Nonspecific Aortoarteritis Research Articles

Malignant arterial hypertension in a child with nonspecific aortoarteritis

The article presents a clinical case of malignant renovascular hypertension in a child, resulting from non-specific aortoarteritis. The disease initially manifested with abdominal pain and convulsions, leading to hospitalization. During the hospital stay, blood pressure readings were elevated to 180/100 mmHg and higher. Over the course of a year, the patient underwent pathogenetic (immunosuppressive), combined (triple) antihypertensive treatment, and symptomatic therapy. Due to the malignant nature of the hypertension, progression of organ damage, a high risk of complications and mortality, and the ineffectiveness of conservative treatment, surgical interventions were performed. The first stage involved polyposition abdominal aortography and stent-graft implantation in the abdominal aorta. Despite restored blood flow in the abdominal aorta, blood pressure remained elevated over the following three months, even with ongoing antihypertensive therapy. As a result, a nephrectomy was performed at the second stage. Postoperatively, the patient’s blood pressure significantly decreased to 130/90–120/80 mmHg. Antihypertensive therapy was continued to reach lower target blood pressure values. Non-specific aortoarteritis remains a prognostically unfavorable condition, even after reconstructive surgery, owning to its autoimmune nature, which suggests ongoing arterial inflammation even during clinical remission. This necessitates lifelong monitoring by a rheumatologist.

New imaging strategy for large vessel vasculitis (based on the EULAR-2023 recommendations)

Large vessel vasculitis (LVV), including Takayasu's arteritis (AT, or non-specific aortoarteritis) and giant cell arteritis (GCA), is caused by granulomatous inflammation affecting mainly the aorta and its main branches. Damage to the vascular wall leads to ischemia of the corresponding organs and can be complicated by loss of vision, cerebral insufficiency and other life-threatening phenomena. The early diagnosis of these diseases in clinical practice is a difficult task that can only be solved by comparing the clinical symptoms, the results of the physical, laboratory and instrumental examination and the vascular biopsy.A comparative analysis of the 2018 and 2023 EULAR recommendations for imaging in LVV is presented. Duplex ultrasound (USDS) of not only temporal but also axillary arteries is recommended for GCA and magnetic resonance imaging (MRI) for AT. Alternative methods for GCA are MRI or positron emission tomography (PET) in combination with computed tomography (CT) and intravenous administration of fluorodeoxyglucose labelled with the short-lived fluoride isotope 18 (FDG-PET/CT), and for AT – FDG-PET/CT, CT or ultrasound examination. MRI, CT or ultrasound can be used for long-term monitoring of structural damage, especially to assess pre-existing vascular inflammation.

Characteristics of patients with non-specific aortoarteritis and arterial hypertension based on retrospective analysis

