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Background Isolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. Methods Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. Results We identified nine patients (six female, median age 48 (23–55) years) with IPV. The diagnosis was confirmed after histopathological examination of all surgical materials. The mean duration of disease before surgery was 88.0 ±70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in six patients and unilateral in three. No mortality was observed, however, one patient had pulmonary artery stenosis and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30(19–67) mm Hg to 21(15–49) mm Hg after surgery (p Conclusions Isolated pulmonary vasculitis can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore, surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients.

Aim:Certolizumab pegol (CZP), an Fc-free, PEGylated tumor necrosis factor inhibitor (TNFi), has shown rapid and sustained reduction in signs and symptoms of rheumatoid arthritis (RA). Elevated rheumatoid factor (RF) level has been associated with RA disease progression and poorer TNFi response. We assessed the efficacy of CZP in patients with early and established RA across baseline RF levels. Methods:This post-hoc analysis included data from 6 trials: C-OPERA (NCT01451203), pooled RAPID trials (RAPID-1 [NCT00152386], RAPID-2 [NCT00160602], J-RAPID [NCT00791999], RAPID-C [NCT02151851]), and EXXELERATE (NCT01500278). Patients who received CZP or placebo/comparator with methotrexate (MTX) were categorized by baseline RF quartiles. Efficacy was assessed with Disease Activity Score-28 erythrocyte sedimentation rate (DAS28-ESR). Results:Overall, 316, 1537, and 908 patients were included in C-OPERA, pooled RAPID trials, and EXXELERATE, respectively. Patient demographics and baseline disease characteristics were similar between treatment groups and across RF quartiles. DAS28-ESR low disease activity (LDA) and remission (REM) rates were numerically higher in the CZP + MTX group than PBO + MTX group at weeks 12 and 24, across RF quartiles. LDA and REM rates in the CZP + MTX groups were comparable across RF quartiles at weeks 12 and 24. Mean DAS28-ESR decreased from week 0 to week 24 in the CZP + MTX groups, across RF quartiles. Conclusion:CZP showed steady efficacy across baseline RF quartiles in patients with early and established RA, over 24 weeks. CZP treatment may be considered in patients with RA irrespective of baseline RF levels and time from diagnosis.

Objective: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). Methods: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. Results: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration

Objective: The aim of this study was to evaluate the relationship between oral ulcer activity and medication adherence according to gender in Behcet's disease (BD) patients. Material and Methods: The study group included 330 BD patients (F/M: 167/163, mean age: 38.5 +/- 10.5 years). Oral ulcer activity and medication adherence were evaluated in the previous month. Medication adherence was evaluated using the 8-item Morisky Medication Adherence Scale (MMAS-8) having a score range of 0 to 8 with high scores indicating better adherence. Low adherence was defined as < 6 points on MMAS-8. Results: Over half of the group had active oral ulcers (n= 219, 66.4%) within the month preceding the visit. The number of oral ulcers was significantly higher in female patients with low medication adherence (2.39 +/- 3.24) than in the rest of the female group (1.28 +/- 2.05; p= 0.023). Although a similar trend was also observed in male patients (2.14 +/- 3.3 vs. 1.81 +/- 2.31), a significant relationship was not observed (p= 0.89). The frequency of medication intake per day was lower in patients with high medication adherence than in the rest of the study group (p= 0.04). Conclusion: Low medication adherence is a hidden risk factor in the management of BD. Poor adherence was associated with oral ulcer activity in female BD patients.

