[System of microcirculation, markers of vascular wall damage and systematicity of the process in rheumatic diseases].

The work was aimed at studying the interrelationship of the microcirculation system and the parameters of endothelial activation with markers of inflammatory process activity in rheumatic diseases (RD). We carried out a comprehensive examination of a total of 330 patients presenting with systemic diseases of connective tissue (SDCT), rheumatoid arthritis (RA) and systemic vasculitis (SV). Studying microcirculation included impregnation of filmy preparations according to the V.V. Kupriyanov technique and biomicroscopy of the conjunctiva of the eyeball. We also determined markers of endothelial activation and lesion of vascular wall, indices of activity of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and vasculitis clinical activity index (VCAI), common carotid artery intima-media thickness (CCA-IMT), biopsy materials of the musculocutaneous flap, of the operational and autopsy materials. Determining the indices of microcirculation showed that first of all the process involves postcapillaries and venules which are dilated, becoming tortuous, with the formation of microaneurysms and stellate venules. Capillaries, postcapillaries and venules were found to contain parietally located small-grained conglomerates of blood platelets and thrombocytic masses plugging up the lumens of microvessels. Intravascular alterations were characterized by the presence of erythrocyte aggregates, stases, microthrombovasculitis, «sludge¼ phenomenon, and a decrease in capillary blood flow. Extravascular changes included perivascular haemorrhages. In arterioles and precapillaries the inflammatory process manifested itself by swelling, dystrophy and desquamation of endothelial cells, plasmatic impregnation of the walls, luminal thrombosis followed by the development of severe sclerosis and glialinosis. The morphological study showed the presence of destructive alterations in the vascular wall, fibrinoid necrosis, and infiltration-proliferative cellular reaction. The most pronounced changes in the autoimmune inflammation markers had place in RA and systemic lupus erythematosus (SLE). We revealed increased indices of inflammatory process activity such as interleukin-8, C-reactive protein (CRP). We also revealed the signs of endothelial dysfunction, manifesting itself as a statistically significant (p<0.01) increase in concentrations of the soluble vascular cell adhesion molecule (sVCAM-1), von Willebrand factor antigen (VWFA), the number of desquamated endotheliocytes (DE). Also observed was a clear-cut dependence of the level of endothelial activation markers from the degree of the processes activity. We revealed a positive correlation between the level of CRP, IgG RF, the level of sVCAM-1 and the number of DE. The levels of interleukin-8, sVCAM-1 and VWFA were elevated in patients with RD. Increased activity of the disease was accompanied by impairments at the level of the microcirculatory bed, an increase in the concentration of inflammation markers and indices of endothelial dysfunction.

[Systemic vasculitides: diagnostic stages].

AIM: To present systemic vasculitis (SV) diagnostic stages. SUBJECTS AND METHODS: Immunological and hemostatic parameters were determined, vascular scanning, histological and immunomorphological studies were performed in 360 patients. RESULTS: The main diagnostic searching stages were presented, which could reveal the key clinical signs of vasculitis and systemacy of the process, differentiate primary and secondary vasculitides, conduct clinical and instrumental studies, detect specific markers of vascular wall injury, perform a morphological study of biopsy specimens, identify the major pathogenic components of vascular bed lesion, define the possible etiology and form of vasculitis, and make a nosological diagnosis. CONCLUSION: The proposed diagnostic steps will be able to specify the nosological form of SV and the activity of the process and to define approaches to pathogenetic therapy.

[Myocardial remodeling in systemic lupus erythematosus and systemic scleroderma].

AIM: To define the significance of myocardial remodeling and its association with the activity of an inflammatory process in systemic lupus erythematosus (SLE) and scleroderma systematica (SDS). SUBJECTS AND METHODS: One hundred and sixty-seven patients, including 102 with SLE and 65 with SDS, were examined. Intracardiac hemodynamic parameters were estimated by ultrasonography on an Acuson 128 XP/10 computed sonography system, by using 3.5-MHz frequency ultrasound transducers in accordance with the standard procedure recommended by the American Echocardiography Association (1987). The Systemic Lupus Activity Measurement (SLAM) and European Consensus Lupus Activity Measurement (ECLAM) scales were used to estimate the activity of SLE and its stages in SDS. RESULTS: In patients with rheumatic diseases (RD), the spectrum of heart changes varied from latent diastolic dysfunction (DD) to the development of myocardial remodeling with signs of chronic heart failure. Examination of the types of myocardial remodeling in the patients with RD revealed all 4 geometric cardiac model types. There was a normal cardiac model in 59.2%, eccentric left ventricular (LV) hypertrophy in 18.4%, concentric hypertrophy in 19.5%, and concentric remodeling in 2.9%. In SLE, the disease activity determined the magnitude of LV hypertrophic processes (r = 0.57; p = 0.005) and DD (r = -0.43; p = 0.023). In the patients with SDS, the high activity was also associated with LV hypertrophy (r = 0.52; p = 0.015), but DD was primarily determined from the duration of disease (r = -0.44; p = 0.024). The patients with RD had LV DD no matter whether hypertension was present or absent. CONCLUSION: There is evidence for myocardial remodeling and intracardiac hemodynamic disorders in SLE and SDS and their association with the activity of the process.