The aim was to study the medical history, laboratory and instrumental data, the trends of arterial hypertension (AH), risk factors for cardiovascular events, target organ damage (TOD), and the development of cardiovascular complications in patients with NSAA (Non-Specific Aortoarteritis).Materials and Methods. The study included 33 women with confirmed NSAA who had been treated at the 5th Cardiology Department of the E.I. Chazov National Medical Research Center of Cardiology since 2005. Complete blood count, biochemical blood analysis, urine analysis, the following instrumental examinations, such as, ECG, echocardiography, ultrasound of the brachial, femoral, aortic, and renal arteries, MRA or CT angiography with contrast, blood pressure measurement in the arms and legs were performed for all patients. Telephone interviews were conducted to assess some patient's condition over time.Results. The most common symptom (84% of patients) was AH, the average age of AH onset was 30 [19; 40] years and the AH average duration is 7,5 [2; 13] years. Nearly half of the patients (45%) had AH as the leading symptom that led them to perform the examination that helped to the diagnosis of NSAA. The average systolic and diastolic blood pressure values were 123±35 mmHg and 66±17 mmHg in the upper extremities, and 166±31 mmHg and 78±18 mmHg in the lower extremities, respectively. About half of patients (51%) had stage 3 AH, and almost all of them had type 3 according to E. Lupi-Herrera classification. The most common risk factors for cardiovascular events were dyslipidemia (82%), a family history of early cardiovascular events (51%), resting heart rate above 80 bpm (25%), and carbohydrate metabolism disorders (21%). When assessing cardiovascular risk, moderate risk of developing cardiovascular events was observed in 3 patients (10,7%), high risk in 16 patients (57,1%), and very high risk in 9 patients (32,1%). The most common TODs were nervous system damage (53%), including cerebrovascular ischemic events (14%) and left ventricular hypertrophy (42%). This group of patients was characterized by resistant hypertension, and combination with other risk factors for cardiovascular events, it leds to severe TOD and influenced survival rate. In terms of antihypertensive therapy, 61% of patients received combination therapy, with CCB, beta-blockers, and imidazoline receptor agonist being the most prescribed. Two fatal cases were identified in the long-term follow-up, and cardiovascular events, most frequently stroke, were the main complications observed. Additionally, AH progression and its uncontrolled course despite going through multiple drug therapy were characteristic.Conclusion: AH is often difficult to diagnostic in patients with NSAA, and it significantly contributes to the structure of complications and mortality in this patient cohort, progressively worsening over time. It is important to measure blood pressure in both arms and legs to detect elevated values early. The main goals of treatment are achieving NSAA remission and blood pressure control and preventing cardiovascular events.

Difficulties in the Diagnosis and Management of Patients with Takayasu’s Arteritis: A Description of a 5-Year Clinical Follow-Up

Takayasu’s disease (nonspecific aortoarteritis) is a granulomatous inflammation of the aorta and its main branches with a progressive course and development of severe ischemic disorders. The difficulty of diagnosis and the possibility of applying various methods of pathogenetic anti-inflammatory treatment of Takayasu’s arteritis make it expedient to study a clinical case. The analysis of a clinical case of a patient with Takayasu’s arteritis with manifestation of the disease in the form of general inflammatory syndrome and manifestations of severe cerebral ischemia due to bilateral stenotic carotid artery lesion was performed. The patient has been under observation since September 2017 up to the present time, various methods of pharmacotherapy and surgical correction were used in her therapy. The dynamics of clinical symptomatology of Takayasu’s arteritis and clinical results of step therapy with high doses of methylprednisolone, bolus administration of cyclophosphan followed by long-term oral cyclophosphan administration were analyzed. In the course of treatment, the patient underwent carotid angioplasty. Due to the unstable effect of the therapy, the patient was administered intravenous infusions of IL-6 blocker tocilizumab, which led to remission of the disease. The presented clinical case demonstrates the important diagnostic value of vascular imaging methods in early diagnosis and control of the disease course and the effectiveness of IL-6 inhibitors in achieving and maintaining remission of Takayasu’s arteritis.

Неспецифічний аортоартеріїт (хвороба Такаясу): клінічний випадок у педіатричній практиці

The article presents modern data on a relatively rare and life-threatening disease - non-specific aortoarteritis (Takayasu's disease) and its features in childhood. This disease belongs to the group of systemic vasculitis, which is based on non-specific non-infectious inflammation of the aorta and its branches, which leads to deformation of the vascular lumen and secondary tissue ischemia. Damage to vital organs in this disease leads to the development of severe complications and the risk of sudden death. Purpose - to cite a case of Takayasu's disease from our own practice, taking into account the rarity of the disease, lack of awareness and clinical vigilance of pediatricians and family doctors regarding this pathology. A clinical case of non-specific aortoarteritis in a 17-year-old patient who was examined and treated at the Ivano-Frankivsk Regional Children's Hospital was considered. Features of clinical symptoms, laboratory biomarkers, methods of visualization the level of lesions of main vessels are given. Information on methods of treatment of this patient according to the updated recommendations of the European Antirheumatic League (EULAR, 2018) is provided. Conclusions. Nonspecific aortoarteritis is a rare, difficult to diagnose and insufficiently studied autoimmune disease. Late diagnosis significantly increases the risk of a child's sudden death. Modern non-invasive imaging methods are crucial for making a diagnosis, assessing the extent of the disease and choosing treatment tactics. The research was carried out in accordance with the principles of the Declaration of Helsinki. The informed consent of the child's parents and the patient was obtained for the description of the clinical case. No conflict of interests was declared by the authors.

