations and the results of the examination and treatment. The differential diagnostics enabled us to diagnose LCV in 2 cases, in one case – generalized urticaria due to taking furazolidone. It should be noted that drug reactions with skin lesions constitute a serious medical problem, whose relevance is increasing every year. This is largely due to widespread uncontrolled drug intake among the population, and frequent polypharmacy during treatment under the supervision of medical professionals. Multidisciplinary problems, which are solved by doctors of various specialties at different stages and levels of medical care, including drug-induced skin lesions in patients, are particularly difficult. Strengthening medical control over the prescription and administration of medicinal products, and improving the awareness of doctors of all specialties regarding the diagnosis and treatment of medicinal reactions is the way to solve the problem.

Clinicopathologic Spectrum of Renal Lesions Following Anti-TNF- α Inhibitor Therapy: A Single Center Experience.

Anti-tumor necrosis factor (TNF)-alpha inhibitors, as biological agents, are used in autoimmune inflammatory states, rheumatoid arthritis (RA), psoriatic arthritis (PA), and Crohn's disease. They can induce autoimmune serologic responses and clinical disorders including systemic vasculitis and lupus-like diseases, affecting the kidney. Retrospective analysis of clinicopathologic features of kidney disease following anti-TNF alpha therapy (treatment group) from our renal biopsy files from 2000-2018 is conducted and compared with 106 cases without therapy (control group). Forty-eight patients using anti-TNF-alpha agents had renal biopsies, RA in 30, PA in 6, Crohn's disease 6, RA and PA 1, RA and Crohn's disease 1, and others 4. 20 received etanercept, 15 adalimumab, 8 infliximab and 5 had 2 forms of agents manifesting new onset nephritic syndrome or CKD, 17 with acute kidney injury and 16 nephrotic syndrome, with recent ANCA and/or lupus serology. The renal lesions were crescentic glomerulonephritis in 8, 5 pauci-immune type and 5 ANCA+. Lupus or lupus-like nephritis seen in 6: ISN/RPS 2018 class II-2, class V-2, class III+V-1, class IV+V-1, 3 with concurrent fibrillary GN, scleroderma/TMA and amyloidosis. Renal lesions unrelated to anti-TNF-alpha therapy or underlying autoimmune disease in 23 (eg diabetic nephropathy, interstitial nephritis, acute tubular injury, infection-related GN), IgA nephropathy, renal sarcoidosis and AA amyloidosis were noted in 5 cases. Thrombotic microangiopathy was recognized in 5 cases, 1 associated with scleroderma and 1 antiphospholipid antibodies, and 2 nephrotic syndrome secondary to podocytopathy. The control group was similar with higher immune mediated GN, interstitial nephritis and amyloidosis. The renal lesions during anti-TNF-alpha therapy have an autoimmune basis such as ANCA, lupus or lupus-like disease, correlated with new onset serology, while others were similar to those observed in the control group. Renal biopsy findings integrated with clinical features and therapy can identify the underlying pathophysiologic process for appropriate management.

Alternating Facial Palsy- Decoding the Enigma.

Facial palsy is a neurological emergency with a wide spectrum of aetiologies. The term 'alternating facial palsy', a very rare presentation, refers to facial paralysis, the onset of which occurs at different points in time on both sides of the face. It can occur in systemic vasculitis, trauma, tumours and infectious diseases. We report the case of a middle-aged female who presented with complaints of alternating facial palsy and the diligent journey that we took to finally reach a diagnosis of Lyme neuroborreliosis.

Diagnostic significance of noncoding RNAs in kawasaki disease: A systematic review and meta-analysis.

Kawasaki disease (KD) is a systemic vasculitis disease, and early effective intervention would reduce the occurrence of coronary artery lesions (CALs). Recently, many scholars have been committed to studying the relationship between noncoding RNAs and KD. This systematic review aimed to analyze the diagnostic value of noncoding RNAs(ncRNAs) in distinguishing different KD status. We searched for the literature about diagnostic values of ncRNAs in KD in CNKI, VIP, Wanfang, China Biomedical Literature Database as well as PubMed, Web of Science, Embase, and Cochrane Library up to April 15, 2022. All included studies were further analyzed using STATA 12.0, Meta-disc 1.4 and RevMan 5.4 software. A total of six studies investigating the diagnostic performance of ncRNAs in differentiating KD-CAL (n = 101) from KD-NCAL patients (n = 123) were included in this this meta-analysis. The calculated area under the curve(AUC) was 0.83 (0.80-0.86). Four studies on the diagnostic performance of ncRNAs in differentiating acute KD patients (n = 139) from convalescent KD patients (n = 109) were included. The calculated AUC was 0.87 (0.84-0.90). Four studies focused on the diagnostic performance of ncRNAs combined with other laboratory indexes in KD by assessing 137 KD patients and 152 febrile controls. The calculated AUC was 0.90 (0.87-0.92). Four studies assessed the diagnostic performance of ncRNAs in differentiating intravenous immunoglobulin (IVIG)-resistant KD patients from IVIG-responsive KD patients. The calculated AUC was 0.9135 ± 0.0307. These results indicated that ncRNAs have a good diagnostic efficacy in KD. This meta-analysis showed that ncRNAs have potential as a biomarker for distinguishing different KD status. However, since limited studies were included in this meta-analysis, larger and well-designed diagnostic studies should be conducted to validate these results. INPLASY.COM, identifier: doi: 10.37766/inplasy2022.10.0035.

