Takayasu arteritis associated with autoimmune/inflammatory syndrome induced by adjuvants: a case-based review.

Takayasu's arteritis (TA) is a chronic granulomatous vasculitis that predominantly affects the aorta and its major branches. Despite advancements in the understanding of the pathogenic pathways of vascular inflammation, the etiology and predisposing factors of TA remain to be fully understood. In susceptible individuals, exposure to adjuvants may trigger, unlock or unmask an autoimmune disorder, presenting as non-specific constitutional symptoms or a fully developed autoimmune syndrome such as vasculitis. Here, we hypothesize that TA could be triggered by siliconosis, a subtype of the autoimmune/inflammatory syndrome induced by adjuvants (ASIA). ASIA, also known as Shoenfeld syndrome, encompasses a wide range of autoimmune and immune-mediated diseases resulting from dysregulation of the immune response after exposure to agents with adjuvant activity. This case report describes the development of large artery vasculitis, TA, in an individual one year following the placement of silicone breast implants. The patient initially presented with non-specific symptoms, and multiple imaging methods were employed, including ultrasound diagnostics, CT angiography, and 18-fluorodeoxyglucose positron emission tomography/CT. These techniques revealed vasculitic alterations in the carotid arteries and thoracic aorta. Initial treatment with glucocorticosteroids proved ineffective, prompting the addition of steroid-sparing immunosuppressive agents. Due to the distinct clinical symptoms, disease progression, implant-associated fibrosis, and resistance to therapy, the potential involvement of implants in the development of large-vessel vasculitis was considered, and a potential association with ASIA was postulated. Although there is limited evidence to support a direct link between adjuvants and the pathogenesis of TA, similarities in cellular immunity between the two conditions exist. The diagnosis of this complex and potentially debilitating condition requires a comprehensive clinical examination, laboratory evaluation, and instrumental assessment. This will aid in identifying potential contributing factors and ensuring successful treatment.

COVID-19 vaccine uptake, hesitancy and clinical effects on patients with Takayasu's arteritis: A web-based questionnaire survey from a large cohort.

Objective: This study aimed to investigate the Coronavirus disease 2019 (COVID-19) vaccination rate, reasons for vaccine hesitancy and clinical effects on patients with Takayasu's arteritis (TAK). Methods: A web-based survey was administered to a TAK cohort established by the Department of Rheumatology, Zhongshan Hospital through WeChat in April, 2022. Responses from a total of 302 patients were received. The Sinovac or Sinopharm inactivated vaccination rate, side effects, and vaccine hesitancy reasons were analyzed. In addition, disease flare, new disease onset, and changes of immune-related parameters after vaccination were analyzed in vaccinated patients. Results: Among 302 patients, 93 (30.79%) received the inactivated COVID-19 vaccination. Among the 209 unvaccinated patients, the most common reason for hesitancy were concern about side effects (136, 65.07%). Vaccinated patients had a longer disease duration (p = 0.08) and lower use of biologic agents (p < 0.001); 16 (17.20%) of the 93 vaccinated patients developed side effects, and most of them were mild; 8 (8.60%) developed disease flares or new-onset disease 12-128 days post-vaccination and 2 (2.15%) developed serious adverse effects (vision defect and cranial infarction). Immune-related parameters of 17 patients indicated decreases in IgA and IgM after vaccination (p < 0.05). Eighteen (19.35%) of the 93 vaccinated patients were diagnosed post-vaccination.These patients had a significantly higher percentage of CD19+ B cells at disease onset (p < 0.05) than the unvaccinated patients diagnosed at the same time. Conclusion: The vaccination rate was low in TAK, which was mainly caused by concerns about negative effects of vaccination on their disease. An acceptable safety profile was observed in vaccinated patients. The risk of disease flare associated with COVID-19 vaccination warrants further investigation.

Heart failure caused by Takayasu's arteritis in the time of COVID-19: a case report.