The impact of the COVID-19 pandemic on patients with systemic vasculitis: a single-centre retrospective study.

This study aimed to study the impact of the COVID-19 pandemic on patients living with systemic vasculitis in Kazakhstan. A single-centre retrospective study of the medical histories of 82 patients was carried out based on the regional clinical hospital of the city for all admissions with systemic vasculitis in the period from January 2019 to December 2021. The following qualitative (gender, disability, concomitant diseases) and quantitative (age, disease experience, laboratory data, etc.) variables were studied. To conduct the study, the criteria for the inclusion and exclusion of patients in the study were determined. According to the results of the study, there is a decrease in the number of hospitalized patients with vasculitis in the rheumatology department of the regional clinical hospital. Compared to 2019, in 2021, the number of hospitalized patients decreased by almost half (Table 1).Out of 82 cases, the most common was Takayasu disease (nonspecific aortoarteritis) (43.9%), IgA-vasculitis (Schenlein-Genoch disease) (31.71%), and they are typical mainly for females of rural origin, who were admitted to the hospital in a comorbid state (p < 0.001). 41.6% of patients have disabilities, and the majority of patients have a II disability group. The average body mass index is 24.2; 27 patients out of the total number of patients suffer from obesity. The most common clinical symptoms of patients with systemic vasculitis were injuries of the musculoskeletal system (75.6%). A negative average correlation was found between the indicators of the level of ESR and haemoglobin, the correlation coefficient is -0.535. The patients had concomitant diseases, such as diabetes mellitus, iron deficiency anaemia, coronary heart disease, hypertension, gastrointestinal tract diseases and hepatitis. Women of reproductive age from rural areas are often diagnosed with systemic vasculitis. A high rate of disability revealed among the patients can be explained by two main factors, the first is that the patients consulted the doctors untimely and the second is that the medical community are insufficiently informed about the management of autoimmune rheumatic diseases, in particular about systemic vasculitis, which hinders timely diagnosis and treatment, respectively. Patients, included in this survey, were mostly suffering from diseases of the musculoskeletal system, but depending on the type of vasculitis, other organs and systems may be affected. Table 1 Frequency of patients with systemic vasculitis over 3years Year Frequency % p-value 2019 42 51.2 χ2 = 12.463a; p = 0.002 2020 23 28.0 2021 17 20.7 Total 82 100.0.

Артериит Такаясу после перенесенной коронавирусной инфекции у молодой женщины: клиническое наблюдение

The article describes the clinical onset of Takayasu's arteritis (non-specific aortoarteritis) which occurred after coronavirus disease in a young woman with Still's disease in her medical history. Takayasu's arteritis primarily affects young women, most commonly of Asian race. Clinical manifestations of the disease can be conditionally divided into two groups: non-specific symptoms, indicating a systemic inflammatory response, and specific symptoms associated with the damage of the aorta and its branches. Physical examination plays an important role in diagnosing Takayasu's arteritis. Characteristic signs of the disease include vascular bruits over the large arteries, arterial blood pressure differentials in the right and left extremities, and failure to palpate pulse on the radial arteries (therefore it is also called pulseless disease). Imaging diagnostic techniques include contrast-enhanced angiography and Duplex sonography of the vessels. Etiology of the disease is not fully understood. Research papers present data showing that novel coronavirus infection could be a triggering factor in the emergence of vasculitis of the large vessels, including Takayasu's arteritis. This should be taken into consideration in the management of patients belonging to the risk group. KEYWORDS: Takayasu's arteritis, non-specific aortoarteritis, COVID-19, post-COVID-19 syndrome, vasculitis, Stills' disease. FOR CITATION: Gordeev I.G., Vechorko V.I., Otarova S.M. et al. Takayasu's arteritis after coronavirus disease in young woman: case report. Russian Medical Inquiry. 2023;7(3):181–186 (in Russ.). DOI: 10.32364/2587-6821-2023-7-3-181-186.

