Hemorrhagic Vasculitis Research Articles

27395 ANCA-positive hemorrhagic vasculitis with severe myositis—Atypical presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Patient history: A 41-year-old female with a past medical history of asthma presented to the emergency department for diffuse myalgias and a cutaneous eruption gradually worsening over two weeks. Physical examination showed symmetric violaceous papules and nodules on the dorsal arms and hemorrhagic vesicles overlying palpable purpura on the lower extremities. During admission she developed profound dysphagia and severe systemic muscle weakness.

Fundamentals of differential diagnosis of hemorrhagic vasculitis

It is quite rare, but in the practice of a family doctor, a condition may occur when, after an upper respiratory tract infection, flu or tonsillitis, the patient develops petechial rashes, hemorrhages in the mucous membranes, articular syndrome develops and abdominal pain appears. The first elements of the rash appear symmetrically first on the lower extremities, and then spread throughout the body. Joint lesions are characterized by severe pain syndrome and swelling; changes usually affect large joints – knee and ankle, but sometimes smaller articular surfaces are affected. Hemorrhages in the intestinal wall and mesentery are the cause of abdominal pain, flatulence, bowel disorders; nausea and vomiting are possible, sometimes mixed with blood. All these signs are characteristic of hemorrhagic vasculitis, which is also called Henoch–Schonlein purpura, capillary toxicosis or allergic purpura. For the first time, a description of several cases of purpura associated with arthritis was given by a German doctor Johann Schonlein in 1937, and a few decades later this nosology was described in detail by Eduard Henoch in his scientific work. In this pathological condition, autoimmune damage to small arteries is observed, as a result of which they become fragile, their permeability increases, resulting in the development of microthrombosis and multiple small hemorrhages in the skin, mucous membranes and internal organs. The disease most often occurs in children and adolescents, however, cases of diagnosis of hemorrhagic vasculitis have been described in adults as well [7].

Новообразование орбиты у пациента с хроническим вирусным гепатитом С с системными проявлениями (клиническое наблюдение)

Aim. To present the case report of orbital tumor in patient with chronic hepatitis C with systemic manifestations, diagnostic and treatment approaches in such patients. Material and methods. We describe a clinical case of 48 y.o. female patient complaining of right-sided upper eyelid swelling and ptosis. According to MRI an orbital tumor was previously visualized in the location of right lacrimal gland. It was known, that the patient suffers from HCV infection since 1992. She was prescribed local and systemic immunosuppressive treatment with a positive effect, however due to hemorrhagic vasculitis development and eyelid swelling recurrence 2 weeks later she was admitted to the hospital. In diagnostic purpose the orbital tumor was surgically excised followed by morphological and immunohistochemical investigation. Results. A differential diagnosis between chronic dacryoadenitis, lymphoid tissue hyperplasia (due to longstanding antigene stimulation (MALT – tissue) and marginal lymphoma (MALT – lymphoma) was performed. Immunohistochemical staining and PET – CT failed to confirm the lymphoproliferative disease. However, in 1 year due to tumor recurrence, repeated biopsy followed by previously described analyses confirmed extranodal marginal zone B-cell lymphoma (MALT – lymphoma). Repeated PET-CT showed metabolic activity in the right orbit and in the location of paraesophageal lymph node. The patient received polychemotherapy inducting lymphoma remission. Conclusion. Current clinical case shows that differential diagnosis of orbital tumors in patients with chronic hepatitis C should include lymphoproliferative diseases. Key words: orbit neoplasm, dacryoadenitis, lymphoma, chronic viral hepatitis C.

Pathogenetic influence of liver steatosis in patients with hemorrhagic vasculitis: correlation analysis

According to own data, 74% of patients with hemorrhagic vasculitis have sonographic signs of liver steatosis, but its influence on clinical and laboratory parameters of vasculitis is not established yet.
 The aim – to reveal the pathogenetic links of liver steatosis influence using correlation analysis, which could help in the planning of management strategy.
 Matherial and methods. We retrospectively analyzed the data of 50 inpatients with the help of Pearson’s correlation analysis.
 Results. In patients with hemorrhagic vasculitis, liver steatosis was more prominent in older ones and in higher body weight patients. Liver steatosis was accompanied by more significant changes in the right ventricle, left atrium, and aorta. It was also associated with systemic inflammation activity, serum proteolytic and thrombotic activity. In patients with liver steatosis, their age – a non-modifiable cardiovascular risk factor – was strongly associated with the deterioration of heart condition and changes of serum proteolytic and thrombotic activity, which, in turn, creates the background for adverse cardiovascular events. Liver steatosis was accompanied by the activation of systemic inflammation, which led to the transformation into steatohepatitis.
 Conclusion. The influence onto the liver steatosis is the only way of prevention of cardiovascular events and steatohepatitis in patients with hemorrhagic vasculitis.

