Anti‑NMDA encephalitis is a rare autoimmune disease of the central nervous system caused by the synthesis of autoantibodies to the NR1/NR2 subunits of the NMDA receptor, characterized by the development of acute mental, cognitive, motor, autonomic disorders, epileptic syndrome and central hypoventilation.The article presents a three‑year observation of patient 34 years old with anti‑NMDA ncephalitis associated with late‑ stage ovarian teratoma, accompanied by an increase titer of antibodies to NMDA receptors in serum to 1:640.Based on a detailed analysis of clinical, neurological, neuropsychological (MMSE, MoСA, FAB, 10 words test A.R. Luria) and laboratory‑instrumental characteristics of the disease (titer anti‑NMDA, level of IgG, IgM, IgA, lymphocyte subpopulations, EEG, MRI of the brain, pelvis) suggested a combination scheme of first and second line therapy. The sequential use of two cycles of medium‑volume membrane plasmapheresis (25–30 % of the circulating plasma volume, No. 5 + 5) was carried out in combination with pulse therapy with methylprednisolone 1.0 (No. 4 + 3) and cyclophasphamide 1.0 (No. 2 + 1) on background of persistent ovarian teratoma. Symptom regression was achieved by the end of the first cycle, and full recovery to the initial level of cognitive functions occurred after the second cycle, while maintaining the anti‑NMDA antibody titer to 1:160. After removal of ovarian teratoma, the level of anti‑NMDA decreased in a month to 1:40, and after 7 months it reached normal values (<1:10) against the background of basic pill therapy with methotrexate 12.5 mg/week.Thus, a rational combination and sequence of first and second line therapy and therapeutic apheresis, taking into account the pathogenetic features of each phase of the disease, can quickly achieve complete stable remission in patient with anti‑NMDA encephalitis.

Programmed immunoadsorption (IA) with regeneration of adsoption columns is a promising and safe technique to treat lupus nephritis (LN) in case of ineffective immunosuppressive therapy and its severe adverse reactions. This technique makes it possible to control disease activity, to maintain kidney function, and to ensure a normal quality of life. Due to the reusability of IA columns, it is possible to remove any required amount of IgG and to reduce the cost of an extracorporeal procedure.The paper describes a clinical case of 3-year prolonged IA in a female patient with systemic lupus erythematosus (SLE) and LN with the insufficient efficacy of drug therapy and related complications. Seventy IA sessions were performed during a follow-up period. Combined treatment with glucocorticoids, cytotoxic drugs, and IA resulted in improved clinical and laboratory parameters, lower SLE activity according to SELENA-SLEDAI scores, and better quality of life according to the SF-36 scale. No adverse reactions were recorded during IA sessions.

Programmed immunoadsorption (IA) with regeneration of adsoption columns is a promising and safe technique to treat lupus nephritis (LN) in case of ineffective immunosuppressive therapy and its severe adverse reactions. This technique makes it possible to control disease activity, to maintain kidney function, and to ensure a normal quality of life. Due to the reusability of IA columns, it is possible to remove any required amount of IgG and to reduce the cost of an extracorporeal procedure. The paper describes a clinical case of 3-year prolonged IA in a female patient with systemic lupus erythematosus (SLE) and LN with the insufficient efficacy of drug therapy and related complications. Seventy IA sessions were performed during a follow-up period. Combined treatment with glucocorticoids, cytotoxic drugs, and IA resulted in improved clinical and laboratory parameters, lower SLE activity according to SELENA-SLEDAI scores, and better quality of life according to the SF-36 scale. No adverse reactions were recorded during IA sessions.

The determination of antinuclear antibodies is included in the criteria for the diagnosis of connective tissue diseases, differential diagnosis and determines the prognosis and treatment tactics of the patient. The existing strategy for laboratory diagnosis of connective tissue diseases involves screening (ANA Hep-2 cell + ENA / ELISA), followed by the selection of refinement tests (ELISA, immunoblot). The assessment of the actually used algorithms, however, revealed that every third patient was assigned almost all available tests simultaneously to confirm a connective tissue disease. Rational screening was performed in 1/3 of the cases. Low frequency of application of ANA immunoblots significantly reduced the effectiveness of diagnosis. It is advisable to provide more information about the primary screening methods for connective tissue diseases of practicing doctors, namely therapists and general practitioners, who act as the primary diagnostic link for this category of patients.