АРТЕРИИТ ТАКАЯСУ: СОВРЕМЕННЫЕ ПРЕДСТАВЛЕНИЯ ОБ ЭТИОЛОГИИ, ПАТОГЕНЕЗЕ И ДИАГНОСТИКЕ

The paper discusses the etiology, pathogenesis and problems of modern diagnosis of Takayasu arteritis. The analysis of the information available in modern sources describing this pathology was carried out. An analysis of the clinical case of a patient with nonspecific aortoarteritis is also given. The results of the work determined the dependence of clinical manifestations on the anatomical type of aortoarteritis, as well as difficulties in the modern diagnosis of this pathology, including by the example of a clinical case.

Coronary-Vertebral Collaterals in Takayasu Arteritis: Case Report

INTRODUCTION: PNonspecific aortoarteritis is a rare autoimmune disease with the involvement and narrowing of the aorta and its branches leading to ischemia of the respective arterial region. In the territory of the Russian Federation, the most common manifestation of Takayasu nonspecific aortoarteritis is lesion of the branches of the aortic arch, which in rare cases leads to development of the so called bald arch syndrome. In response to hypoxia, intersystemic collaterals are formed through the neoangiogenesis or redirection of the blood flow from the occluded vessels to small-diameter vessels. In patients with bald arch syndrome, the key role in blood supply to the brain is played by the vertebral arteries. Here, collateral blood supply is realized through the intersystemic anastomoses, most often through the anastomotic leaks between the intercostal and internal thoracic arteries. In the literature, single cases of formation of collaterals between coronary and bronchial arteries are reported. The article presents a clinical case of coronary-vertebral anastomoses in a patient with extremely severe course of Takayasu arteritis with occlusion of the brachiocephalic trunk, right common carotid artery (CA), left common CA, right internal CA, left internal CA (bald arch syndrome). The probable cause of such course of the disease was late referral for medical help by the patient and lack of adequate basic therapy.&#x0D; CONCLUSION: The demonstrated case is the fourth case in the world literature describing the existence of collaterals between the coronary arteries and cerebral arteries, and the first case in the world describing the existence of collaterals from the right and left coronary arteries to the vertebral artery. Such unusual pathway of collateral blood supply in the patient is explained by the absence of the possibility for collateral compensation from the systems of subclavian and intercostal arteries, severe chronic cerebral ischemia. Usually, the causes of angina pectoris in patients with nonspecific aortoarteritis are spread of arteritis to the coronary arteries, insufficiency of the aortic valve, hypertrophy of the left ventricle. In the described case, none of these conditions was present, and angina can only be attributed to the existence of unusual collaterals and the development of a transient steal syndrome.

Role of Cardiac MRI Including LGE, T1 and T2 Mapping in the Assessment of Cardiac Involvement in Patients of Nonspecific Aorto-arteritis: A Prospective Study.