Rituximab for rheumatic diseases in children: Results of a retrospective study of the safety of therapy

Objective: to analyze the safety of rituximab (RTM) in children with various rheumatic diseases.Materials and methods. The retrospective study included 81 pediatric patients with a confirmed diagnosis of rheumatic disease. Data on the safety of RTM were analyzed for all patients who received at least one infusion of the drug. All patients underwent a standard clinical, laboratory and instrumental examination in accordance with the verified diagnosis before the appointment of RTM therapy. The dose of RTM for administration was calculated based on 375 mg/m 2 of body surface area, the drug was administered once a week for 1 to 4 consecutive weeks, depending on the number of CD19 lymphocytes determined after the infusion, and the tolerability of therapy.Results. Among the patients included in the study, 38 (46.9%) were with systemic lupus erythematosus (SLE), 16 (19.75%) – with juvenile idiopathic arthritis (JIA), polyarticular variant (14 (87,5%) of them – RF-positive), 9 (11,1%) – with juvenile idiopathic arthritis with systemic onset (sJIA), 6 (7.4%) – with systemic sclerosus (SSc), 5 (6.2%) – with primary Sjogren’s syndrome, 2 (2.5%) – with juvenile dermatomyositis, 4 (4.9%) – with mixed connective tissue disease, and 1 – with livedoid vasculopathy. 53 (65.4%) patients underwent more than one course of RTM therapy, with a maximum of 10 courses. The total number of infusions was 198. The median time between each course was 182 [156–315] days. RTM was effective in 67 (95%) patients, ineffective in 2 (2.5%) patients with sJIA, 2 (2.5%) patients with SLE and macrophage activation syndrome (MAS).Adverse reactions (AE) of mild to moderate severity were reported in 23 (28.4%) patients, including upper respiratory tract infections – in 7 (8.6%), urinary tract infections – in 2 (2.5%), mild infusion reactions that did not require discontinuation of therapy – in 2 (2.5%), clinically insignificant neutropenia (I–II degree) – in 4 (4.9%), a decrease in IgG levels – in 14 (17.5%) patients (median – 5.5 [4.0; 6.9] g/l). Two patients with sJIA had persistent hypogammaglobulinemia for 3 and 5 years after the last RTM infusion, respectively. The incidence of infections in patients with low IgG levels was 35.7%, and no cases were registered in patients with neutropenia. Serious AE was reported in 16 (19.7%) patients: sepsis – in 4 (4.9%), pneumonia – in 3 (3.7%), herpes zoster – in 1 (1.2%), serious infusion reactions – in 2 (2.5%), serious postinfusion reactions within 3–10 days – in 4 (4.9%) (in 3 patients (3.7%) – MAS, in 1 (1.2%) – hemorrhagic vasculitis); death was registered in 2 cases of SLE and MAS (therapy of RTM was inefficient). In general, various AE were reported in 55.6% of patients with sJIA, 52.6% of patients with SLE, 50% of patients with SSc and juvenile dermatomyositis, and 80% of patients with primary Sjogren’s syndrome. Discontinuation of therapy due to serious AE was observed in 15 (18.5%) patients.Conclusion. Our study demonstrated that RTM therapy is highly effective with an acceptable safety profile in children with rheumatic diseases. The safety data obtained indicate the need for careful monitoring of therapy, primarily taking into account the risk of infection, despite the fact that in this study the frequency of infectious complications was not high. A decrease in IgG level was observed in a small proportion of patients and did not correlate with the incidence of infections.