The aim of this study is to investigate the prevalence of subclinical ankle involvement by ultrasound in patients with rheumatoid arthritis (RA). The study was conducted on 216 patients with RA and 200 healthy sex- and age-matched controls. Patients with no history or clinical evidence of ankle involvement underwent US examination. For each ankle, tibio-talar (TT) joint, tibialis anterior (TA) tendon, extensor halux (EH) and extensor common (EC) tendons, tibialis posterior (TP) tendon, flexor common (FC) tendon and flexor hallux (FH) tendon, peroneous brevis (PB) and longus (PL) tendons, Achilles tendon (AT) and plantar fascia (PF) were assessed. The following abnormalities were recorded: synovitis, tenosynovitis, bursitis, enthesopathy and rupture. BMI, DAS28, RF ESR and CRP were also obtained. A total of 432 ankles of patients with RA and 400 ankles of healthy controls were assessed. In 188 (87%) patients with RA, US showed ankle abnormalities whereas, in control group, US found abnormalities in 57 (28.5 %) subjects (p = 0.01). The most frequent US abnormality in RA patients was TP tenosynovits (69/216) (31.9 %), followed by PL tenosynovitis (58/216) (26.9 %), TT synovitis (54/216) (25 %), PB tenosynovitis (51/216) (23.6 %), AT enthesopathy (41/216) (19 %) and AT bursitis (22/216) (10.2 %). In 118 RA patients out of 216 (54.6%), a positive PD was found. No statistically significant correlation was found between the US findings and age, disease duration, BMI, DAS28, RF, ESR and CRP. The present study provides evidence of the higher prevalence of subclinical ankle involvement in RA patients than in age- and gender-matched healthy controls identified by US.

Objective.To investigate the effect of depression, anxiety, fatigue, and fibromyalgia (FM) on the remission status in patients with rheumatoid arthritis (RA), defined according to the 28-joint count Disease Activity Score (DAS28)-erythrocyte sedimentation rate (ESR) and the Boolean-based new American College of Rheumatology/European League Against Rheumatism remission criteria.Methods.The subjects were patients with RA who participated in a hospital-based observational cohort. Patients who met the DAS28-ESR remission criteria at their latest visit were invited to participate in our study. The patient groups fulfilling or not fulfilling the Boolean remission criteria were identified and compared with each other with regard to the presence of depression, anxiety, fatigue (0–50), and FM. The relationship between psychosocial factors and Simplified Disease Activity Index (SDAI) remission, which is the index-based definition of remission in RA, was also investigated.Results.A total of 87 out of 428 patients (20%) with RA met the DAS28-ESR remission criteria and 32 (37%) of these also met the Boolean remission criteria, while 55 (63%) did not. Forty patients were also in SDAI remission. In the Boolean remission group, 2 patients had depression and 2 had anxiety (p = 0.004). In the Boolean nonremission group, 19 patients had depression and 13 had anxiety (p = 0.04). Continuous scales of anxiety (3.34 ± 3.76 vs 5.83 ± 4.70, p = 0.012) and depression (2.18 ± 2.75 vs 4.63 ± 4.10, p = 0.001) were also lower in the Boolean remission group in comparison with the nonremission group. Though FM syndrome was detected in only 1 patient of the Boolean remission group and in 7 patients of the Boolean nonremission group (p = 0.249), patients’ polysymptomatic distress scores of FM in the Boolean remission group were significantly lower than those of the nonremission group (3.12 ± 3.25 vs 6.27 ± 5.19, p = 0.001). The mean fatigue scores were 9.5 ± 10.6 in the Boolean remission group and 16.8 ± 12.8 in the Boolean nonremission group (p = 0.006). In multivariate analysis, patient’s global assessment (PtGA) and depression were found as the independent discriminators of Boolean-based definition. Similar relationships were also observed between psychosocial factors and SDAI remission.Conclusion.In patients with RA who do not fulfill the Boolean remission criteria, to avoid overtreatment, assessment of anxiety, fatigue, FM, and especially depression must be considered if PtGA scores and disease activity variables are significantly different.