Abstract. Detection of circulating autoantibodies provides valuable diagnostic criteria for autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). In this study, a panel of autoantibodies (ANA, ASMA, AMA) and ANCA was determined by indirect immunofluorescence assay in 191 patients, including those with AIH type 1 (n = 42), with PBC (n = 39), with overlapping AIH-1/PBC syndrome (n = 20), and 100 patients with viral hepatitis С (HCV). Autoantibodies against PDC/AMA-M2, M2-3E, PML, Sp100, gp210, SSA-Ro52, SLA/LP, LC-1 were detected by means of immune blotting in 53 patients with autoimmune liver diseases (AIH, 19; PBC, 23; AIH/PBC, 11). ANA were detected in 88.1% of AIH-1 patients, 89.7% of PBC cases, and 100% of combined AIH-1/PBC syndrome, with highest ANA titers observed in the latter group. In patients with HCV, ANA were detected in 20%, and ASMA in 2% of the cases.ASMA and ANCA were found in monovariant AIH only (61.9% for ASMA, р < 0.001), and 35.7% for ANCA (р < 0.01). Anti-SLA/LP were detected in 15% of AIH patients, mostly with negative ANA and ASMA. PDC/AMA-M2 were identified in all patients with PBC and AIH-1/PBC, whereas AMA was detectable in 84.6% of PBC patients. Anti-M2-3E antibodies were found only in patients with PBC including those with an overlap syndrome. Occurence of detectable antibodies to gp210, Sp100, PML and SSA-Ro52 was similar in all groups. In our study, we have not confirmed a view that anti-gp210 and anti-Sp100 antibodies are highly specific for primary biliary cirrhosis.

Methods: We conducted a retrospective study including patients admitted in our department for severe acute colitis of inflammatory bowel disease (IBD) between 2000 and 2012. Diagnosis of severe acute colitis was made on the basis of Truelove and Witts criteria. Response to cyclosporine therapy was assessed clinically and biologically after 3 and 7 days of treatment and was defined as a Lichtiger score less than 10/20. Statistical analysis was performed with SPSS software version 21.0. Results: One hundred and sixteen patients were referred for severe acute colitis. Cyclosporine was administered in 40 patients after failure of intravenous steroid therapy. There were 18 males and 22 females with a mean age of 31.4 years old (17 55). There were 12 Crohn’s disease cases and 28 of ulcerative colitis cases. Response to cyclosporine was obtained in 20 patients (50%). In univariate analysis, presence of mucosal bridges during initial colonoscopy (p = 0.033), absence of anterior maintenance therapy (p = 0.021) and a decrease of platelet count >65,000/mm3 after 7 days of treatment (p = 0.013) were associated to response to cyclosporine. In multivariate analysis, presence of mucosal bridges during initial colonoscopy was independently associated with response to cyclosporine (p = 0.0001). Conclusions: Cyclosporine is effective in preventing surgery in patients with severe steroid resistant colitis. Response rate of 50% encourages selecting candidates to this treatment with similar benefit in case of Crohn’s disease or ulcerative colitis.

Antibodies to the cytoplasm of neutrophils (p-ANCA) are detectable in 67% of patients with ulcerative colitis (UC). We have revealed typical clinical features of the patients with diagnostic p-ANCA titer, i.e., longer disease duration, prolonged fevers, and increased stool frequency. Moreover, thrombocytosis, leukocytosis and hypoalbuminemia are more common in this group. By endoscopic examination, mucosal ulcers of the colon are significantly more frequent in this group of patients. We have also noted higher rates of severe and relapsing cases among p-ANCA-positive patients. The data obtained allow us to suggest that the diagnostic titers of p-ANCA are predictive for unfavorable prognosis in UC.

The study was carried out to determine clinical and immunologic predictors of unfavorable variant of course of ulcer colitis. The sample included 89 patients (48 females 53.9% and 41 males 46.1°%) with ulcer colitis established on the basis of clinical, endoscopic and morphologic data. The age of patients was 18-79 years and mean age 42.49±1.61 years. The patients were divided on two groups depending on clinical course of disease: group 1 with favorable course and group 2 with unfavorable course. The group 2 included patients with frequently relapsing form of disease, patients with hormone-depended/hormone-resistant form of disease and patients with severe exacerbation ща ulcer colitis at the moment of examination. The groups were compared by gender and age. All patients underwent medical history and complaints acquisition and total clinical examination. The clinical and biochemical analysis of blood was made too. The severity of disease was established using the calculation of Trulove-Witts indicator. The anti-neutrophil cytoplasmic antibodies of classes IgG and IgA were analyzed using indirect immunofluorescence (Euroimmun AG, Germany). The diagnostic anti-neutrophil cytoplasmic antibodies titer was established in 58 out of 87 of examined patients (66.6%). The antineutrophil cytoplasmic antibodies of class IgG was revealed in 42 patients and anti-neutrophil cytoplasmic antibodies of class IgA in 27 patients. The combination of both classes of anti-neutrophil cytoplasmic antibodies was established in 11 examined patients. In the group of favorable course of disease the diagnostic titer of anti-neutrophil cytoplasmic antibodies was revealed in 20 patients (51%). At the same time, in the subgroups with frequently relapsing, hormone-depended/hormone-resistant and severe forms of disease these antibodies were revealed with rate of 76, 77 and 86.3% correspondingly. Hence, the anti-neutrophil cytoplasmic antibodies can be used both in diagnostic of ulcer colitis and in prognosis of course of disease.