P053 Fulminant purpura in a patient with SARS-CoV-2

Background/Aims According to available data children with SARS-CoV-2 has asymptomatic or a milder clinical course, but there are known cases of multisystem inflammatory syndrome. Methods we observed a case of fulminant purpura in a boy with SARS-CoV-2. A three years old boy had febrile fever, vomiting, abdominal pain for no apparent reason. On the 2nd day this boy had joint pain, petechial rashes, fever and abdominal pain persisted. The patient was hospitalized in the pediatric Department, where he was diagnosed with hemorrhagic vasculitis, there was prescribed therapy with glucocorticosteroids (1 mg/kg per day), dipyridamole (2.5 mg/kg per day), oral cephalosporin. Against the background of ongoing therapy, the child's condition became worth. On the 5th day of the disease appeared necrosis in the area of the ankle joints, heels, and toes, patient was transferred to the pediatric Department of Children's Clinical Regional Hospital. Results at the time of hospitalization, the child's condition was severe: the boy had persistent high fever, petechial rash on the torso and legs, necrosis in the area of the left ankle, outer surface of the right ankle and in the area of both heels, edema of the feet and hands. The boy was in a forced position, couldn’t not walk and had intense pain in the shoulders, elbows, wrists, hips, knees and ankles. Blood tests revealed anemia (HGB 73 g/l), thrombocytopenia (PLT 120 x 109/L), leukocytosis (WBC 23 x 109/L), ESR 60 mm/h; in the clinical analysis of urine - proteinuria. In the blood test was detected significantly increased levels of CRP (129 mg/L), ferritin (637 ng/mL), triglycerides (1.39 mm/L), hypoproteinemia (48 g/L), hypoalbuminemia (31 g/L); the level of ALT, AST, LDH was normal. The level of D-dimer was 4792ng/mL, fibrinogen - 8.8 g/L, antithrombin III - 156%, protein C - 64%, coagulation Factor IX activity - 150 %, coagulation Factor VIII activity 289%. An increase of antinuclear antibodies, cardiolipins, phospholipids and ANCA were excluded. A nasopharyngeal swab was negative for SARS-CoV-2 (was made on the 5th day of the disease). On 14th illness day we detected IgM antibodies directed toward SARS-CoV-2 in this patient (IgG antibodies directed toward SARS-CoV-2 were absent). An echocardiogram showed no coronary artery abnormalities, echocardiogram was normal, a chest CT and MRI of the brain also were normal. The diagnosis was fulminant purpura in a patient with SARS-CoV-2. This boy received heparin, IVIG 2 g/kg, dexamethasone 10 mg per meter of body surface a day, antibiotic therapy was continued. As a result fever, petechial rash and necrosis regressed. After 4 weeks we detected IgG antibodies directed toward SARS-CoV. Conclusion the current case demonstrates the possibility of developing a multi-system inflammatory syndrome like fulminant purpura in patients who had SARS-CoV-2. Disclosure N. Tsurikova: None. S. Rodionocskaya: None. E. Ligostaeva: None. V. Avdeenko: None. N. Kobzeva: None. I. Tsiganok: None.

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.

ИММУНОЛОГИЧЕСКАЯ ХАРАКТЕРИСТИКА ГЕМОРРАГИЧЕСКИХ ВАСКУЛИТОВ У ДЕТЕЙ

Currently the incidence of hemorrhagic vasculitis in children is characterized by an increase in all age groups what may be associated with environmental problems, chronic infection as well as food and drugs allergy. To date there are no biomarkers predicting the further development of hemorrhagic exanthema at the onset of the disease and followed its outcome. The quantitative and functional features of cellular and humoral immunity in children with hemorrhagic vasculitis of various origins accompanied by hemorrhagic exanthema at the onset of the disease are presented in this article. The reduction of γδТ-cells number in the peripheral blood correlated with levels of specific antineutrophil cytoplasmic antibodies and IgG EBNA-1 antibodies against a high ability of γδТ-cells to activation were established in children with systemic (immunocomplex) vasculitis. At the contrast, the background of a normal γδT-lymphocytes’ content was associated with the decrease in γδТ-cells activation capacity in children with hemorrhagic vasculitis of infectious-allergic genesis. Thus, quantitative and functional changes in γδТ-lymphocytes can be a potential labor biomarker for early diagnosis of systemic or infectious-allergic hemorrhagic vasculitis in children what may be used for the further disease course prediction.