Objective Nonspecific aorto-arteritis (NSAA) may involve the myocardium in the form of edema and fibrosis. We conducted this study to investigate role of cardiac MRI including late gadolinium enhancement (LGE), T1 and T2 mapping in the assessment of cardiac involvement in NSAA. Methods and Materials Over the period between 2016 and 2019, 36 patients with NSAA presenting with uncontrolled hypertension, left ventricular dysfunction, congestive cardiac failure, or tachyarrhythmia were included in the study. We also had 16 voluntary control patients for providing normal T1 and T2 mapping values. Results The average age of patients was 27.1 years and the majority were females. MRI is more sensitive than echocardiography in the detection of LV dysfunction and RWMA. Out of 36 patients, 10 (27.8%) had LGE. The most common pattern of midmyocardial enhancement was present in 5 out of 10 patients. Five (13.8%) patients show mid-myocardial enhancement, followed by epicardial enhancement, which was seen in four (11.11%) patients. The values of post-gad T1 mapping values were significantly lower than pre-gad T1 mapping values. At a cut-off global native T1 mapping value of 1019 milliseconds had the sensitivity of 83.3% and specificity of 81.2% in detecting an abnormal T1 map. No significant association of MRI contrast enhancement with elevated ESR and CRP levels. There was no significant relation of myocardial T2 mapping values between NSAA and control groups. Conclusion Quantitative tissue characterization in the myocardium with native T1 mapping values help in the detection of cardiac involvement in patients with NSAA. T1 mapping may provide incremental value in the assessment of myocardial involvement in NSAA in addition to LGE imaging.

Nonspecific aortoarteritis as a Cause of Aortic Dissection in a Middle Aged Man

Nonspecific aortoarteritis (Takayasu's disease) is a systemic inflammatory disease characterized by granulomatous lesions of the aorta and its large branches. The article describes the clinical manifestations, methods of diagnosis and treatment of this pathology. It also describes a clinical observation that illustrates the delayed diagnosis of the disease in a 47-year-old man, where Takayasu arteritis with lesions of the root, ascending aorta, both common carotid arteries, thoracic and abdominal aorta (type V according to the angiographic classification proposed by Moriwaki R.) manifested a syncopal state, just a few days before the development of a life – threatening complication-aortic dissection of type I according to the DeBakey classification. From a clinical point of view, it is important that Takayasu's disease does not always develop in young people, women, and Asian origin, and, unfortunately, it is often very late to diagnose, although the effectiveness of therapy, including surgical correction of this disease, is entirely determined by the timeliness of its diagnosis.

Surgical treatment of aneurysms of aortic arch branches and vessels of the upper extremities

Aneurysms of theaortic arch branches and vessels of the upper extremities are dangerous and there is a high probability of mortality due to rupture of the aneurysm or stroke. The indication is surgical treatment, regardless of the age and size of the aneurysms. The frequency of deaths and strokes that occurred during operations for carotid artery aneurysms is about 2%, which is associated with distal embolism of intracerebral vessels and the contents of the aneurysm cavity. One of the most common causes of aneurysms of the branches of the aortic arch and vessels of the upper extremities are atherosclerosis, nonspecific aortoarteritis and posttraumatic aneurysms. The study included 38 patients with aneurysms of the branches of the aortic arch and arteries of the upper extremities operated in the vascular surgery department over the past 10 years, whose total age ranged from 19 to 76 years. There were 84% men, 16% women. Aneurysms of the carotid arteries and its branches (temporal artery) were diagnosed in 58%, subclavian arteries in 16%, arteries of the upper extremities in 26%. The size of aneurysms of the branches of the aortic arch and arteries of the upper extremities ranged from 2.5 cm to 10 cm. The average size is 6.3±1.8 cm. When choosing the method of treatment of aneurysms of the branches of the aortic arch and arteries of the upper extremities, preference was given to performing reconstructive operations without the use of additional materials, which were performed in 52% cases), in 13 of these cases, excision of a false aneurysm with stitching of the defect was performed, in 6 of these cases, resections of aneurysms with end-to-end anastomosis were performed. In 48% cases, operations with the use of synthetic materials and autovenous plastic surgery were performed, and endovascular interventions were also performed in 4 cases. The most frequent type of surgery performed is resection of an aneurysm with a lateral suture – 34% cases. Resection of the anastomosis aneurysm at the end of the arteries – 16%, and prosthetics – in 8% cases (synthetic alloprosthetics in one and with autovenous prosthetics in two cases). After resection of subclavian artery aneurysm due to large diastasis of blood flow, reconstruction was performed by bypass surgery or prosthetics. Arterial aneurysm resection with autovenous patch was performed in 13% cases, and with allosunting – in 8% cases. Excision of an aneurysm of the axillary artery on the left with the restoration of a synthetic patch – in 5% cases. Ligation of an aneurysm in the temporal artery was performed in 2 cases (5%), this type of operation was performed with an aneurysm of non-main vessels, as well as in vessels with collateral blood flow in the distal area of the vessels. Implantation of a stentgraft into the right subclavian artery – in 3 cases (8%). X-ray endovascular embolization of aneurysm – in 3% case. Positive results in the postoperative period were observed in 92% patients. One (3%) patient developed complications in the form of hematomas after aneurysm resection. In 5% cases - lymphorrhea after excision of a false aneurysm

