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Biomedical subjects

I Fresko

Publications and source records attributed to I Fresko.

At least 19 recordsLinked to original sources

Interleukin (IL)-12, IL-2, and IL-6 gene polymorphisms in Takayasu's arteritis from Turkey.

Takayasu's arteritis (TA) is a chronic arterial inflammation of unknown etiology involving mainly the aorta and its major branches. Genetic polymorphisms of cytokines are screened as susceptibility factors for TA in Turkey. A total of 94 patients with TA were investigated for the genetic polymorphisms of the interleukin genes IL12, IL2,and IL6 and were compared with 108 healthy control subjects using polymerase chain reaction-sequence-specific primer method. The frequencies of IL12B 1188 C allele (p = 0.03, OR = 1.7) and CC genotype (p = 0.007, OR = 3.7) were both higher in TA patients than in control subjects. TT genotype at IL2-330 (p = 0.006, OR = 2.4) and GG genotype at IL6-174 (p = 0.04, OR = 1.9) were more frequent in TA patients. Lower prevalence of GT genotype at IL2-330 (p = 0.005, OR = 0.4), CG genotype at IL6-174 (p = 0.001, OR = 0.4), and AG genotypes at IL6-598 (p = 0.01, OR = 0.4) were also detected. The polymorphism of IL-12 as well as IL-6 and IL-2 genes may contribute to susceptibility and pathogenesis of TA by altering cytokine production and inducing inflammation.

Adult↗

Hair diameter in systemic lupus erythematosus.

It is widely appreciated that patients with systemic lupus erythematosus (SLE) get thinner and shorter hair. However little work has been done to quantitate this. We assessed hair thickness of SLE patients and compared this to that of patients with rheumatoid arthritis (RA) and healthy controls (HC). Fifty-seven female patients with SLE (mean age: 32 +/- 8 years) and 77 female patients with RA (mean age: 50 +/- 12 years) were studied along with 75 healthy women (mean age: 27 +/- 6 years). Five strands of hair were taken from each subset and mounted on glass slides. Two independent observers, blind to the sources of the hair, measured the hair strands under a light microscope, using a micrometer. Finally, the mean hair thickness between each of the three groups was calculated. The hair in both SLE and RA patients was found to be thinner than that of HC by both observers (P < 0.001). Age adjusted analysis between SLE and HC showed similar results. However, there was no significant difference in hair thickness between SLE and RA. SLE patients have thinner hair compared to HC. More studies are needed to investigate the effect of disease activity, therapy and other factors on hair diameter.

Adult↗

A double-blind trial of depot corticosteroids in Behçet's syndrome.

OBJECTIVES: Corticosteroids are widely used in Behçet's syndrome despite the absence of controlled studies. We assessed the effect of depot corticosteroids primarily for genital ulcers and secondarily for the other mucocutaneous manifestations of Behçet's syndrome. METHODS: We randomized 86 patients who had active disease with genital ulcers to receive either intramuscular corticosteroid injections (40 mg methylprednisolone acetate) or placebo every 3 weeks for 27 weeks. RESULTS: Seventy-six patients (88%) completed the treatment. There were no significant differences in the mean number of genital and oral ulcers, or folliculitis between groups. The mean number of erythema nodosum lesions was less in the corticosteroid group as a whole (P = 0.0046); subgroup analyses revealed that this was significant for females (P = 0.0148) but not for males (P = 0.1). CONCLUSION: Low-dose depot corticosteroids did not have any beneficial effect on genital ulcers. However, it was useful in controlling erythema nodosum lesions, especially among the females.

Adolescent↗

The relationship between arthritis and human parvovirus B19 infection.

In order to evaluate the role of human parvovirus B19 in the etiopathogenesis of autoimmune diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), synovial fluid and blood specimens were collected at 1-month intervals from 20 patients with early synovitis (ES) and 31 with RA. Blood specimens were also collected from 25 patients with SLE, 25 with osteoarthritis (OA) as the diseased control group, and 50 healthy blood donors (HBD) as the healthy control group. Detection of B19 IgM and B19 IgG were performed by enzyme-linked immunosorbent assay from serum specimens, and B19 DNA was detected by polymerase chain reaction from synovial fluid samples. B19 IgM, B19 IgG, and B19 DNA were found in the three patients of the ES group. Subsequently, two of them were diagnosed with RA and one with SLE. B19 DNA was also detected in the synovial fluid of eight patients in the RA group. Of them, all were positive for B19 IgG and half were positive for B19 IgM. B19 IgM was not detected in either of the control groups. To define the role of B19 in the etiopathogenesis and prognosis of undiagnosed arthritis and other chronic inflammatory diseases such as RA and SLE, we need broader serial and prospective studies based on clinical and laboratory collaboration. In conjunction with case reports, these studies would also serve to detect other possible factors in the etiopathogenesis of chronic inflammatory diseases.