[Arterial hypertension in patients with systemic connective tissue diseases and hemorrhagic vasculitis].

AIM: To study the specific features of 24-hour blood pressure (BP) profile and its association with plasma renin activity in patients with systemic connective tissue diseases and hemorrhagic vasculitis (HV). SUBJECTS AND METHODS: One hundred patients aged 22 to 58 years, including 45 patients with systemic lupus erythematosus (SLE), 25 with scleroderma systematica (SDS), and 30 with HV, were examined. A control group included 30 healthy individuals. 24-hour BP profile, renal function, and plasma renin activity were studied. RESULTS: Arterial hypertension (AH) was revealed in 53% of patients. AH occurred in 62% of patients with SLE, in 40% of those with SDS, and in 50% of those with HV. In patients with systemic connective tissue diseases and HV, the 24-hour BP profile was characterized with increases in the mean values and indices of pressure load and with a predominance of subjects with inadequate decreases in nocturnal BP (non-dippers), and with the higher values of its variability in the presence of elevated plasma renin concentrations in patients with SLE. In all the patients, the elevation of BP and its circadian dynamics depended on the renal functional status that correlated with the activity of a systemic inflammatory process. CONCLUSION: The patients with systemic connective tissue diseases and HV were found to have prognostically poor types of 24-hour BP profile (night-peaker, non-dipper), the magnitude of which elevation depended on renal function and plasma renin activity.

[Neurohumoral regulation of blood pressure in rheumatic patients].

AIM: To study characteristics of neurohumoral regulation of blood pressure (BP) in patients with systemic diseases of the connective tissue and hemorrhagic vasculitis (HV). MATERIAL AND METHODS: The trial included 45 patients with systemic lupus erythematosus (SLE), 25 patients with scleroderma systematica (SS), 30 HV patients and 30 healthy controls. The following parameters were estimated: activity of plasmic renin, aldosteron concentration in plasma, catecholamines (noradrenaline and adrenalin), serum level of endotheline-1, number of desquamated endotheliocytes by J. Hladovec (1978) with use of Goryaev's camera. A BP 24-h profile was obtained by the standard method with the device Kardiotekhnika 4000 AD. Renal function was assessed by blood creatinine (Reberg's test). RESULTS: Contribution of different factors to pathogenesis of arterial hypertension (AH) in rheumatic conditions was different. SLE activity enhancement was associated with renal dysfunction and growth of plasmic renin leading to AH resultant from activation of the renin-angiotensin-aldosteron system (RAAS), sympathico-adrenal system (SAS) and suspended by endothelial dysfunction. AH in SS patients presented with SAS activation, endothelial dysfunction and moderate pathology of the kidneys. HV activation provoked renal and endothelial dysfunction, SAS activation leading to development of AH. CONCLUSION: In rheumatic diseases AH develops with activation of SAS, RAAS, endothelial and renal dysfunction.

[The diastolic dysfunction of the left ventricle in patients with systemic lupus erythematosus and system scleroderma].

The subjects of the study were 22 patients with systemic lupus erythematosus (SLE) and 18 patients with system scleroderma (SS). The mean age of the subjects was 36.3 +/- 2.4 years, the onset of the disease had taken place 5 to 10 years ago. The control group consisted of 20 practically healthy individuals with no complaints, clinical signs or instrumental data suggesting cardiovascular pathology. In order to evaluate the character of left ventricular (LV) diastolic filling, all the patients underwent transthoracal Doppler analysis with measurement of transmitral flow in four-chamber heart position using apical approach with the control volume at the level of the ends of mitral valvular cusps (computed sonography system ACUSON 128 XP/10). The study found no significant difference between SLE and SS patients in such parameters as LV myocardial mass and LV mass index. All the patients with rheumatic diseases, with or without arterial hypertension (AH), had diastolic dysfunction, which was manifested by increase of atrial systolic contribution into LV filling, prolongation of blood flow slowdown time in the stage of its early filling, and prolongation of LV isometric relaxation time; heart diastolic disorder was accompanied by significant increase of end diastolic pressure in LV cavity. It should be noted that the most prominent changes were found in rheumatic patients with AH, which must be caused by the hypertrophy and remodeling of the myocardium. Myocardial hypertrophy was associated with substantial changes in the ventricular septum, which consisted in its hypokinesia, associated with impairment of myocardial contractility (ejection fraction of 48.3 +/- 3.5%).