The case of a 35-year-old female with heart failure is presented, where the symptoms overlap with the heterogeneous manifestations of coronavirus disease 2019 (COVID-19). Those similarities and a recent shift in priorities during the SARS-CoV-2 pandemic delayed the recognition of acute heart failure in this patient. During the differential diagnostic process, obliterative disease was discovered in the bilateral subclavian and right renal arteries, and the latter resulted in uncontrolled hypertension, which played a significant role in the development of heart failure. The aetiology of vascular alterations turned out to be Takayasu's arteritis. Diagnosing Takayasu's arteritis is typically not straightforward due to its nonspecific signs and symptoms. Therefore, it can be concluded from our case report that the rising incidence of COVID-19 and focus on ruling out infection can potentially defer alternative, but appropriate diagnostic tests, particularly for certain conditions like rare diseases. Early identification and intervention is especially important for treating acute heart failure, whereas delay increases the risk of severe complications and mortality.

The effect of COVID-19 pandemic in a large series of patients with Takayasu arteritis.

BACKGROUND: Patients with inflammatory rheumatic diseases faced several challenges during the COVID-19 pandemic. Uncertainties such as the lack of evidence regarding the use of immunosuppressive (IS) therapies and deferred patient care because of limited health resources affected negatively on many aspects of treatment decisions and routine follow-up of the patients. In this study, we aimed to investigate the prevalence and severity of SARS-CoV-2 infection, the impact of the pandemic on delays in routine clinical follow-up, changes in IS treatment, and COVID-19 vaccination status of patients with Takayasu arteritis (TAK). METHODS: The study was performed between July and September 2021. TAK patients who registered in our database were investigated with regards to the COVID-19 infection and vaccination status, delays in routine clinical visits, changes in their IS treatments, and flares during the pandemic. Physical examination, laboratory tests, and imaging of the patients were performed and ITAS2010 scores were calculated. RESULTS: There were 56 adult TAK patients (87.5% female and median age 47 years). A total of 44 (78.6%) patients experienced a delay with routine follow-up visits to their physicians and about 20% of patients stopped their antirheumatic treatments without consulting their physicians. Compared to the pre-COVID-19 pandemic, 16 (28.5%) patients flared. In total group, 13 (23.2%) patients had a mild COVID-19 infection and about 90% of the patients had received the COVID-19 vaccine. DISCUSSION: Deferred patient care and disease flares are the most significant problems in TAK patients during the pandemic. The risk of TAK flares may outweigh the risk of COVID-19 infection.

Treatment efficacy and safety of tofacitinib versus methotrexate in Takayasu arteritis: a prospective observational study.

OBJECTIVE: To compare the treatment efficacy and safety of tofacitinib (TOF) versus methotrexate (MTX) in Takayasu arteritis (TAK). METHODS: Fifty-three patients with active disease from an ongoing prospective TAK cohort in China were included in this study. Twenty-seven patients were treated with glucocorticoids (GCs) and TOF, and 26 patients were treated with GCs with MTX. The observation period was 12 months. Complete remission (CR), inflammatory parameter changes, GCs tapering and safety were assessed at the 6th, 9th and 12th month. Vascular lesions were evaluated at the 6th and 12th month, and relapse was analysed during 12 months. RESULTS: The CR rate was higher in the TOF group than in the MTX group (6 months: 85.19% vs 61.54%, p=0.07; 12 months: 88.46% vs 56.52%, p=0.02). During 12 months' treatment, patients in the TOF group achieved a relatively lower relapse rate (11.54% vs 34.78%, p=0.052) and a longer median relapse-free duration (11.65±0.98 vs 10.48±2.31 months, p=0.03). Average GCs dose at the 3rd, 6th and 12th month was lower in the TOF group than that in the MTX group (p<0.05). A difference was not observed in disease improvement or disease progression on imaging between the two groups (p>0.05). Prevalence of side effects was low in both groups (3.70% vs 15.38%, p=0.19). CONCLUSION: TOF was superior to MTX for CR induction, a tendency to prevent relapse and tapering of the GCs dose in TAK treatment. A good safety profile for TOF was also documented in patients with TAK.

Updates in cutaneous manifestations of systemic vasculitis.