Joint Manifestations in Children with Familial Mediterranean Fever in Armenia: Clinical and Genetic Characteristics

Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disease in the group of Hereditary periodic fever syndromes (HPFS), characterized by recurrent, self-limited attacks of fever and polyserositis, which last 2-4 days. It manifests mainly in childhood and often has early onset, resolve without sequels. Genetic testing of FMF is efficient and allows diagnosing of atypical cases. FMF is a significant health care problem in Armenia because of high frequency of carriers of MEFV mutations 1:3 - 4 (0.21) and marked FMF prevalence 54.7 per 10`000 of the total population. Articular symptoms are found in about 45% of cases. They usually manifest as acute recurrent arthritis (ARA) or arthralgia, but more rarely also chronic arthritis can be found. We aimed to investigate clinical and genetic characteristics of the joint manifestations in Armenian children with FMF. The charts of all 715 patients with proven diagnosis of FMF at the National Pediatric Center (NPC) for FMF were reviewed for joint manifestations. The diagnosis of FMF and disease severity were determined according to the Tel-Hashomer criteria and molecular-genetic detection of 12 MEFV mutations common for Armenians. The characteristics of joint manifestations and overall FMF disease were then compared to MEFV mutation analysis. There were 438 boys and 277 girls with an age at diagnosis between 3 months and 17 years (mean age: 8.64±0.17). Joint involvement was observed in 56.4% of all 715 cases. The manifestation was ARA in 30.5%, arthralgia in 21.2% and chronic arthritis (CA) in 4.7%, which would also qualify for a diagnosis of JIA. The frequency of ARA was associated with M694V mutation, mainly M694V homozygous and M694V heterozygous genotypes. The risk of CA depended on MEFV genotype of the FMF patients and was the highest in M694Vheterozygotes and M694Vhomozygotes. M694V heterozygous genotype was noticed significantly more frequently among FMF patients with spondyloarthritis in comparison to those without it. The probability of the development of CA among FMF patients without M694V mutation was significantly higher also in heterozygotes in comparison to compound-heterozygotes. We concluded, that the frequency of joint involvement among Armenian pediatric FMF patients was high - 56.4%, especially for CA. Chronic arthritis may be the first manifestation of FMF and occurred usually in patients with severe M694V mutations. Patients of Armenian origin with refractory arthritis should be asked for isolated febrile attacks, hemorrhagic vasculitis, episodes of pleuritis and family history to rule out FMF.

Modern views at the morbidity and course of Henoch-Schönlein purpura in children: a literature review

The article presents data on the prevalence, etiology, pathogenesis and clinical features of hemorrhagic vasculitis in children, based on the modern scientific literature. The aim of the study was to establish frequency and features of Henoch-Schonlein purpura course in children over the past decade. Materials and methods. Scientific and metric systems Google Scholar, PubMed, CyberLeninka, Cochrane database for the period of 2015–2019 were searched, using the keywords “hemorrhagic vasculitis”, “IgA-аssociated vasculitis”, “acute respiratory infections”, “bacterial infections”, “etiology and pathogenesis”, “clinics”. The frequency of the disease in the population was also analyzed, according to the data of the initial presentation at the inpatient and polyclinic of the Ivano-Frankivsk Regional Children's Clinical Hospital of the Ivano-Frankivsk Regional Council, for the period between 2015 and 2019 respectively. Conclusion. It was established that hemorrhagic vasculitis is the most common in childhood, and is diagnosed approximately in 50 % of cases. The disease is triggered mostly by the genetic, infectious and non-identified factors. The authours have distinguished such pathogenic links as glycosylation disorders of immunoglobulin A, the accumulation of circulating immune complexes, resulting in specific inflammation and blood clotting disorders with subsequent development of thrombosis. There are following clinical findings for hemorrhagic vasculitis: palpable purpura, diffuse abdominal pain, accumulation of IgA (confirmed by biopsy of any organ); arthritis or arthralgia; kidney damage (hematuria and/or proteinuria). These data need to be clarified, and further clinical, cohort, and prospective studies should be conducted.