The study was carried out to determine clinical and immunologic predictors of unfavorable variant of course of ulcer colitis. The sample included 89 patients (48 females--53.9% and 41 males--46.1%) with ulcer colitis established on the basis of clinical, endoscopic and morphologic data. The age of patients was 18-79 years and mean age--42.49 +/- 1.61 years. The patients were divided on two groups depending on clinical course of disease: group 1 with favorable course and group 2 with unfavorable course. The group 2 included patients with frequently relapsing form of disease, patients with hormone-depended/hormone-resistant form of disease and patients with severe exacerbation ua ulcer colitis at the moment of examination. The groups were compared by gender and age. All patients underwent medical history and complaints acquisition and total clinical examination. The clinical and biochemical analysis of blood was made too. The severity of disease was established using the calculation of Trulove- Witts indicator The anti-neutrophil cytoplasmic antibodies of classes IgG and IgA were analyzed using indirect immunofluorescence (Euroimmun AG, Germany). The diagnostic anti-neutrophil cytoplasmic antibodies titer was established in 58 out of 87 of examined patients (66.6%). The antineutrophil cytoplasmic antibodies of class IgG was revealed in 42 patients and anti-neutrophil cytoplasmic antibodies of class IgA in 27 patients. The combination of both classes of anti-neutrophil cytoplasmic antibodies was established in 11 examined patients. In the group of favorable course of disease the diagnostic titer of anti-neutrophil cytoplasmic antibodies was revealed in 20 patients (51%). At the same time, in the subgroups with frequently relapsing, hormone-depended/hormone-resistant and severe forms of disease these antibodies were revealed with rate of 76, 77 and 86.3% correspondingly. Hence, the anti-neutrophil cytoplasmic antibodies can be used both in diagnostic of ulcer colitis and in prognosis of course of disease.

Rheumatoid arthritis (RA) is a classic autoimmune disease associated with the production of wide range of autoantibodies, and their detection has diagnostic and prognostic implication. The objective of this study was to estimate the diagnostic value of antibodies against modified citrullinated vimentin (AMCV) and nuclear antigen RA33 of the IgA rheumatoid factor (RF) versus the value of routinely used profile of autoantibodies in diagnostic work-up of RA. Material and methods. 253 patients with RA prehistory of varying duration were included into the study group. The control group was comprised of 92 patients, including patients with seronegative spondyloarthropathies and diffuse connective tissue diseases, as well as sex and age matched healthy controls. Serum levels of IgM and IgA RF, antibodies against cyclic citrullinated peptide (ACCP), ACMV, anti-keratin antibodies (AKA), antibodies against RA33 antigen (ARA33) and antinuclear factor (ANF) were measured in all patients and controls. Results and discussion. Diagnostic sensitivity of AMCV equaled 78%, ACCP — 77%, IgM RF — 71%, IgA RF — 43%, AKA — 43%, ARA33 — 31% and ANF — 31%. All anti-citrullinic antibodies (AKA, ACCP, ACMV) were significantly more commonly associated with IgM RF. Among RF and ACCP seronegative patients ACMV were found in 24% cases with 20 IU/Ml detection threshold, and in 21% — with 30 IU/Ml, allowing to increase diagnostic specificity of the test up to 91% with the increment of diagnostic threshold. Incidence of ARA33 was not significantly different among the RF and ACCP positive or negative subgroups, thus making ARA33 an independent RA marker. Specificity of this marker was 87,9%, thus making it inferior to RF and ACCP by a composite of diagnostic characteristics. Conclusions. Integrated measurement of ACMV and ARA33 is a rational approach at the second stage of serologic testing work-up in suspected cases of RA onset, when initial RF and ACCP tests were negative.