Pathohistological characteristics of muscles in patients with chronic lower limb ischemia

To assess the signs of damage (the extent of necrosis and apoptosis, capillary density (CD)) and regeneration (the cross-sectional muscle fiber area (CSMFA), the proportion of centrinucleated muscle fibers (CNMFs), and that of connective tissue), by using the gastrocnemius medial head biopsy specimens obtained from patients with heterogeneous forms of chronic lower limb obliterating diseases (CLLODs). The investigation included the biopsy specimens obtained from 44 men under 65 years of age (their mean age was 54±9.8 years) with Stage IIB-IV chronic limb ischemia (according to the A.V. Pokrovsky-Fontaine classification) with its history of at least six months. The nosological entities were atherosclerotic lesion in 33 patients (distal atherosclerosis n=13), multistage lesion (n=8), and Leriche's syndrome (n=12); autoimmune-mediated vascular injury in 11 patients (Buerger's disease (n=7) and nonspecific aortoarteritis (n=4)). The similar muscle fragments obtained during autopsy from the deceased without obvious signs of cardiovascular system diseases were examined as a control. It was found that there was a statistically significant difference between the nosological entities, as compared to the control in terms of CD and CSMFA (a decrease), the proportion of CNMFs and that of connective tissue (an increase). No substantial differences were found in the studied parameters between the nosological entities. The findings may suggest the universal mechanism for damage to striated muscle tissue because of circulatory hypoxia, regardless of its etiology and the common character of tissue compensatory-adaptive responses (regeneration).

Surgical treatment of visceral and renal abdominal artery aneurysms of various etiology

Visceral artery aneurysms are detected when complications occur or accidentally during examination for other diseases gastrointestinal tract, aimed at determining the functional significance. Over the past 10 years, we have observed 31 patients with abdominal visceral artery aneurysms, 10 patients had renal aneurysms, 15 had splenic arteries, hepatic artery and pancreatoduodenal artery aneurysms – in one case. The combination of aneurysms – in 2 cases: in one case – the renal artery with the splenic artery, in the other – the renal artery and the abdominal trunk. The patients ranged in age from 24 to 78 years, the average age is 43±6.5. Previously, infection was considered the most common cause of splenic artery aneurysms in 10 cases, with most patients complaining of fever, abdominal pain and a pulsating mass in the abdomen. And two patients with splenic artery aneurysm noted abdominal trauma. 3 had atherosclerotic changes. The causes of renal artery aneurysms are atherosclerosis in 6 cases, fibromuscular dysplasia in 2 cases, and Nonspecific aortoarteritis in 2 cases. Also, atherosclerotic etiology is present in aneurysms of the external iliac arteries aneurysms. Reconstructions on hepatobiliary aneurysms were performed in 6 (20%) cases, in 5 cases - resection with end-to-end anastomosis and in 2 cases - resection of splenic artery aneurysm, resection of splenic artery aneurysms with autovenous patch, clipping of splenic artery aneurysms – in 2 cases (6.5%), endovascular techniques were applied in 18 cases (58%). In 2 cases, nephroectomy was performed due to the spread of the aneurysm into the kidney gate and the lack of conditions for reconstruction. In the immediate and long-term (after 6 months and 3 years) postoperative period, the preservation of organ function in reconstructive and ligating methods of surgical treatment is the same. Signs of ischemia and impairment in the immediate postoperative period were not observed with reconstructive and ligating methods of surgical treatment. The choice of treatment method depends on the severity of the patient’s condition, clinical manifestations, as well as the localization of the aneurysm and its morphology. Preference is given to endovascular techniques, which are not traumatic enough and provide a good long-term result. However, in each case, the patient’s management tactics are individual, the choice of the optimal method of treatment of visceral artery aneurysms is based on data from the entire spectrum of possible diagnostic manipulations.