[Pulmonary pathology in patients with systemic lupus erythematosus].

AIM: To characterize pulmonary lesion in systemic lupus erythematosus (SLE) on the basis of clinical device and biochemical examination regarding features of the disease onset and development. MATERIAL AND METHODS: The study included 60 SLE patients. Mean age 41.1 +/- 1.32 years. Mean SLE duration 12.42 +/- 1.06 years. Activity according to the SLEDAI and ECLAM indices--16.23 +/- 0.93 and 3.09 +/- 0.18 scores, respectively. The comparison groups: 30 patients with bronchial asthma (BA), 15--with chronic bronchitis (CB), 15--with chronic obstructive bronchitis (COB), 30 healthy donors. The following parameters were studied: spirometric, bodyplethismographic evidence, diffuse ability of the lungs (DAL), plasm concentrations of adrenaline, noradrenaline, dopamine, serotonine, histamine, hemodynamics, anxiety, depression, social adaptation (quality of life) and vegetative dysfunctions. Statistics were obtained with BIOSTATISTIKA program. RESULTS: DAL depends on duration of SLE, severity of lung hypertension (LH), severity of anemia. LH in SLE deteriorated vegetative disorders and social adaptation. Lowering of plasm dopamine concentration was accompanied with LH, formation of vegetative dysfunction and worse social adaptation. CONCLUSION: Affection of the lungs in SLE patients runs without evident clinical symptoms. Initial signs of lung affection manifest with low DAL, LH, moderate restrictive, obstructive and mixed disorders of external respiration function.

[Systemic vasculitis as an interdisciplinary problem]. / Sistemnye vaskulity kak mezhdistsiplinarnaia problema.

Systemic vasculitis (SV) is characterized by generalized vascular bed lesion involving vessels of different sizes into a pathological process. The paper presents the results of a follow-up of 500 patients with different forms of SV, by making studies of immunity and the hemostatic system, angioscanning, Doppler ultrasound study of vessels, electrophysiological studies (rheoencephalography, encephalography), computed and magnetic resonance imaging of the brain, and visceral ultrasonography. A variety of clinical symptoms and involvement of different organs determine the interest of physicians of different specialties in the diagnosis and treatment of SV. The involvement of the nervous system in the process occurs in all forms of vasculitis, by afflicting the central, peripheral, and autonomic nervous systems with the development of regulatory and functional disorders. Lesions of the visual organ are typical of nonspecific aortoarteritis (Takayasu's disease), Wegener's granulomatosis, giant-cell arteritis. Recurrent uveitis is characterized in Behcet's syndrome. Cutaneous manifestations are included into the classification criteria of nodal polyartheritis, hemorrhagic vasculitis, and Kawasaki's disease. ENT and oral involvement are observed in Wegener's granulomatosis.

[Neurovascular syndrome in some rheumatic diseases]. / Neirososudistyi sindrom pri nekotorykh revmaticheskikh zabolevaniiakh.

AIM: Assessment of neuromotor system and suprasegmentary vegetative structures in patients with systemic vasculitis (SV), systemic lupus erythematosus (SLE), scleroderma systematica (SS) for determination of the vegetative profile and pathogenetic links underlying vegetative disorders. MATERIALS AND METHODS: The examination of 125 rheumatic patients included clinical, laboratory, instrumental, neurological, neuropsychic, electroneuromyographic, vegetologic, pathomorphologic and biochemical investigations. RESULTS: Rheumatic patients presented affections of the peripheral and central venous systems, vegetative dysfunction, disturbed higher nervous activity manifesting as polyneuropathy, mononeuropathy, pyramid syndrome, dystonia, hypothalamic syndrome, reduced adaptive ability, low tonicity of the sympathetic nervous system, terminal branches of the motor axons, etc. CONCLUSION: Therapy of nervous disorders in rheumatic patients comprises treatment of cerebral circulation (vascular, nootropic drugs, cerebrolysin), asthenic, neurotic and vegetative disorders (sedative and vegetotropic drugs, etimisol, adaptogens), abnormalities of peripheral nervous system (amiridin, anticholinesterase preparations, vitamins B and others). Follow-up and correction of the on-going therapy contribute to a decrease in the number of invalidating and lethal neurological complications.