Adult↗

Oral health is impaired in Behçet's disease and is associated with disease severity.

OBJECTIVES: This study aimed to investigate the oral health of Turkish patients with Behçet's disease (BD) and whether it is associated with the disease course. METHODS: One hundred and twenty patients with BD, 35 patients with recurrent aphthous stomatitis (RAS) and 65 healthy Turkish controls (HC) were included in the study. Oral health was investigated by indices applied in a BD out-patient clinic. RESULTS: The mean scores of plaque, sulcus bleeding and gingival indices, probing depth and the number of extracted teeth were observed to be higher in patients with BD and RAS compared to HC (P<0.05). In the linear regression analysis, plaque index score was associated with the presence of oral ulcers and male gender. An elevated plaque index score was observed to be a significant risk factor for increased severity score in patients with BD in the logistic regression analysis (P = 0.034). CONCLUSIONS: Oral health is impaired in BD and associated with disease severity. Improvement of the oral health of BD patients may affect their disease course, leading to a better prognosis.

Adult↗

Comparative analysis of the association of HLA-B*51 suballeles with Behçet's disease in patients of German and Turkish origin.

The distribution of the different HLA-B*51 suballeles among patients with Behçet's disease (BD) of German (n=33) and Turkish (n=92) origin in comparison to their presence in the respective ethnically matched healthy control groups (German: n=325, Turkish: n=93) was studied. HLA-B*51x was significantly increased in both patient groups in comparison to the controls (Germans: 58% vs. 12%, OR 9.76, P<0.001; Turkish: 75% vs. 25%, OR 9.13, P<0.001). Molecular subtyping of B*51x revealed HLA-B*51011 and B*5108 as the predominant suballeles in both patient groups and controls although with a slightly increased frequency of HLA-B*5108 in the diseased individuals. HLA-B*5105 was the only further HLA-B*51x subtype detected in one Turkish patient heterozygous also for HLA-B*5101. HLA-B*5107 although present in a Turkish as well as German control was absent in the patient groups. There was also a tendency towards a higher degree of homozygosity for HLA-B*51x in both patient groups versus the matched controls (Germans: 10% in patients vs. 2,5% in controls; Turkish: 27% in patients vs. 13% in controls). Our study further supports previous hypothesis of an association of BD with B51 suballeles which share amino-acid residues at positions 63 and 67 as well as at positions 77-83 for specific peptide binding and natural killer (NK)-cell interactions. This applies to HLA-B*5101 and B*5108, but not to HLA-B*5107 different at position 67, which could be negatively associated with BD.

Alleles↗

A reappraisal of amyloidosis in Behçet's syndrome.

OBJECTIVE: To evaluate the clinical features and outcome of patients with Behçet's syndrome (BS) and amyloidosis and to assess the associated risk factors. METHOD: A chart review was done to determine the frequency of amyloidosis in BS among 4000 patients. Data from 14 BS patients with amyloidosis were compared with data obtained from 718 patients with BS without amyloidosis. Multiple stepwise logistic regression analysis was used to assess the risk factors. RESULTS: All patients with amyloidosis presented with the nephrotic syndrome or significant proteinuria. The mean time to the onset of amyloidosis was 8.1 yr (range 3-15 yr). The mean duration of follow-up after amyloidosis was 3.4 yr (range 1-11 yr). Seven out of 14 patients were alive at the time of the evaluation. Peripheral and pulmonary arterial involvement and arthritis were associated with amyloidosis (P<0.05). CONCLUSION: Amyloidosis in BS is rare and has a 50% mortality rate at 3.4 yr (range 1-11 yr). Peripheral and pulmonary arterial involvement and arthritis seem to be the strongest predictors of amyloidosis in BS.

Adult↗

Intestinal permeability in Behçet's syndrome.

OBJECTIVE: To measure the intestinal permeability in patients with Behçet's syndrome (BS) and to compare the results with those obtained from healthy and diseased controls. METHOD: The study group comprised 34 patients with BS without known gastrointestinal disease. Ten patients with ankylosing spondylitis (AS), 6 with inflammatory bowel diseases (IBD), 17 with systemic lupus erythematosus (SLE), and 15 healthy subjects (HC) constituted the controls. All patients received 100 microCi (3.7 MBq) of chromium-51 EDTA ((51)Cr-EDTA) as a radioactive tracer after a 72 hour abstinence from all drugs. The percentage of the isotope excreted in a 24 hour urinary specimen was the measure of permeability. RESULTS: The percentage (SD) rate of excretion of (51)Cr-EDTA was 4.6 (2.6) in BS, 6 (2.4) in AS, 5.2 (1. 9) in IBD, 5.56 (1.78) in SLE, and 2.3 (1) in healthy controls. (Analysis of variance: f=6.4, p=0.0002. BS v HC, AS v HC, SLE v HC significant.) CONCLUSION: The intestinal permeability in BS was significantly more than that seen among the healthy controls. Similar results in all the diseased controls cast doubt on its specificity.