Characterization of high virulent strains of equine rhinopneumonitis virus

This article presents results of screening samples collected from horses during acute outbreaks of abortion and neurological disorders recorded in 2019-2020 in Russia. The signs of disease were characterized by severe hemorrhagic vasculitis. Massive hemorrhages were observed in organs of aborted fetuses and dead horses. Equine herpesvirus type one was isolated in cell culture. Testing to identify genes encoding glycoprotein B and polymerase catalytic subunit of certain viral isolates were carried out. Virus isolated from horses with signs of encephalomyelitis from the Northwest region had a high degree of phylogenetic relationship with the strain “89c25” of equine herpesvirus type one from Japan, 1989. The same virus was found in horses with signs of rhinopneumonia from the Ural region. The virus isolated after equine abortion from the North Caucasus region had a high degree of phylogenetic relationship with the strain “IT19m10” of equine herpesvirus type one from Italy, 2019. It was established that all viral isolates belong to non-neuropathogenic genotype - A2254.

PATHOGENETIC ASSOCIATION OF VASCULAR AND CARDIAC LESIONS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: CHARASTERISTICS AND PREVALENCE

Introduction. Vascular and cardiac lesions are the most common lesions in patients with systemic lupus erythematosus (SLE). They also top the list of mortality reasons in patients with SLE and yet have to be addressed individually.
 Objective. To characterize and clarify the prevalence of vascular and cardiac lesions that are pathogenetically associated with systemic lupus erythematosus.
 Materials and methods. 370 patients (331 women and 39 men) were included in the study with a prior stratification by age, SLE duration and its degree of severity. The patients were comprehensively examined and diagnosed according to the requirements of modern medicine. In particular, the patients were subjected to electrocardiography, echocardiography, ultrasonography of lower limb veins and aortic arch branches, blood pressure monitoring, Holter monitoring, capillaroscopy, as well as ophthalmoscopy. The obtained data was processed in Microsoft Excel by means of descriptive statistics, χ2 test and z-test for comparing two proportions; the relationship was considered to be statistically significant when p < 0.05.
 Results and discussion. 350 patients (94.60%) with systemic lupus erythematosus had vascular and cardiac lesions, especially Raynaud syndrome, atherosclerosis, retinal angiopathy, livedo reticularis, hemorrhagic vasculitis, capillaritis, varicose veins, veinous thrombosis, post-thrombotic syndrome, lymphedema, venous ulcers, thrombophlebitis, secondary hypertension, primary hypertension, pulmonary hypertension, myocarditis, mitral insufficiency, aortic insufficiency, tricuspid insufficiency, atherosclerotic heart disease, ischaemic heart diseases: stable angina, old myocardial infarction, cardiomyopathy. Of these lesions, Raynaud syndrome, capillaritis, hemorrhagic vasculitis, stable angina, retinal angiopathy, livedo reticularis, atherosclerosis, veinous thrombosis, pulmonary hypertension, secondary hypertension, myocarditis were pathogenetically associated with systemic lupus erythematosus. Other lesions were induced by co-occurring circulatory system diseases, since there were no relationship between their prevalence and the severity degree of the underlying disease (SLE). The patients with systemic lupus erythematosus were predominantly diagnosed with stage II Reynaud syndrome, and it affected mostly hand fingers. the intima-media thickness ranged from 8.0 mm to 14.9 mm. The retinal angiopathy was of the first degree of severity, livedo reticularis predominantly affected lower limbs (legs). All patients had cutaneous form of hemorrhagic vasculitis (first degree of severity), and capillaritis affected hands. The veinous thrombosis was observed at tibial and popliteal vein segments. The secondary hypertension was of first degree of severity, pulmonary hypertension was asymptomatic (first degree of severity), myocarditis was mild, and stable angina was of functional class II. The lesions that were pathogenetically associated with systemic lupus erythematosus had their own factors of prevalence: Raynaud syndrome was significantly more prevalent in women and patients aged 18 to 24, retinal angiopathy, livedo reticularis and secondary hypertension were significantly more prevalent in women and patients with the disease duration of more than 10 years. The veinous thrombosis and stable angina were significantly more prevalent in elderly patients and patients with the disease duration of more than 10 years. Myocarditis was significantly more prevalent in men; atherosclerosis and pulmonary hypertension were significantly more prevalent in elderly patients.

