[Signs of hypercoagulation and activation of fibrinolysis in patients with Wegeners's granulomatosis and Schoenlein-Genoch's purpura].

The patients with Wegener's granulomatosis and Schoenlein-Genoch's purpura included in this study were examined in the E.A.M. Tareev Clinic during 2006-2008. They showed signs of hypercoagulation and activation of fibrinolysis, viz. enhanced SFMC, D-dimer and antiphospholipid antibody levels, platelet aggregation. Hypercoagulation correlated with the activity of both Wegeners's granulematosis and Schoenlein-Genoch's purpura.

[Pulmonary lesions in Wegener's granulomatosis].

Pulmonary lesions are the commonest and prognostically unfavourable visceral pathology in Wegener's granulomatosis. The author discusses clinically and reongenologically detectable lesions associated with this systemic vasculitis with special reference to its most severe forms (alveolar bleeding and infectious complications frequently caused by active immunosuppressive therapy). Diagnostic (including early) and modem therapeutic modalities of Wegener's granulomatosis are considered.

[Experience with rituximab treatment (monoclonal antibodies to B-lymphocyte CD20 receptors) of Wegener's granulomatosis patients with renal impairment].

AIM: To assess efficacy of rituximab in patients suffering from Wegener's granulematosis (WG) with renal disease. MATERIAL AND METHODS: We analysed clinical laboratory data from 9 WG patients with renal disease treated with rituximab in E.M. Tareev clinic. RESULTS: The treatment led to a complete remission in 7 (77.8%) patients, a partial remission--in 2 (22.2%) patients. Vasculitis activity regressed including renal damage (a decrease of 24 h proteinurea, enhanced glomerular filtration rate). A prednisolone dose decreased from 38.3 to 10 mg/day (to month 6) and to 7.5 mg/day (to month 12). Non-selective cytostatic drugs were discontinued in all the patients. Recurrences occurred in 3 (33.3%) patients, one with lethal outcome after 6 months of rituximab administration, in two cases one more course of rituximab produced another complete remission. Serious side effects were absent. CONCLUSION: Rituximab demonstrated high efficacy in the treatment of WG with renal affection resistant to standard therapy, in multiple side effects of standard immunosuppressive therapy and recurrent course. Rituximab help overcome steroid dependence even at late stages of the disease.

[Severe Wegener's granulomatosis successfully treated with rituximab].

The paper discusses the problems of the diagnosis and treatment of Wegener's granulomatosis, the most common anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. It describes a clinical case in which the administration of rituximab could achieve remission of severe Wegener's granulomatosis in a young man having numerous adverse reactions associated with the long-term use of high- and average-dose glucocorticoids and nonselective immunosuppressive agents. A place of rituximab in current drug therapy for Wegener's granulomatosis is shown.

[Takayasu syndrome in Horton's disease].

Problems of diagnosis (including early diagnoses) and treatment of giant cell arteritis the commonest form of vasculitis in subjects above 50 years. An aged woman presenting with rheumatic polymyalgia is described in whom cell arteritis was diagnosed by positron emission tomography. Etiology of this disease and it medicamentous treatment are discussed with reference to the patients' age and concomitant pathologies.

[Myocarditis and fibroplastic endocarditis in Wegener's granulomatosis].

The paper describes an autopsy case of Wegener's granulomatosis with a rare variant of a cardiac lesion--the concurrence of aortic valvular disease (aortic regurgitation), fibroplastic endocarditis, and complete transverse blockade, followed by pacemaker implantation. The direct cause of death in the female patient was multiple organ dysfunction: progressive chronic heart failure, adult respiratory distress syndrome, acute hepatorenal failure, and pancreonecrosis. The data available in the Russian and foreign literature on investigations dealing with cardiac pathology in patients with Wegener's granulomatosis are analyzed.

[Churg-Strauss syndrome in patients with cardiac problems].

Clinical observations of patients with Churg-Strauss syndrome and cardiac disorders are reported. In one case, both myocardium and endocardium were affected. Severe cardiac insufficiency was the immediate cause of death in one patient. Another observation revealed a benign variant of the disease associated with exudative pericarditis. A review of domestic and foreign literature concerning cardiac pathology in patients with Churg-Strauss syndrome is presented.

[Cardiac lesion in the Churg-Strauss syndrome].