Obesity as a comorbidity in children and adolescents with autoimmune rheumatic diseases.

Childhood obesity is the public health issue with alarming rates recorded throughout developed world and an important modifiable health risk for developing various chronic diseases, with childhood-onset autoimmune rheumatic diseases among them also. The aim of this article was to summarize epidemiological, pathophysiological and clinical implication of obesity on juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (cSLE), juvenile dermatomyositis (JDM), IgA vasculitis (IgAV) and Kawasaki disease (KD). We reviewed PubMed database and selected 74 relevant articles. Epidemiological data of obesity among children with autoimmune rheumatic diseases indicate an increased prevalence of it. Pathophysiological link between obesity, humoral adipokines and cytokines released from fat tissue and childhood-onset autoimmune rheumatic diseases is complex and still not entirely clear. From the clinical point of view, obesity was not associated with disease activity in JIA and cSLE, but proved to contribute on functional impairment in both diseases and affect poor treatment response in JIA patients. Early atherosclerosis and cardiovascular disease (CVD) development in obese children and adolescents with JIA, cSLE and JDM are certainly important obesity-related complications. Understanding how obesity affects children and adolescents with autoimmune rheumatic diseases may encourage clinicians to consider taking better preventive strategies in this population to improve their long-term outcome.

Diffuse alveolar hemorrhage

Diffuse alveolar hemorrhage can accompany many diseases, including systemic vasculitis and other connective tissue diseases. Rapid diagnostics and immediate implementation of adequate treatment are extremely important. In this paper is presented a 63-year-old patient with fever, dyspnoea and massive haemoptysis, admitted as part of the emergency department.

Association of Genetic Polymorphisms in Kawasaki Disease with the Response to Intravenous Immunoglobulin Therapy.

Kawasaki disease (KD) is an acute febrile and systemic vasculitis disease mainly affecting children < 5years old. Although the first case of KD was reported in 1967 and despite extensive research on KD since then, the cause of the disease remains largely unknown. The most common complications of KD are coronary artery lesions (CAL), which significantly increase the risk of coronary heart disease. The standard treatment for KD is high-dose intravenous immunoglobulin (IVIG) plus aspirin within 10days from symptoms' appearance, which has been shown to decrease the incidence of CAL to 5-7%. Despite the benefits of IVIG, about 25% of the patients treated with IVIG develop resistance or are unresponsive to the therapy, which represents an important risk factor for CAL development. The cause of IVIG unresponsiveness has not been fully elucidated. However, the role of gene polymorphisms in IVIG response has been suggested. Herein, we comprehensively review genetic polymorphisms in KD that have been associated with IVIG resistance/unresponsiveness and further discuss available models to predict IVIG unresponsiveness.Kindly check and confirm inserted city in affiliation [1] is correctly identified.confirm.

Peripheral Ulcerative Keratitis and its Management

Peripheral ulcerative keratitis (PUK) is a destructive inflammation of the peripheral cornea associated with corneal epithelial sloughing and keratolysis. It could be associated with various ocular and systemic infections and noninfectious diseases. Rheumatoid arthritis is the most frequent underlying disease. Various systemic autoimmune vasculitic diseases that can prove fatal may present as PUK. If not treated properly, PUK can progress to perforation resulting in significant ocular morbidity and when associated with a systemic autoimmune condition, may be life-threatening. PUK-associated complications may be prevented with timely diagnosis, detection of the underlying systemic inflammatory disorders, and proper treatment.