Eradication of hepatitits C virus in patient with cryoglobulinemic vasculitis and mutations D168E, L31V

The case of chronic hepatitis C 1b genotype with grade 3 fibrosis according to Metavir, complicated by the development of mixed type III cryoglobulinemia, cryoglobulinemic vasculitis with damage to the skin vessels of the skin (hemorrhagic vasculitis), and the liver (alternatively proliferative vasculitis) is demonstrated. The introduction of daclatasvir + asunaprevir was virologically unsuccessful: mutations D168E and L31V were detected against the background of a virological breakthrough. A repeated course of antiviral therapy with the combination of Grazoprevir + Elbasvir in combination with sofosbuvir led to a stable virologic response, partial immunological and clinical remission.

Peculiarities of hemorrhagic vasculitis coexisting with liver steatosis

Our previous study revealed that 73.9 % of patients with adult-onset hemorrhagic vasculitis (HV) - IgA vasculitis have sonographic features of liver involvement and 61.3% of patients have gallbladder pathology, whereas liver functional tests were within normal limits. This study aimed to determine the clinical characteristics of adult-onset HV coexisting with liver steatosis (LS) as a first step of nonalcoholic non-alcoholic fatty liver disease. Methods. We retrospectively reviewed the medical records of 50 adult-onset HV patients, 20 were male and 30 female with a mean age of 46.0±2.0 years. Clinical, echocardiography, electrocardiography, abdominal sonogram and routine laboratory tests data (complete blood count, liver and renal function tests, C-reactive protein (CRP), glucose, uric acid, fibrinogen, serum protein electrophoresis) were analyzed. Patients were divided into two groups in order to evaluate the impact of LS on clinical course of adult-onset HV. In the main group with LS there were 15 male and 16 female with a mean age of 49.4±2.5 years, duration of treatment was 11.5±0.7 days). The control group without LS comprised five male and 14 female (39.0±3.0 years) with treatment duration 12.3±1.2 days. For normally distributed data, means for the two groups were compared using Student's t-test; Pearson's correlation was calculated to examine the association between two continuous variables. Results. In adult-onset HV patients with LS, inflammatory responses with elevated serum CRP level and leukocytosis have been observed. High functional activity of hepatocytes with increased acute-phase protein synthesis (CRP 2.2±0.37 vs 0.71±0.47, р<0.05), uric acid (0.35±0.02 vs 0.28±0.02 mcmol/l, р<0.05), and leakage of alanine aminotransferase (0.33±0.05 vs 0.21±0.02 mmol/g/l, р<0.05) from the mitichondrias of hepatocytes into the blood occurs following a hepatocellular injury. Adult-onset HV patients with LS had a higher frequency of gallbladder disorders (OR 3.43), and significantly larger sizes of left and right ventricles, left atrial and aortic root diameters (all р<0,05).

DECREASE IN SERUM BILIRUBIN AS AN UNFAVORABLE MARKER OF CARDIOVASCULAR DISORDERS

Serum bilirubin, the end product of heme metabolism, is a routine biochemical parameter. Bilirubin is not a liver function parameter exclusively: its concentration correlates with ischemic heart disease (IHD) risk, estimated glomerular filtration rate, retinopathy or neuropathy in diabetes mellitus, atherosclerosis etc. The aim of this paper was to estimate the clinical value of bilirubin analysis according to literature data and own clinical observations in patients with IHD and acute and chronic rheumatologic diseases. Materials and methods. We conducted a literature overview in Pubmed database and domestic sources and also analyzed the standard examinaions of 515 patients: 353 patients with coronary heart disease (acute forms, coronary bypass grafting – 98; acute myocardial infarction, pharmacotherapy – 75; unstable angina pectoris – 101; stable angina pectoris – 79) and 162 rheumatologic patients (haemorrhagic vasculitis – 71; rheumatic fever – 57; chronic rheumatic heart disease with valvular defects – 34). Control group consisted of 22 patients with gastroduodenal zone diseases without helicobacter (esophagitis, gastritis, peptic ulcer). Results and discussion. It was revealed that in case of diseases with oxidative stress in their pathogenesis (acute forms of coronary heart disease, haemorrhagic vasculitis, rheumatic fever) bilirubin level was lower than in case of non-oxidative disorders (non-infectious esophagitis, gastritis, ulcer). Increase of inflammation potency was accompanied by bilirubin decrease. Correlation analysis showed that both bilirubin increase and decrease were unfavourable. Conclusions. Bilirubin concentration correlated with parameters of cytolysis, intoxication, anemia, inflammation, carbohydrate and lipid metabolism, heart structure. Bilirubin decrease associated with the increase of stenosis of coronary arteries (left, left circumflex and anterior interventricular) in a logarithmic way. Hypobilirubinemia (< 9.6 mkmol/L) significantly more often accompanied diseases with oxidative stress in pathogenesis, acute forms and more active systemic inflammation. Keywords total bilirubin, hypobilirubinemia, coronary heart disease, haemorrhagic vasculitis, rheumatic fever, chronic rheumatic heart disease.