The Churg-Strauss syndrome is a systemic vasculitis, the manifestations of which are asthma, eosinophilia, pulmonary infiltrates, poly- and mononeuropathy, polyserositis. Along with nodular polyarteritis and nonspecific aortoarteritis, the Churg-Strauss syndrome belongs to a group of systemic vasculitis, in the clinical picture of which cardiac lesion is recognized as one of the leading visceral manifestations and may be a common cause of fatal outcomes. In the Churg-Strauss syndrome, cardiac pathology may be associated with the involvement of the endocardium, myocardium, and pericardium. The paper describes a case showing the poor course of the disease in a young female patient in whom the heart is involved in a pathological process with the development of severe heart failure, resulting in death. There is a rare concomitance of diffuse myocardial damage, coronary lesion, and valvular pathology - eosinophilic endocarditis. The diagnosis has been verified on the basis of the data of clinical and additional studies and the results of microscopic studies. The data available in the Russian and foreign literature on cardiac pathology in patients with the Churg-Strauss syndrome are analyzed.

[Clinical significance of echocardiography in patients with systemic vasculitis].

The paper reports results of echocardiographic studies of patients with systemic vasculitis treated at E.M. Tareev Clinic during 1991-2006. The major findings include valve pathology, disturbed regional contractility, exudative pericarditis, systolic and diastolic dysfunction. Most patients examined in 2005-2006 turned out to have enhanced left ventricular systolodiastolic (Tei) index.

[Aortal regurgitation and atrioventricular block III in Wegener's granulomatosis].

The article presents a clinical observation of a female patient suffering from Wegener's granulomatosis with a rare variant of cardiac involvement--a combination of an aortal valvular disease (aortal regurgitation) and total atrioventricular blockade--who later underwent pacemaker implantation. The direct cause of the lethal outcome in this patient was destructive pancreatitis. Data from Russian and foreign literature on cardiac pathology in patients with Wegener's granulomatosis are analyzed.

[The peculiarities of renal lesion in nodular polyarteritis].

Analysis of the medical records of 122 patients with nodular polyarteritis showed that the most frequent visceral manifestations were renal (93.4%), cardial (72%), and gastrointestinal (57.4%) ones. Central nervous system (CNS), pulmonary, and peripheral arterial lesions were less frequent (36.8%, 17.2%, and 6.6%, respectively). Renal lesions were manifested by arterial hypertension (AH) in 107 patients, urinary syndrome in 97, nephrotic syndrome in 7, and rapidly progressing glomerulonephritis in 5 patients. Monofactor analysis revealed the following predictors of poor prognosis: malignant AH, nephrotic proteinuria, male sex, body mass reduction, intestinal ulcers, gastrointestinal hemorrhage, CNS lesion, heart failure, and pulmonary lesion. The relative risk of lethal outcome was the highest in nephrotic proteinuria (3.5) and malignant AH (2.9). In 56% of cases death was caused by cardiovascular complications, in 18%--chronic renal failure, in 11%--abdominal complications.

[Cardiac lesion in systemic vasculites: manifestations, diagnosis, and treatment].

The article contains analysis of Russian and foreign literature on cardiac lesions in patients with system vasculites (SV). The frequency of cardiovascular involvement in various SV forms was evaluated. These forms include non-specific aortoarteritis, giant-cell arteritis, nodular polyarteritis, Kawasaki disease, Wegener's granulomatosis, eosinophilic vasculitiis, and granulomatosis. The authors consider the variants of coronary arterial, endocardial, myocardial, and pericardial involvement, as well as the role of arterial hypertension and the clinical features of different SV with cardiac lesions. Modern diagnostic techniques (echoCG, perfusion myocardial scintigraphy, multispiral computed tomography, and coronaroangiography) are covered.

[Vascular purpura in patients with chronic diffuse hepatic diseases].

The purpose of the study was to evaluate clinical significance of vascular purpura (VP) in diagnostics and establishing prognosis of chronic diffuse hepatic diseases (CDHD). The subjects were 660 patients with CDHD of viral and non-viral origin, who were under long hospital observation. 72 CDHD patients had cutaneous vasculitis. 11% of CDHD patients developed VP, which corresponded to the frequency of other cutaneous manifestations--minor hepatic signs (palmar erythema, spider-like blood vessels in the skin, xanthelasmas etc.) There was insignificant difference in the frequency of VP between the patients with type B and C hepatitis (9.6%), on the one part, and patients with non-viral hepatic diseases--primary sclerosing cholangitis, autoimmune hepatitis, and Wilson-Konovalov disease (14.2%). According to morphological data, 84.3% of CDHD patients with VP developed the latter or had a VP relapse at the cirrhotic stage (50%) or at the stage of forming hepatic cirrhosis (34.3%). Renal involvement, which was significantly more frequent in CDHD patients with VP vs. patients without VP (32% and 11.4%, respectively), may deteriorate the prognosis. The authors conclude that cutaneous vasculitits is a universal sign of viral and non-viral CDHD, which should be taken into consideration when diagnosing and establishing prognosis of these diseases. The fact that VP is revealed mainly at the cirrhotic stage suggests that an important role in its development is played by dysfunction of the liver, one of the main organs that clear antigen and immune complexes (including those of viral origin), appearing via the portal system or circulating in blood.

