BACKGROUND: Tuberculosis (TB) is a highly prevalent disease associated with significant morbidity and mortality globally, and is reported to be associated with the onset of autoimmunity. This study investigated the association between TB and the incidence of systemic vasculitides (SV).
METHODS: Data were obtained from the South Korean National Claims database to identify patients with TB and controls (who had undergone appendectomy). The overall occurrence of SV and disease subtypes during the observation period was compared between the two groups. Adjusted Cox proportional hazards regression and Kaplan-Meier analysis were performed to identify the relationship between TB and SV and to compare SV incidence.
RESULTS: We identified 418 677 patients with TB and 160 289 controls. The overall SV incidence rate was 192/1,000 000 person-years during a mean follow-up of 7.5 years and was higher in patients with TB than controls. Cox regression revealed that the risk of SV was elevated in the TB group independently (adjusted hazard ratio [aHR]: 1.72, 95% confidence interval [CI]: 1.45-2.05). Furthermore, the risk of SV was significantly higher in extrapulmonary TB (aHR: 4.28, 95% CI: 3.52-5.21) when the TB group was categorized into pulmonary and extrapulmonary TB. The findings remained identical even after applying a stabilized inverse probability of treatment weighting analysis.
CONCLUSIONS: Patients with TB have increased risk of SV, which is prominent in extrapulmonary TB. As well as confirming TB is associated with increased incidence of immune-related vasculitis, our findings highlight the need for clinical vigilance for early diagnosis and initiation of treatment.

Systemic vasculitides are the most complex and problematic autoimmune rheumatic diseases characterized by affections of large, medium, or small vessels. Although the immunopathogenesis of vasculitides is thoroughly studied, the epidemiology and etiology are poorly explored. The main triggers of vasculitides are environmental, genetic, and various infectious factors. Diagnosis of vasculitides is complicated due to the non-specific nature of their symptoms. Vasculitides affect various organ systems with abrupt or slow (weeks-months) development of symptoms. This study aims to analyze the demographic and clinical-anamnestic characteristics of patients with systemic vasculitides in a single centre before and during the COVID-19 pandemic in Kazakhstan. A single-centre retrospective study of medical records of 80 patients above 18 years was conducted in the Almaty City Rheumatology Center. Medical records of 24 males (30%) and 56 females (70%) with systemic vasculitides, diagnosed from January 2019 to December 2021, were analyzed. Age, gender, damaged organ systems, disability, concomitant diseases, disease experience, laboratory data, and other variables were recorded. The records of hospitalized patients with systemic vasculitides were analyzed. Of 80 patients registered in 2019-2021, the most common were those with IgA vasculitis (n = 32, 40%), Takayasu arteritis (n = 17, 21.25%), and granulomatosis with polyangiitis (n = 12, 15%). Behçet disease was diagnosed less frequently (n = 9, 11.25%). Patients with systemic vasculitides had pre-obesity (n = 19), class 1 obesity (n = 13), and class 2 obesity (n = 2). Musculoskeletal affections were present in 52 patients (65%). Gastrointestinal, cutaneous, and cardiovascular affections were recorded in 45 (56.3%), 37 (46.3%), and 39 (48.8%) cases, respectively. Only 8 patients (10%) had affections of the nervous system. Most patients had elevated C-reactive protein (n = 29, 36.3%) and leukocytosis (n = 33, 41.3%). One-third of patients with vasculitides had a history of abortions. Musculoskeletal, cutaneous, gastrointestinal, and cardiovascular affections are common in patients with systemic vasculitides. Obesity is a frequent comorbidity in vasculitides. Comorbidities and abortions complicate the disease course and its management.

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), namely granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis constitute a group of rare systemic vasculitides, affecting small vessels. Genders are equally affected, with symptoms most commonly presenting during and/or after the fifth decade of life, but AAV may also present in younger individuals. As advanced maternal age is becoming common and safe over the last decades, it is now more feasible for middle-aged women suffering from AAV to get pregnant. Although adverse pregnancy outcomes have been thoroughly investigated in other systemic diseases, the exact prevalence of pregnancy complications and unfavorable outcomes in pregnant women with AAV has not been systematically evaluated.
We researched PubMed, Scopus, Cochrane Library and Cinahl databases until September, 2022. Three blinded investigators extracted data and assessed the risk of bias. A random effects model was used for the analysis. The outcomes studied were pre-term delivery, intrauterine growth restriction (IUGR) neonates and disease flare.
We included six studies with 92 pregnancies in patients with AAV. The prevalence of pre-term delivery, IUGR neonates and disease flare were 18% (CI: 0.10-0.30, P=non-significant), 20% (CI: 0.11-0.33, P=non-significant) and 28% (CI: 0.09-0.59, P<0.01), respectively.
The analysis demonstrated higher occurrence of adverse outcomes in pregnant women suffering from AAV accompanied by an increased risk of disease flare during pregnancy. These findings underline the importance of preconception counseling and the necessity of close monitoring in these patients similarly to other systemic inflammatory diseases.

暂无摘要。

Systemic vasculitides represent uncommon conditions characterized by the inflammation of blood vessels that can lead to different complex disorders limited to one organ or potentially involving multiple organs and systems. Systemic vasculitides are classified according to the diameter of the vessel that they mainly affect (small, medium, large, or variable). The pathogenetic mechanisms of systemic vasculitides are still partly unknown, as well as their genetic basis. For most of the primary systemic vasculitides, a single gold standard test is not available, and diagnosis is often made after having ruled out other mimicking conditions. Current research has focused on new management protocol and therapeutic strategies aimed at improving long-term patient outcomes and avoiding progression to multiorgan failure with irreversible damage. In this narrative review, authors describe different forms of systemic vasculitides through a review of the literature, with the aim of highlighting the current knowledge and recent findings on etiopathogenesis, diagnosis and therapy.

Investigation\'s history and nomenclature\'s evolution of the IgA-vasculitis are presented in the article. Pathogenesis of the renal and skin damages is discussed in details, particularly abnormalities of the IgA-immunity and systemic endotoxemia. Relevant world\'s literature is cited.

The paper describes clinical cases in 2 patients (brothers) with giant cell arteritis. It analyzes the genetic and epigenetic aspects of the disease. The data available in the Russian and foreign literature are given.
Представлены клинические наблюдения 2 больных (родные братья) с гигантоклеточным артериитом. Анализируются генетические и эпигенетические аспекты заболевания. Приводятся данные отечественной и зарубежной литературы.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are near universally fatal conditions if untreated. Although effective therapeutic options are available for these diseases, treatment regimens are associated with both short- and long-term adverse effects. The recent identification of effective B-cell-targeted therapy with an anti-CD20 monoclonal antibody has transformed the treatment landscape of AAV. Questions, nevertheless, remain regarding the appropriate timing, dose, frequency, duration, and long-term effects of treatment. The aim of this article is to provide an overview of the current information, recent advances, ongoing clinical trials, and future treatment possibilities in AAV.

The greater understanding of pathophysiology and behavior of systemic vasculitis, together with the development of therapeutic regimens with increasingly better safety and efficacy profiles, dramatically changed the prognosis of patients diagnosed with these clinical entities. Recently, the use of rituximab in the treatment of patients with ANCA-associated vasculitis in randomized clinical trials showed an important alternative in selected cases, especially patients refractory or intolerant to standard therapy with cyclophosphamide and corticosteroids. This article presents the report of seven cases of systemic vasculitis successfully treated with rituximab.