PURPOSE OF REVIEW: The main purpose of this review is to present newly reported cutaneous manifestations of systemic vasculitis, updates in investigations to verify systemic involvement in cases with cutaneous vasculitis and new therapeutic guidelines. The spectrum of COVID-19-related vasculitis is also covered. RECENT FINDINGS: Only a few reports highlighted new cutaneous presentations or associations with some systemic vasculitic entities. For example, the association of inflammatory disorders with Takayasu arteritis, the importance of considering Kawasaki disease in febrile children with erythema nodosum, the development of necrotic ulcers on fingers and toes in Behçet's disease and the possible presence of polyarteritis nodosa-like pathological features in vulvar ulcers of Behçet's disease. New attempts to classify cutaneous manifestations of giant cell arteritis (GCA) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and the diagnostic investigations for cutaneous vasculitis cases to verify systemic involvement are discussed. Treatment of systemic vasculitis with cutaneous vasculitis should be tailored according to disease status. A plethora of reports in the past 2 years focused on the broad spectrum of COVID-19 vasculitic manifestations. SUMMARY: Although newly reported cutaneous manifestations of systemic vasculitis are relatively uncommon, the plethora of reports in the past 2 years on COVID-19 vasculitis necessitates the expansion of the classification of vasculitis associated with probable cause to include severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) vasculitis.

Treatment of Giant Cell Arteritis and Takayasu Arteritis-Current and Future.

PURPOSE OF REVIEW: Guidelines for the management of large vessel vasculitides have been recently updated by several scientific societies. We have evaluated the current recommendations for treatment of giant cell arteritis (GCA) and Takayasu arteritis (TA) and addressed potential future therapeutic strategies. RECENT FINDINGS: While glucocorticoids (GCs) remain the gold standard for induction of remission, many patients relapse and acquire high cumulative GC exposure. Thus, GC-sparing therapies such as methotrexate are recommended for selected patients with GCA and all patients with TA. Recent high-quality evidence shows that tocilizumab is an effective GC-sparing agent in GCA. Non-biologic and biologic immunomodulators also appear to have GC-sparing properties in TA. Tocilizumab is now considered to be part of the standard treatment for GCA, particularly with relapsing disease, but questions on its use such as length of treatment and monitoring of disease activity remain open. High-quality evidence to guide treatment of TA is still lacking.

Survival After In-Hospital Cardiac Arrest In Critically Ill Patients: Implications For The Covid-19 Pandemic? (preprint)

The coronavirus disease 2019 (COVID-19) outbreak is placing a considerable strain on U.S. healthcare systems. Due to presumptions of poor outcomes in such critically ill patients, many hospitals have started considering a universal do-not-resuscitate order in patients with confirmed Covid-19 given a limited supply of intensive care unit (ICU) beds and the potential risk of transmission of infection to healthcare workers during resuscitation. However, empirical data on survival of cardiac arrest in Covid-19 patients are unavailable at this time. To inform this debate, we report survival outcomes following cardiopulmonary resuscitation in a cohort of similar critically ill patients with pneumonia or sepsis who were receiving mechanical ventilation in an ICU at the time of arrest. The probability of survival without severe neurological disability (CPC of 1 or 2) ranged from less than 3% to over 22% across key patient subgroups, For patients with an initial rhythm of asystole or PEA, who were also receiving vasopressors at the time of arrest, fewer than 10% were discharged without severe neurological disability (CPC of 1 or 2), and this number dropped to less than 3% in patients over 80 years old. In contrast, survival rates were much higher in younger patients, patients with an initial rhythm of VF or pulseless VT, and in patients receiving ventilatory support without vasopressors. Our findings suggest caution in universal resuscitation policies. Even in a cohort of critically ill patients on mechanical ventilation, survival outcomes following in-hospital resuscitation were not uniformly poor and varied markedly depending on age, co-morbidities and illness severity. We believe that these data can help inform discussions among patients, providers and hospital leaders regarding resuscitation policies and goals of care in the context of the COVID-19 pandemic.