[Vascular purpura associated with chronic diffusive hepatic pathologies]. / Sosudistaia purpura, assotsiirovannaia s khronicheskimi diffuznymi zabolevaniiami pecheni.

The study results related with the rate of vascular purpura in 660 patients with different-type chronic diffusive hepatic pathologies both of the viral and other natures are described in the paper. The main regularities characterizing the phenomenon (spread and possible cause of purpura development) are defined. According to an analysis of actual materials, dermal vasculitis is not an exclusive feature of virus-associated hepatitis or liver cirrhosis. Vascular purpura can be regarded, on the basis of the obtained data, as a universal sign typical of any liver pathologies.

[The parietal mucin microbial population in different sections of the human gastrointestinal tract]. / Mikrobnye soobshchestvo pristenochnogo mutsina razlichnykh otdelov zheludochno-kishechnogo trakta cheloveka.

Microbiological examinations of the human-colon parietal mucin were made in different sections of the human gastrointestinal tract (GIT). Biopsy samples of the human colon mucus tunic were used as the research material. Convincing data were found by research on the importance of the anatomic-and-morphological factor in shaping-up of microbiocenosis of the colon-wall parietal mucin. It was established that, depending on a GIT section, not only the quantitative and qualitative composition of normal microflora but also the nature of inter-microbe interactions are subject to changes. In particular, biopsy materials of the small-intestine parietal mucin are much different from that of the colon. The colon section from the ascending colon to the sigmoid colon has, with some exceptions, an identical microbial composition. At the same time, the rectum is significantly different from other colon sections. The results are suggestive of the below cluster-type pattern of parietal biological material: microorganisms are clustered as small domains with certain specific and quantitative compositions. It was established that, although feces and parietal mucin have a similar species composition of microorganisms, still, there is a number of essential differences between them in as far as the frequency ratio and the microorganism concentration are concerned, which signifies a certain degree of isolation of the above biopsy materials. Finally, a certain isolation degree of the feces biopsy materials and of the parietal ones was established.

[Clinical and prognostic value of lesions in the gastrointestinal tract in systemic vascular purpura]. / Klinicheskoe i prognosticheskoe znachesnie porazheniia zheludochno-kishechnogo trakta pri sistemnykh sosudistykh pupurakh.

AIM: To evaluate clinical peculiarities and prognostic value of abdominal syndrome in adult patients with systemic vascular purpura (SVP). MATERIAL AND METHODS: Incidence, clinical picture, complications of abdominal syndrome, its relations with principal extraintestinal manifestations of SVP were studied in 125 SVP patients. Absorption and barrier functions of the small intestine, their correlations with clinical activity of the disease were investigated in 32 SVP patients with no clinical signs of intestinal affection. Computer-assisted morphometry of duodenal mucosa biopsies was conducted and the results were compared to those of intestinal permiability and clinical activity of SVP. RESULTS: Negative correlation was found between the occurrence of abdominal syndrome and the patients' age. The gastrointestinal tract suffered more frequently in men. 21% patients had abdominal complications, association of abdominal syndrome with higher frequency of glomerulonephritis. In the absence of abdominal symptoms, the barrier function of the small intestine for protein macromolecules was impaired. This correlated with exacerbations of skin purpura. Increased intestinal permeability and activity of skin purpura correlated with severity of inflammation in the duodenal mucosa. CONCLUSION: Abdominal syndrome in SVP is an unfavourable prognostic factor indicating possible severe complications and high risk of glomerulonephritis. Subclinical intestinal involvement presenting as barrier dysfunction may contribute to aggravation of skin vasculitis.

[Abdominal emergencies in systemic vasculitis]. / Abdominal'nye katastrofy pri sistemnykh vaskulitakh.

Abdominal lesions are characterized in a patient with acute vascular purpura and a patient with polyarteritis. The former patient developed obturation ileus of the small intestine, the latter--necrosis and multiple intestinal and gall bladder perforation with subsequent peritonitis. In both cases the abdominal syndrome was manifestation of the basic disease. Surgical treatment saved life of the patients. Stable remission was achieved after long-term medication.