Артериит Такаясу – актуальная проблема современной ревматологии (обзор литературы)

Takayasu’s arteritis is a rare chronic large-vessel vasculitis that predominantly affects the aorta, its major branches and pulmonary arteries. The exact occurrence rate of this pathology is unknown. However, it has been found that the disease is registered in 2.6 people per 1 million of the population in Russia, more oft en in young females. As with all rheumatic diseases, the issues of pathogenesis and therapy of non-specific aortoarteritis remain open. The complexity of pathogenetic reactions appears to be a problem for early diagnosis and selection of the correct therapy. Th is review covers the issues of pathogenesis and modern pharmacotherapy: tumor necrosis factors, pharmacotherapy and genetically engineered drugs. The review was written based on analysis of scientific literature. The eLIBRARY.RU, Cyberleninka, Pub Med, Cochrane Library databases were searched for materials published within the period of 2002-2021 with an emphasis on the past 5-10 years for the following keywords: Takayasu arteries, nonspecific aortroartery, systemic vasculitis, rheumatological diseases

Takayasu’s arteritis in a child with prolonged fever. Differential diagnosis. Clinical case analysis

Takayasu’s arteritis is a chronic granulomatous arteritis with predominant involvement of the aorta and its main branches. It occurs both in youth and childhood, manifests between the ages of 10 and 30 years, female patients predominate. This article presents a clinical case of Takayasu’s arteritis in a child with prolonged fever. Clinical case. A 10-year-old girl E. was hospitalized with complaints of febrile fever episodes during the last two months; weakness and back pain were also noted. Observed by an endocrinologist since 2015 with a diagnosis of Stunted growth. Delayed physical development. Diffuse changes of the thyroid gland, euthyroidism. Since 2020 has been observed with the diagnosis Constitutional immaturity. A tendency toward constipation was noted. Observed by an allergist with atopic dermatitis, food and respiratory allergies were diagnosed. By the time of hospitalization, the girl had been ill for two months. During this period, four episodes of fever with scanty catarrhal manifestations were recorded. The diagnostic search went in different directions, infectious, systemic and oncological diseases were excluded. The echographic pattern was typical and highly specific for Takayasu’s arteritis. Additional examination also revealed changes in the celiac trunk in the form of narrowing of its lumen. Clinical diagnosis «Non-specific aortoarteritis, type III, acute phase, stenotic variant, grade 1–3 activity with lesions of the right and left common carotid arteries and the celiac trunk» was made as a result of clinical, laboratory and instrumental examination. This clinical observation demonstrates the difficulty in making a diagnosis due to the variety of clinical forms and the absence of specific disease markers. It takes on average a year and a half from disease debut to clinical diagnosis of the patient. However, lack of therapy over a long period can be crucial for an adverse outcome of the disease.

Non-specific aortoarteritis (NSAA) in children: a prospective observational study

ObjectiveProspective data on clinical profile, natural history and outcomes of NSAA (non-specific aortoarteritis) in children is limited. We initiated this prospective study to evaluate the short-term and medium-term outcomes of...

