muramidase and Behcet-Syndrome

We investigated the association between autoantibodies against non-myeloperoxidase (MPO) neutrophil granule antigens and activity of Behçet's disease (BD).. We consecutively enrolled 51 BD patients. We assessed clinical data and BD activity using patients' index scores from the Behçet's Disease Current Activity Form and we performed tests for antibodies against proteinase 3 (PR3), MPO, bactericidal permeability increasing protein (BPI), cathepsin G, elastase, lactoferrin and lysozyme.. The median patient index score was 2.0, and 56.9% of patients had active BD. In multivariate analysis of variables with significant correlations, only anti-lysozyme showed a significant correlation with BD activity (P = 0.002). In multivariate logistic regression analyses of variables, when patients were classified into groups according to the optimal cutoff levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and anti-lysozyme (ESR > 42.5 mm/h, CRP > 1.35 mg/L and anti-lysozyme > 2.95 IU/mL), the variable with independent predictive value was anti-lysozyme (odds ratio 8.384, P = 0.015).. Anti-lysozyme was significantly correlated with disease activity score and it was the only independent value to predict active disease in patients with BD. Furthermore, patients having anti-lysozyme levels ≥ 2.95 IU/mL had a significantly higher risk of having active BD than those who did not.

Topics: Adult; Aged; Autoantibodies; Behcet Syndrome; Biomarkers; Chi-Square Distribution; Female; Humans; Linear Models; Logistic Models; Male; Middle Aged; Multivariate Analysis; Muramidase; Odds Ratio; Predictive Value of Tests; Prognosis; Severity of Illness Index; Up-Regulation; Young Adult

Increased serum levels of angiotensin converting enzyme and lysozyme are considered as inflammatory markers for diagnosis of sarcoidosis which is an autoimmune inflammatory disease. The purpose of this study is to evaluate the significance of differences in serum angiotensin converting enzyme and lysozyme levels of patients with ocular involvement of other autoimmune inflammatory and infectious diseases.. This is a prospective study involving patients with ankylosing spondylitis, behcet's disease, presumed sarcoidosis, presumed latent tuberculosis, presumed latent syphilis, and control group. The serum levels of angiotensin converting enzyme and lysozyme were analyzed by enzyme-linked immunosorbent assay. Bonnferoni analysis was used to assess pairwise comparisons between the groups.. There was a significant increase in serum angiotensin converting enzyme level in patients with presumed sarcoidosis compared to ankylosing spondylitis (p = 0.0001), behcet's disease (p = 0.0001), presumed latent tuberculosis (p = 0.0001), presumed latent syphilis (p = 0.0001), and control group (p = 0.0001). The increase in serum lysozyme level was significant for patients with presumed sarcoidosis with respect to ankylosing spondylitis (p = 0.0001), behcet's disease, (p = 0.0001) presumed latent tuberculosis (p = 0.001), presumed latent syphilis (p = 0.033), and control group (p = 0.0001).. Elevated serum angiotensin converting enzyme levels are significant for patients with presumed sarcoidosis compared to ocular involvement of other autoimmune diseases such as behcet's disease and ankylosing spondylitis, and ocular involvement of infectious diseases such as presumed latent tuberculosis and presumed latent syphilis. However, elevated serum lysozyme level might be also detected in ocular involvement of infectious diseases such as presumed latent tuberculosis and presumed latent syphilis.. NCT02627209. Date of registration: 12/09/2015.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Autoimmune Diseases; Behcet Syndrome; Child; Communicable Diseases; Enzyme-Linked Immunosorbent Assay; Female; Humans; Latent Tuberculosis; Male; Middle Aged; Muramidase; Peptidyl-Dipeptidase A; Prospective Studies; Sarcoidosis; Spondylitis, Ankylosing; Syphilis

Unusual histopathologic structures were observed in lobular panniculitis of a patient with Behçet's disease. These were polyp-like in shape and protruded into cavities made of lysed fat cells. Various stages of development were observed. To determine the origin of cells making up these structures, we applied various histochemical and immunohistochemical techniques and found the cells to be histiocytes (macrophages) engaged in phagocytizing degenerated fat cells and cell membranes which had formed into membranocystic lesions.