Long-Term Outcomes of Kidney Transplant Recipients with Glomerulonephritides by Induction Type and Steroid Avoidance

Kidney transplant programs have different approaches to induction immunosuppression, and conflicting data exist on the role of steroid maintenance in recipients with glomerulonephritis (GN). GN patients are unique because of a higher risk for immune system exhaustion due to prior exposure to immunosuppressants to treat their GN; this raises questions regarding the optimal immunosuppression needed for transplant success and reduction of complications. We sought to assess the effect of induction type and steroid maintenance on the recipient and kidney graft survival in those with IgA nephropathy (IgAN), systemic lupus erythematosus related GN (SLE), small-vessel vasculitis (SVV), and anti-glomerular basement membrane disease (anti-GBM). We analyzed the Scientific Registry of Transplant Recipients (SRTR) database for adult, primary kidney recipients with the above glomerulonephritides through September 2019. Kaplan–Meier curves were generated to examine kidney graft and recipient survival. We used multivariable Cox proportional hazard models to investigate the impact of induction type and steroid maintenance in each group separately. Our study included 9176 IgAN, 5355 SLE, 1189 SVV, and 660 anti-GBM recipients. Neither induction type nor steroid maintenance therapy influenced recipient or death-censored graft survival in this cohort of recipients. Our findings provide an opportunity for recipients with a history of one of the studied glomerulonephritides to receive a more tailored immunosuppression regimen, considering their previous exposure to immunosuppressants.

The Role of Cardiac Imaging in the Evaluation of Cardiac Involvement in Systemic Diseases.

For systemic diseases like rheumatoid arthritis, systemic lupus erythematosus (SLE), systemic sclerosis, systemic vasculitis, myopathies, and mixed connective tissue diseases, cardiac disease is a major contributing factor for morbidity and mortality. The cardiovascular manifestations are the result of various pathophysiological components, which complicate management. Furthermore, the signs and symptoms can be subtle and missed due to the complex nature of the underlying condition. As a result, various imaging approaches play an imperative role in diagnosis and prognosis. The evolving role of these modalities could lead to risk stratification and improved therapies in the future. In conclusion, our review article will highlight the role of cardiac imaging in the evaluation of cardiac involvement for systemic diseases.

Old and New Challenges in Uveitis Associated with Behçet’s Disease

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.

Isolated CNS involvement in eosinophilic granulomatosis with polyangiitis treated with mepolizumab: A case report

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis disease involving small-sized vessels. The literature has reported involvement of the central nervous system (CNS) in 5% cases, and isolated CNS involvement is extremely rare. Due to its rarity and scarcity of clinical data, standardized treatment of isolated CNS involvement in EGPA is unclear. Mepolizumab, an anti-interleukin-5 monoclonal antibody, was previously introduced to treat EGPA with longer remission period, more patients showing remission, and reduction in prednisolone dose of those who already taking prednisolone compared to placebo. We describe a case of isolated CNS involvement in EGPA, confirmed by brain biopsy and treated with mepolizumab, which was refractory to conventional immunotherapy.

Non-Infectious, Necrotizing and Granulomatous Aortitis in a Female Gorilla

A 41-year-old female captive gorilla with progressive weight loss and hydrothorax of unknown origin was euthanized and submitted for necropsy. The ascending aorta showed intimal aortic thickenings, consistent with so called 'tree bark' changes. Microscopic examination revealed a non-infectious, necrotizing and granulomatous aortitis with no evidence of systemic vasculitis or infectious disease elsewhere in the body. While rare, large vessel vasculitides should be considered as a differential diagnosis in gorillas presenting with progressive non-specific signs and vascular intimal changes.

Risk factors for coronary artery lesions in children with Kawasaki disease.

Kawasaki disease (KD) is a nonspecific systemic vasculitic disease that frequently occurs among children, and coronary artery lesion (CAL) is the most serious complication. We aimed to study the risk factors for CAL in children with KD. KD children in accordance with diagnostic criteria, who were hospitalized from January 2014 to December 2017, were selected as subjects. Univariate and multivariate logistic regression analyses were conducted to explore the relationships between CAL and gender, age, clinical diagnosis, erythrocyte sedimentation rate (ESR), platelet count, hemoglobin level, C reactive protein level, white blood cell count, initiation time of IVIG administration and duration of fever. The enrolled 982 KD children were divided into a CAL group (n = 104) and an NCAL group (n = 878) according to cardiac color Doppler ultrasonography. The incidence rate of CAL was 10.6 % (104/982). Univariate analysis showed that the two groups had significantly different gender, ESR, platelet count, initiation time of IVIG administration and duration of fever (P &#60; 0.05). Multivariate logistic regression analysis revealed that male gender, elevated ESR and delayed use of IVIG were independent risk factors for KD complicated with CAL. Male gender, increased ESR and delayed use of IVIG were independent risk factors for KD complicated with CAL.

Management of a giant aortic root aneurysm in a young patient with Marfan syndrome: a case report

IntroductionMarfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age.CaseThe patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed 190.5 cm of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. Transthoracic echocardiography and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we performed Bentall procedure based on the results of multidisciplinary team discussion and intraoperative exploration. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later.ConclusionIt is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. A mechanical Bentall procedure seems to be an acceptable option for some selected cases.