Paraneoplastic Vasculitis in a Patient with Astrocytoma of the Brain

Clinical case of paraneoplastic vasculitis аssociated a brain tumor was presented. Paraneoplastic vasculitis is a rare type of paraneoplastic syndrome. The frequency of detection of paraneoplastic vasculitis in cancer patients is 0.01-5%. In 70% of cases, the manifestation of vasculitis is observed long before the clinical manifestations of the tumor. Most studies report so-called leukocytoclastic vasculitis (allergic) or allergic angiitis. Vasculitis is usually accompanied by slowly progressing tumors such as breast and prostate cancer. It also develops with of stomach cancer, lung cancer, kidney adenocarcinoma, epithelioma, sarcoma, cholangiocarcinoma, other solid tumors, multiple myeloma, non-Hodgkin’s lymphoma. The nosological forms of paraneoplastic vasculitis include called polyarteritis nodosa, hemorrhagic vasculitis, Wegener’s granulomatosis, non-specific aortoarteritis, idiopathic pulmonary hypertension syndrome, thrombovasculitis, allergic hemorrhagic vasculitis, cutaneous vasculitis, systemic necrotizing vasculitis with increased ANCA titer. The patient suffered from paraneoplastic vasculitis with the development of amyloidosis of vascular tissues and arterial hypertension. The progression of the vascular process led to damage of the arteries of the brain and heart, the development of ischemic stroke and hemodynamically significant stenosis of the coronary arteries, the development of acute myocardial infarction complicated by acute heart failure, which caused death. The clinical significance of the case lies in the fact that paraneoplastic vasculitis, which was developed due to a brain astrocytoma with the formation of amyloidosis was firstly described.

Aortic regurgitation: epidemiologic, etiologic, and pathophysiologic characteristics

The presented review concerns aortic regurgitation which occupies a significant place in the structure of valvular heart disease. The detailed anatomic and physiologic description of the aortic valve is provided. The characteristics of sinotubular, ventricular-aortic junctions, and virtual aortic annulus are presented. There are data about prevalence of aortic regurgitation on the basis of results of population studies, indicating the increase in incidence of aortic regurgitation among individuals older 70–74 years. The detailed etiologic structure of this valvular pathology is described with specifying of the most common causes of both aortic disease and aortic cusps alterations. In particular, there are some aortic diseases, resulting in acute aortic regurgitation, including acute aortic dissection and paravalvular regurgitation in incompetence of the prosthetic aortic valve; in chronic one – idiopathic dilation of the aortic root, inherited connective tissue dysplasias (Ehlers–Danlos, Marfan, and Loeys–Dietz syndromes), bicuspid aortic valve, aortitis of various origin, seronegative arthropathies (reactive, psoriatic arthritis, ankylosing spondylitis) etc. Infective endocarditis and traumatic exposure are commonly responsible for development of acute regurgitation due to aortic cusps abnormalities. Chronic aortic regurgitation as a consequence valve defects occurs in rheumatic heart disease, degenerative changes, congenital anomalies, systemic connective tissue diseases (systemic lupus erythematosus, rheumatoid arthritis), non-specific aortoarteritis, etc. The special attention is paid to pathophysiologic features of acute and chronic aortic regurgitation in the review. Acute aortic regurgitation is characterized by sudden increase in end-diastolic volume and due to the noncompliant left ventricle of normal size, it undergoes abrupt exposure a significant pre-load and after-load which results in decrease of left ventricle systolic function and stroke volume despite on relative preservation of contractile function of myocardium. In contrast to acute aortic regurgitation it is remarkable in its chronic form slow, progressive influence by increased overload of the left ventricle with possibility to adapt driven by its gradual dilation and hypertrophy.

Dynamics of Indicators of Immune Status and Endothelial Function in Patients with Nonspecific Aortoarteritis On the Bacround Of Combined Therapy with Eqvator And Plavi?

Dynamics of Indicators of Immune Status and Endothelial Function in Patients with Nonspecific Aortoarteritis On the Bacround Of Combined Therapy with Eqvator And Plavi?