Topics: Adipose Tissue; Aged; alpha 1-Antichymotrypsin; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Behcet Syndrome; Cell Lineage; Cell Membrane; Histiocytes; Histocytochemistry; Humans; Hyaluronic Acid; Immunohistochemistry; Macrophages; Male; Muramidase; Panniculitis; Phagocytosis

Total neopterin (T-N), a by-product in the biopterin biosynthesis and an indicator of activation of the cellular immune system, and total biopterin (T-B) levels in cerebrospinal fluid (CSF), were measured in patients with various inflammatory neurological diseases and Parkinson's disease, and the following results were obtained. (1) In patients with neuro-sarcoidosis, neuro-Behçet's disease and meningitis, CSF T-N levels were markedly elevated in the exacerbation or acute stages of their neurological symptoms and remarkably decreased in the remission or chronic stages. In the neuro-sarcoidosis and neuro-Behçet's disease patients, however, CSF T-B levels showed no substantial change. (2) There was a significant positive correlation between CSF T-N levels and CSF/serum albumin ratios only in the meningitis patients. However, increases of CSF T-N levels were not associated with those of plasma T-N levels. (3) In the Parkinson's disease patients, CSF T-N levels remained normal, although CSF T-B levels significantly decreased. (4) A gradient for the CSF T-N value (lumbar greater than ventricular CSF), being reverse to the CSF T-B value, was observed. These results indicate that the significance of CSF T-N is quite different from CSF T-B, and that CSF T-N appears to be a valuable biochemical marker for evaluating the activity of inflammation within the central nervous system. Its measurement seems useful for therapeutic monitoring, especially of patients showing the chronic exacerbating-remitting course.

Topics: Adult; Angiotensin-Converting Enzyme Inhibitors; Behcet Syndrome; Biopterins; Female; Humans; Immunity, Cellular; Male; Meningitis; Muramidase; Neopterin; Nervous System Diseases; Sarcoidosis

The functions of phagocytes are enhanced in patients with Behçet's disease, therefore, we investigated the neutrophil-derived oxygen intermediates (OI) and lysosomal enzymes from 17 patients receiving glucocorticosteroids (steroids) and colchicine. Cultured endothelial cells were incubated with neutrophils to assess tissue injury. In cases of the complete type, in the active stage of the disease, OI production was markedly increased. The other patients showed significantly higher OI and higher lysosomal enzyme levels than patients with other diseases (controls) receiving drug therapy. Cytotoxicity tests showed that the 51Cr release was also significantly higher. The destruction of desmosomes and cell deformation were demonstrated electron microscopically. The simultaneous addition of superoxide dismutase and catalase in the cell culture decreased the 51Cr release to control levels. These findings suggest that neutrophils from patients with Behçet's disease generate high levels of OI, resulting in endothelial tissue damage.

Topics: Adolescent; Adult; Behcet Syndrome; Cytotoxicity, Immunologic; Endothelium; Female; Glucuronidase; Humans; Hydrogen Peroxide; Luminescent Measurements; Male; Microscopy, Electron; Middle Aged; Muramidase; Neutrophils; Oxygen Consumption

Topics: Adolescent; Adult; Behcet Syndrome; Female; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Muramidase; Neutrophils; Oxygen

The concentrations and sequential changes of some acute phase proteins, factor B, and lysozyme have been assayed in recurrent oral ulceration and Behçet's syndrome. C9 was elevated in both groups of patients and was the sensitive index of disease activity; however, it failed to discriminate between the three types of recurrent oral ulcers and four types of Behçet's syndrome. The level of alpha 1 acid glycoprotein and lysozyme were significantly increased predominantly in the ocular type, whereas factor B was significantly increased especially in the neurological type of Behçet's syndrome. It is suggested that the changes in the concentrations of some plasma proteins may help our understanding of tissue involvement in Behçet's syndrome, as well as in the selection of therapeutic agents in this disease.

Topics: Azathioprine; Behcet Syndrome; Blood Proteins; C-Reactive Protein; Complement C9; Complement Factor B; Humans; Muramidase; Orosomucoid; Prednisolone; Recurrence; Stomatitis, Aphthous; Stomatitis, Herpetic