Adult↗

Behçet's Disease.

Clinical and imaging data suggest that Behçet's disease (BD) can present with a variety of neurologic complications, which may be subclassified into two forms. One is attributable to small venous inflammatory disease with focal or multifocal central nervous system involvement and is seen in the majority of patients. It is designated Central Nervous System-Neuro-Behçet syndrome (CNS-NBS). The other form is due to cerebral venous sinus thrombosis and has limited symptoms and a better prognosis. It is very uncommon for these two types of involvement to occur in the same individual. It is very likely that the two major forms have different pathogeneses. Patients with small vein inflammation should be considered for aggressive treatment. A substantial number of patients in this group will have a relapsing-remitting course; for some, this will evolve into a secondary progressive course. A few patients from this group will have progressive CNS dysfunction from the onset. Acute attacks of CNS-NBS are treated with either oral prednisone (1 mg/kg/d up to 4 weeks or until improvement is observed) or high-dose intravenous methylprednisolone (IVMP), 1 g/d for 5 to 7 days. After either treatment, an oral tapering dose of glucocorticoids should be given over 2 to 3 months to avoid early relapses. Some patients may require the long-term use of low maintenance doses of glucocorticoids to prevent exacerbations. Immunosuppressive agents (eg, azathioprine, cyclosporine A, cyclophosphamide, and chlorambucil), given either alone or in different combinations (eg, azathioprine with cyclosporine) for the long-term treatment of various systemic manifestations of BD, have not been shown to prevent the development of the neurologic complications of the disease, reduce its exacerbations, or stop its progression. Immunomodulatory treatments such as interferon alfa and thalidomide have been shown to be effective in treating some of the systemic manifestations of BD, but there is no information on their effects on the development and progression of CNS-NBS. Cerebral venous sinus thrombosis in BD is treated with either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin given together with a short course of glucocorticoids. Evidence of benefit from this treatment, however, is weak.

Journal Article↗

Increased CD4+CD16+ and CD4+CD56+ T cell subsets in Behçet's disease.

Behçet's disease is a systemic vasculitis of unknown etiology. Various immune abnormalities have previously been shown in Behçet's disease. We investigated T lymphocyte subsets associated with cytotoxic activity and natural killer (NK) cells by flow cytometry in 37 patients with Behçet's disease, 38 healthy controls, and 17 diseased control patients. Compared to the healthy controls, CD4+CD16+ and CD4+CD56+ subsets were found to be higher in the Behçet's disease group as well as in the disease control group (CD4+CD16+: BD = 5 +/- 3, DC = 14 +/- 14, HC = 3 +/- 2, P = 0.001; CD4+CD56+: BD = 11 +/- 5, DC = 18 +/- 17, HC = 8 +/- 6, P = 0.01). CD8+CD16+ and CD8+CD56+ T cell subsets were at normal levels in Behçet's disease but found to be elevated in disease controls. Similarly, NK cells (CD16+CD56+) were high only in the disease control group. Significant increases in CD4+CD16+ and CD4+CD56+ cell subsets in Behçet's patients and disease controls suggest that T cell activation patterns of these subsets in Behçet's disease are similar to those in other inflammatory disorders.

Adult↗

Extrapyramidal type rigidity in rheumatoid arthritis.

OBJECTIVES: We had noted cogwheel rigidity in a number of patients with rheumatoid arthritis (RA). Based on this finding, we aimed to investigate formally the presence of rigidity and cogwheeling in RA patients. Our secondary aim was to survey the co-existence of RA and Parkinson's disease (PD). METHODS: A total of 87 consecutive patients with a diagnosis of RA, 78 patients with PD and 67 otherwise healthy patients attending a dedicated headache clinic participated in the study. RESULTS: Rigidity was observed in 24% of RA, 60% of PD and 2% of headache patients. The frequency among the RA patients was significantly higher compared to that of patients with headache (chi 2 = 15.2; P = 0.00009). The frequency of PD among the RA patients was 2/87 (2.3%), while the frequency of RA among the PD patients was 6/78 (7.7%). CONCLUSION: Rigidity can be observed in approximately a quarter of patients with RA.