The clinical course of Behcet’s disease (BD) as a multisystemic disorder with a remitting-relapsing nature is insufficiently explored. As complete remission should be aimed in all inflammatory diseases, we investigated the frequency of complete remission in patients with BD followed in long-term, routine practice. In this retrospective study, 258 patients with BD who were regularly followed in outpatient clinics were assessed. The demographic and clinical data for active organ manifestations and treatment protocols were evaluated, and “complete remission” for this study was defined as no sign of any disease manifestation in the current visit and the preceding month. Two hundred fifty-eight patients with BD (F/M 130/128, mean age 41.1 ± 11.5 years) were included to the study. Mucocutaneous disease was present in 48.4 % (n = 125). Mean visit number was 6.8 ± 2.7, and mean follow-up duration was 45.8 ± 36.5 months. Patients were clinically active in 67.2 % (n = 1,182) of the total visits (n = 1,757), which increased to 75.6 % (68.1–90.3) when the month preceding the visit was also included. The most common active manifestation was oral ulcers (39.4–63.2 %) followed by other mucocutaneous manifestations and musculoskeletal involvement. When multivariate analysis was performed, oral ulcers, which are the main cause of the clinical activity, negatively correlated with immunosuppressive treatments (β = −0.356, p < 0.000) and age (β = −0.183, p = 0.04). It is fairly difficult to achieve complete remission in BD with current therapeutic regimens. The reluctance of the clinician to be aggressive for some BD manifestations with low morbidity, such as mucocutaneous lesions and arthritis, might be influencing the continuous, low-disease activity state, especially due to oral ulcers, in BD patients.

Summary Objective The aim of this study was to investigate the effects of azithromycin on mucocutaneous manifestations and ex vivo intracellular cytokine responses in patients with Behcet's disease (BD). Methods Ten BD patients with active manifestations and nine healthy controls (HCs) were included in the study. Patients were treated with azithromycin (1500 mg/week) for four weeks. Clinical and immunological responses were evaluated in the pre- and post-azithromycin treatment periods. Peripheral blood mononuclear cells (PBMCs) of patients and controls were stimulated by Streptococcus sanguinis, lipopolysaccharide (LPS), lipoteichoic acid (LTA), and heat shock protein-60 (HSP-60) for three hours. Ex vivo intracellular interferon-γ (IFN-γ) and tumor necrosis factor-α (TNF-α) levels were measured. Results Follicular lesions and genital ulcers completely healed, and the number of oral ulcers decreased after treatment (P = 0.000). The stimulated intracellular IFN-γ response to S. sanguinis was higher in BD patients (5.75%) than in HCs (3.9%) before treatment (P = 0.05). Likewise, the pretreatment IFN-γ response was significantly higher than the post-treatment response (1.95%). In BD patients, pretreatment stimulated intracellular IFN-γ responses to LTA (5.8%) were also higher than post-treatment responses (3.15%), but the difference did not reach statistical significance (P = 0.07). Conclusions Azithromycin treatment decreased the mucocutaneous manifestations in BD patients and suppressed the intracellular IFN-γ responses of PBMCs to S. sanguinis ex vivo, which suggests this treatment has an immunomodulatory effect.

Background Oral ulcer is the cardinal clinical sign and increased neutrophilic activity is a part of the pathogenesis in Behcet's disease (BD). Saliva, as a part of the innate immune response, contains antimicrobial peptides (AMPs) that are derived from both oral epithelial cells and neutrophils. The aim of this study was to investigate the associations between salivary levels of AMPs HNP 1-3, LL-37 and S100 and disease course in patients with Behcet's disease (BD). Methods Fifty-three patients with BD and 44 healthy controls (HC) were included in the study. Disease severity score reflecting organ involvement was calculated. Salivary HNP 1-3, LL-37 and S100 levels were measured in unstimulated saliva samples by ELISA. Results Salivary HNP 1-3 and S100 levels in BD patients (2715.2 ± 1333.4 μg/ml and 430.6 ± 203.9 ng/ml) were significantly higher compared to HC (1780.6 ± 933.2 μg/ml and 365.3 ± 84.7 ng/ml) (p = 0.000 and p = 0.004, respectively). Although LL-37 levels were also higher in BD than HC (190.9 ± 189.1 vs 143.1 ± 128.9 ng/ml), no significant difference was observed (p = 0.53). Salivary HNP 1-3 and LL-37 levels were associated with the severity of BD (mild disease: 1975.1 ± 1174.2 μg/ml and 115.9 ± 109.4 ng/ml vs severe disease: 2955.7 ± 1305.6 μg/ml and 215.3 ± 203.8 ng/ml, p = 0.020 and p = 0.031, respectively). Salivary LL-37 levels also correlated with the number of monthly oral ulcers (r = 0.5 p = 0.000). Conclusion An increase in salivary HNP 1-3 and S100 levels might be associated with enhanced local and systemic innate responses in BD.