Neutrophil to lymphocyte ratio in the diagnosis of viscero-visceral cardioneuropathy in patients with gallbladder disorders

Summary. Neutrophils to lymphocytes ratio (NLR) reflects the activity of systemic nonspecific inflammation and immune system answer. NLR can be influenced by the change of gallbladder condition. It’s a pity, but the diagnostic and prognostic opportunities of NLR are not properly used in routine clinical practice. The aim of this paper was to estimate the NLR value in the assessment of heart condition in the diagnostics of viscero-visceral cardioneuropathy in dependence of gallbladder condition in patients with cardiorheumatic disorders. We analyzed data of 506 inpatients with cardiorheumatic diseases, which were divided into groups according to gallbladder condition: intact gallbladder; sludge, polips and cholesterosis; various deformations of gallbladder body; gallbladder neck deformations and sonographic signs of past cholecystitis; cholelithiasis; anamnesis of cholecystectomy. It was established that NLR levels, revealed in this study, exceeded the levels of healthy people according to our previous studies (1.8±0.46) and literature data (1.65-1.86). NLR was dependent of gallbladder condition: maximal levels always accompanied pathological changes of the latter. On the background of active inflammatory phase of rheumatic diseases NLR was higher, than in case of stable and acute forms of ischemic heart disease. NLR correlations depended on gallbladder condition, and were the most numerous in case of intact gallbladder. NLR was associated with age, heart rate, acute phase inflammatory markers, parameters of endogenous intoxication, cytolysis and structural heart parameters (sizes of chambers, thicknesses of walls, ejection fraction). Ranking analysis of NLR of all included patients showed that its levels were maximal in case of cholecystitis in anamnesis and in patients after cholecystectomy. All other values can be clearly divided into 2 groups: intact gallbladder plus bent gallbladder body and, apart of it, other pathological conditions (sludge, signs of cholecystitis in anamnesis, cholelithiasis and cholecystectomy in anamnesis). Conclusions: NLR was the highest in case of active inflammatory process in hemorrhagic vasculitis (3.66) and rheumatic fever (3.24) than in the stable ischemic heart disease (2.47) and myocardial infarction (1.85), all р<0.05. Regardless of the disorder, NLR was dependent on the gallbladder condition and reached the maximal values in case of gallbladder changes. Taking into account the big amount of NLR correlations with clinical and laboratory parameters, and the results of instrumental diagnostics methods, we can say that NLR may be used in the diagnostics of viscero-visceral cardioneuropathy.

Сlinical and prognostic value of total fibrinogen level and its role in heart disorders on the background of different gallbladder conditions