The clinical signifcance of serodiagnostic assay of rheumatoid arthritis increased nowadays. This is a reason to include the citrullinated antigens' antibodies into the new criteria of diagnostics ACR/EULAR 2010. The approbation of national test system detecting the cyclic citrullinated peptide antibodies was implemented. The analysis was applied to 211 blood serum samples taken of 50 blood donors, 60 patients with rheumatoid arthritis, 66 patients with other rheumatoid diseases. In addition, 35 samples were concurrently analyzed with the comparative test system (CCP2 Euroimmun, Germany). The referential meanings of standard limits were established on the basis of results of study of samples taken from healthy blood donors. When the standard limit was less than 10 arbitrary units the sensitivity made up 75% and the specificity--87.9%. In the case of higher values of citrullinated antigens' antibodies which are more than 15 arbitrary units, the sensitivity made up 68% and the specificity--93.1%. The results of comparing with the comparative test system characterized by high convergence made up 94% (33 out of 35), but the comparative test system detected citrullinated antigens' antibodies in 2 samples. The positive qualitative results of both methods analysis of autoantibodies weakly correlated with one another (r = 0.14). The results testify that the parameters of national test system correspond to the publication data concerning the second generation methods of cyclic citrullinated peptide antibodies detection though yield to the best foreign analogues.

The connective tissue systemic diseases originate from pathologic process following with antinuclear antibodies emergence. To detect these antibodies a significant number of diagnostic tests and techniques has been applied. Besides that, there is no conventional algorithm of antinuclear antibodies diagnostic. To detect antinuclear antibodies a two-fold diagnostic algorithm was applied In the capacity of screening techniques the indirect immunofluorescence technique was applied to the cells of line Hep-2 (antinuclear factor) and detection of antibodies to extractable nuclear antigen. The second stage of diagnostic included the detection of content of more specific antinuclear antibodies using the Lineblott method and the double-helical DNA antibodies. The blood serum from 981 patients with suspected connective tissue systemic diseases, 115 patients with systemic lupus erythematous and 57 healthy individuals was analyzed. The levels of antinuclear factor, nuclear antigen antibodies and double-helical DNA antibodies were detected. The antinuclear factor was detected in 84% and 86% of cases, double-helical DNA antibodies in 55% and 39% of cases depending of reagents using in detecting these characteristics. Among healthy individuals, antinuclear factor was detected in 5% (1/20) of blood serum samples in titers less than 1:160. In the group of patients with suspected connective tissue systemic diseases, antinuclear factor was detected in 48% (474/981) of cases and extractable nuclear antigen in 20% (326/981) of cases. The Lineblott test was positive in 33% (326/981) of patients with suspected connective tissue systemic diseases. Among antinuclear factor positive patients nuclear antigen antibodies were detected in 36% (171/474) and the Lineblott test was positive in 63% (298/474) of cases. Among antinuclear factor negative patients but positive under anti-nuclear antigen identification, the Lineblott test was positive in 6% (28/507) of cases. The two-fold algorithm of nuclear antigen testing is an effective technique to be applied in the clinical diagnostic laboratory. The results of effectiveness of this algorithm demonstrated that this method can ensure 33% of cost savings of testing individuals with higher incidence of diseases.

It’s well known that neutrophils being fundamental effectors of the host protective from bacteria infection can generate various oxidants and proteins with antibacterial functions such as myeloperoxidase (MPO) and lactoferrin (LF). The aim of our study was to determine the MPO and LF concentrations in serum, sputum and lavage of patients with cystic fibrosis in minimum activity and exacerbation periods of disease, with and without Ps. aemginosa. High levels of MPO and LF had been determined in blood, sputum and lavage fluid in minimum activity of disease. There were no difference in MPO and LF serum concentrations between minimum activity and exacerbation periods of disease, but at the same time concentrations of these proteins were significantly higher (p

The protease-antiprotease system and the levels of proinflammatory cytokines IL-8 and TNFα were investigated in the sputum of patients with cystic fibrosis both in remission and in exacerbation of the disease. It was shown that elastase activity correlated with the degree of inflammatory process and was more than 10 times higher in the sputum of patients with the exacerbation of the disease. At the same time αl-PI (the main antiprotease) concentration was only 3-5 times higher. We conclude that imbalance in protease-antiprotease system takes place. Concentration of proinflammatory cytokines IL-8 and TNFα, which play the main part in neutrophil accumulation to the inflammatory site, was also significantly higher in patients with the exacerbation of the disease. (Med. Immunol., 2000, vol. 2, N 4, pp 421-424)