Aim: The laboratory findings are non-specific in Rheumatoid arthritis (RA). We have based our study on this idea and chose cytokines and chemokine to evaluate ; we then tried to establish a relationship between the progress of the disease and the levels of these molecules before and 3-6 months after the TNF-α blocker treatment.Material and Methods: A total of 34 RA patients (28 females, 6 males) who were diagnosed with RA and received TNF-α blocker therapy were included in the study.Blood samples were drawn from the patients before the TNF-α treatment began and then in the following 3rd and 6th months of the treatment.Results: The mean age of the study group is 49.15±11.03. the mean disease period is 9.77±6.29 years and the mean treatment period is 6.88±4.33 years. 84.6% (n=22) of the study group is female and 15.4% (n=4) is male.Patients with good treatment response to TNF-α blocker treatment were younger than the patients with medium treatment response. The increase of mean hemoglobin (Hgb) levels between 0 and 6 months were statistically meaningful (both p0.05).Conclusion: In RA patients receiving TNF-α blocker therapy. cytokine levels decreased with treatment.The relationship between IL-6 and DAS28 change in the pre-treatment and 6-month period may help the clinician to show the disease activity and to guide the course of the disease.

Background Although number, frequency and healing time of oral ulcers and pain are generally used for clinical practice and studies in Behcet's disease (BD) and recurrent aphthous stomatitis (RAS), no standardized activity index is currently present to monitor clinical manifestations associated with oral ulcers. The aim of this study was to develop a standardized composite index (CI) to assess oral ulcer activity in BD and RAS. Methods In this cross-sectional study, 121 patients with BD and 45 patients with RAS were included. Sixty-five percentage of BD and 68.9% of RAS patients were in active stage during the previous 3 months. The developed CI included the presence of oral ulcers, ulcer-related pain and functional status and was evaluated in patients with both active and inactive disease for content validity. Results Composite index score was observed to be higher in active patients with RAS (6.94 + or - 2.19) compared with active BD patients (6.01 + or - 2.04) (P = 0.04). The number of oral ulcers and healing time of oral ulcers were significantly higher in RAS compared with BD (P = 0.018, P = 0.001 respectively). CI score correlated with the number of oral ulcers in both BD and RAS (P = 0.000, P = 0.002 respectively). CI score was '0' for inactive patients without oral ulcer in BD and RAS. Conclusions The presented CI as an oral ulcer activity index seems to be a reliable and suitable tool for evaluating the clinical impact and disease-specific problems in BD and RAS.

Objective To investigate the frequency and clinicopathological features of skin involvement in rheumatoid arthritis (RA), to find out whether early and aggressive disease-modifying treatment is changing the spectrum towards a milder disease pattern. Methods Two hundred and fifteen consecutive RA patients were enrolled. Main outcome measures were the frequency of cutaneous features and their relation to disease severity and treatment modality, ultrasound (USG) examination of nodules, histopathological examination of nodules and papules. Results Mean age and disease duration were 55.3 years and 138.1 months, respectively. Sixty-six percent of the patients had erosive disease, 70% were rheumatoid factor (RF) positive and 44% had Classes III and IV functional index. Among 43 patients having nodules, 20 were diagnosed as rheumatoid nodules (RNs) and the others as synovitis, bursitis, and so on, on clinical basis and by ultrasound. In 7 of 20 RNs, diagnosis was confirmed by histopathological examination. When clinical, histopathological, and USG data were analyzed collectively, sensitivity, and specifity of USG were found as 100 and 75%, respectively. Sixteen patients had relevant papular lesions. Fourteen of these were diagnosed as palisading neutrophilic granulomatous dermatitis (PNGD) and two as rheumatoid neutrophilic dermatitis (RND) on histopathological examination. Accelerated nodulosis was seen in one, and vasculitis in two of the patients. Conclusions We observed a milder disease pattern compared to Anglo-Saxon countries with lower RN and vasculitis frequency. This cannot be explained by early and aggressive treatment as disease onset to treatment interval was long in our patient group. PNGD resembles RN in terms of frequency and association with severe disease.