Total fibrinogen (TF) is in the focus of attention of scientists and doctors since Hippocrates’ time. Fibrous component of thrombus was for the first revealed in the end of XVII century. Term “fibrin” was introduced in 1801, “fibrinogen” – in 1847, despite the latter was discovered in 1859. Nowadays TF is considered to be not only criterion of inflammation activity, but also a marker of cardiovascular risk. The aim of this study: estimation of the modern condition of the problem of TF analysis and usage according to literature data and own clinical results. Material and methods. We organized a literature search in PubMed database and domestic sources. Fibrinogen concentration (gravimetric method of R.A. Rutberg), level of soluble fibrin-monomer complex (SFMC) (Tehnologiya-Standart, Russia) and D-dimers (TECHNOZYM D-dimer ELISA, Austria) were estimated in 489 patients with ischemic heart disease (IHD), hemorrhagic vasculitis (HV), and rheumatic fever (RF) in patients with different gallbladder (GB) conditions. Results were analyzed with the help of variational statistics. Data was considered significant if p<0.05. Results. Fibrinogen is a hexamer glycoprotein of plasma, which acts as a key regulator of inflammation, plays an important role in hemostasis, wound healing and angiogenesis. In patients with IHD, HV and RF its amount varied from 2.20 to 8.05 g/L. The highest levels were revealed in case of unstable angina pectoris (4.23±0.16 g/L, р<0.05 in comparison with groups of HV and RF, where we observed the minimal concentration: 3.31±0.14 g/L). Conclusions. TF level was dependent on GB condition: minimal levels were observed in case of intact GB (3.67 g/L, the lowest ones – in patients with RF: 3.02±0.20 g/L), and maximal – in case of bent GB body (4.04 g/L). Correlational analysis revealed the association of TF with parameters of inflammation, lipid metabolism, liver function, left ventricle hypertrophy, which can be a sign of TF prognostic. In case of GB changes we observed also increase of SFMC and D-dimers. This fact proves the activation of thromboproduction and is a unfavourable prognostic value factor of vascular events.

С-reactive Protein and Its Role in Pathogenesis of Heart Disorders (Literature Review and Own Data)

C-reactive protein is a criterion of inflammation and a significant marker of cardiovascular risk.The objective: to estimate condition of problem of CRP usage according to the literature and own data.Matherials and methods. We held a literature review in Pubmed database and domestic sources. We also analyzed data of 75 patients with hemorrhagic vasculitis, divided into groups according to gallbladder (GB) condition. Data was processed using Statistica 6.0 software.Results. CRP was crystallized in 1947 by Maclyn McCarty, who proposed to use it for control of rheumatism course. CRP is a nonspecific acute phase protein, which is synthesized in liver during inflammation. Main function of CRP is to eliminate pathogens and endotoxins. CRP level can vary between &lt;1,0 mg/L and 1000 times bigger values. CRP is studied as a marker of infection, inflammation and tissue damage in ischemic heart disease, stroke, cognitive disorders, cardiovascular death. High level of CRP is associated with increase of caediovascular morbidity and mortality. In our patients with hemorrhagic vasculitis mean level CRP was 7,51±1,22 mg/L, which is almost twice bigger than the normal values. This can suggest a high cardiovascular risk. CRP level was maximal in cholelithiasis and cholecystectomy groups. Independently of GB condition CRP correlated with left atrium size and acute phase parameters of blood.Conclusions. According to the literature, CRP eliminates pathogens, endotoxins, necrotized cells. In patients with hemorrhagic vasculitis CRP directly correlated with inflammation parameters and left atrium size, indirectly – with anemic syndrome. CRP level was dependent of GB condition, being the highest in groups with cholelithiasis and prior cholecystectomy.

Difficult Differential Diagnosis of Hemorrhagic Vasculitis in the Practice of General Physician-family Practitioner: Own Clinical Observations and Literature Data

The article is dedicated to the problem of differential diagnosis of hemorrhagic vasculitis on the level of primary medicine. Current classification of systemic vasculitides, namely International Chapel Hill Consensus Conference of 2012 is present. Clinical features of the often form of vasculitis – Henoch-Schönlein purpura and the less common form of vasculitis of the small vessels – leukocytoclastic vasculitis (hypersensitivity vasculitis) are analyzed. Material is given basing on scientific literature data and own clinical observations of skin vasculitis.

Herpesvirus Infection in Development of Hemorrhagic Vasculitis in Children (Literature Review)

The authors conducted a systematic review of the literature published in the last fifteen years. The results of the literature review showed that the role of herpesvirus infection in the occurrence of primary vasculitis is not excluded. In the works of Kazakhstan and foreign researchers who consider that ability of viruses to cytopathic effect is important for herpesvirus infection, herpes simplex infection is not excluded as a leading factor in the development of HV. In this regard, the study of the etiology of HV will help to reach a new therapeutic level of the disease. Consequently, the problem of HV is of scientific interest and practical importance.