Adult↗

Evaluation of the Turkish translation of a disease activity form for Behçet's syndrome.

OBJECTIVE: This study examined the interobserver and intra-observer reliability of the Turkish version of the Behçet's Disease Current Activity Form (BDCAF), which was obtained by a translation and back-translation process. METHODS: Fifty Behçet's syndrome (BS) patients were assessed by four rheumatologists in separate morning and afternoon sessions. RESULTS: The results showed good intra- and interobserver agreement for the oro-genital ulcers and eye involvement of BS, but there was poor agreement between (kappa score = 0.14) and within observers (range for kappa scores 0.09-0.25) for their overall impression of disease activity. Individual low kappa scores were also noted for erythema nodosum, vascular involvement, central nervous system involvement and gastrointestinal involvement. CONCLUSION: These results suggest that the Turkish version of BDCAF may be useful for assessing the classic triad of BS (oro-genital ulceration and eye involvement), but more experience is needed for its other parts.

Adult↗

Behçet's syndrome. The Cerrahpasa experience. Members of the Behçet's Syndrome Research Centre.

Behçet's syndrome (BS) is a systemic vasculitis of unknown etiology. There are several reasons for doubting a primary autoimmune pathogenesis of this condition. Recent information suggests evidence for genetic anticipation. Although there is heightened inflammatory activity, as exemplified by the pathergy reaction, wound healing in BS is normal. BS also runs a more stormy course in adults and in the young. However, the amount of androgen receptors in scrotal skin have not found to be increased. Another unsolved problem is the nature of acne-like lesions and how they differ from acne vulgaris (AV). Only lesions in "non-acne" areas were compared between the two conditions, and no differences were detected. Mortality is increased in BS, especially among males. Azathioprine proved to be effective in disease control after 8 years of follow-up. We have also recently finished a 24 week controlled trial of two doses of thalidomide, 100 mg and 300 mg per day. Both doses were superior to placebo, with no real differences in efficacy, in controlling the oral and genital ulcers.

Acne Vulgaris↗

The management of Behçet's syndrome.

Behçet's syndrome (BS), originally described as a triad of oral aphthae, genital ulcerations and uveitis, is a systemic vasculitis that is prevalent in the Middle east, Far East and in the Mediterranean basin. It is characterized by a heightened state of inflammation although the main drive that initiates and sustains this is not yet elucidated. Suppression of this inflammatory state constitutes the major goal of treatment and therapy is tailored according to the specific manifestations observed. We now have considerable more insight on drug management of BS compared to 20 years ago. Particularly, within the recent past we have learned to use more rationally the agents that were already available to us. This is especially true for azathioprine, cyclosporin A, thalidomide and colchicine. Promising data are also being collected with alpha-interferon. With these agents, significant progress has been achieved in the management of uveitis and mucocutaneous symptoms but treatment issues related to thrombotic problems, major vessel involvement and neurological disease have not yet been resolved.

Behcet Syndrome↗

Genetic anticipation in Behçet's syndrome.

OBJECTIVE: To examine the presence of genetic anticipation in families with Behçet's syndrome (BS). METHODS: A total of 18 families with 40 affected members in two successive generations were evaluated by interviewing them for their ages at the onset of the first symptom of BS and for their ages at the time they fulfilled the diagnostic criteria. RESULTS: It was noted that the age of onset of the first symptom was lower in the second generation in 14 families (p = 0.01) with a mean (SD) age of 20.57 (7.47) years in the children compared with 33.29 (9.92) years in the parents (t = 7.79, p < 0.0001), whereas the diagnostic criteria were fulfilled at an earlier age in the children in 15 families (p = 0.01) with a mean age of 21.2 (6.74) years in the children compared with 36.4 (9.55) years in the parents (t = 7.41, p < 0.0001). CONCLUSION: Genetic anticipation was present in 15 of 18 (84%) of the families with BS in the form of earlier disease onset in the children compared with their parents.

Adolescent↗

Fibromyalgia in Behçet's syndrome.

OBJECTIVE: To ascertain the frequency of fibromyalgia (FM) in Behçet's syndrome (BS) and to evaluate the relationship of FM to Behçet's disease activity. METHOD: Self-questionnaires were completed by 108 patients with BS. Each patient was evaluated by an observer blinded to diagnosis; evaluation included assessment of tender points by palpation. Another observer determined the disease activity of patients at that time. RESULTS: Ten of 108 patients (9.2%) met the American College of Rheumatology criteria for FM. Nine of the patients who met the criteria for FM were women. In contrast to patients without FM, patients with FM had mild to moderate disease activity in which musculoskeletal complaints were common. CONCLUSION: There is a trend for an increased frequency of FM in female BS patients.

Adult↗