Background: The aim of the study was to test multidimensional properties of oral health impact profile-14 (OHIP-14) in Behcet's disease (BD) and recurrent aphthous stomatitis (RAS) patients with active oral ulcers. Methods: Ninety-six BD patients, 28 patients with RAS and 117 healthy controls (HC) were included in this study. In patients with active oral ulcers, the frequency and healing time of ulcers were recorded. Multidimensional properties of OHIP-14 were examined by factor analysis. Results: Factor analysis revealed three subscales and explained 66.49% of overall variance in these patients with active oral ulcers. The score of Subscale 1 was positively correlated with the recurrence of oral ulcers per month (P = 0.037). Subscale 3 scores of the patients treated with colchicine were worse than those treated with immunosuppressives (P = 0.035). Conclusions: The factor structure of OHIP-14 was found to be reliable and sensitive to clinical parameters and treatment modalities in active patients.

Objectives: This study aimed to investigate oral and general health related quality of life (QoL) in patients with Behcet's Disease (BD) and to assess the performance of Turkish versions of oral health related quality questionnaires. Subjects and methods: Ninety-four BD patients, 24 patients with recurrent aphthous stomatitis (RAS), 113 healthy controls (HC) and 44 dental patients were investigated. QoL was assessed by oral health impact profile-14 (OHIP-14), oral health related quality of life (OHQoL) and short form-36 (SF-36) questionnaires. Results: OHQoL, OHIP-14 and SF-36 subscale scores were significantly worse in patients with BD compared with those in HC (P

Objective.To compare the Quantiferon-TB Gold test (QTF-G) with the tuberculin skin test (TST) for the detection of latent tuberculosis infection (LTBI) among patients with rheumatoid arthritis (RA) and ankylosing spondylitis (AS), with reevaluation of the patients treated with tumor necrosis factor-α (TNF-α) antagonists in the followup.Methods.The study involved 140 consecutive patients, 82 with RA and 58 with AS. Thirty patients were evaluated with QTF-G for detection of LTBI before and after 6 months of TNF-α antagonist treatment. QTF-G was also performed on 49 healthy controls. QTF-G results were recorded as positive, negative, or indeterminate. A positive TST was defined as ≥ 5 mm for RA and AS.Results.The percentages of positive QTF-G were comparable in RA and AS (37% vs 32%). The rate of positive QTF-G in healthy controls (29%) was also similar to RA and AS. In contrast to QTF-G results, a high rate of TST positivity was observed in AS compared to RA (82% vs 55%; p = 0.02). The total agreement between QTF-G and TST was observed to be 61% (κ = 0.29) in the whole group, 70% (κ = 0.42) in RA, and 49% (κ = 0.14) in AS. After 6 months of treatment with TNF-α antagonists, a high rate of QTF-G change was observed in patients with indeterminate results (23% vs 3%; p = 0.03).Conclusion.The comparable prevalence of LTBI among the study groups according to QTF-G supports the view that QTF-G is less susceptible to external factors than TST. Sequential testing for QTF-G in patients with indeterminate or negative results may also be helpful in discriminating LTBI better.

Mannose binding lectin (MBL), a member of the collectin family proteins, is a major molecule of the innate immune system; MBL deficiency is associated with increased susceptibility to infections. As gastrointestinal and genitourinary infections are suggested to be among the etiological factors of spondyloarthropathies (SpA), we investigated MBL deficiency in ankylosing spondylitis (AS) and undifferentiated SpA (uSpA).One hundred seven patients with AS, 43 patients with uSpA, and 74 healthy controls were studied. Disease activity, radiological scores, and demographic features were recorded. MBL levels were measured with standard ELISA kits.Median MBL levels in AS, uSpA, and controls were 2705 (range 0-5861) ng/ml, 2897 (36-7586) ng/ml, and 3468 (0-7950) ng/ml, respectively. No significant differences were observed in median MBL levels and the prevalence of MBL deficiency between the groups. Bath AS Radiological Index scores were not affected by MBL levels. However, although statistically not significant, radiographic damage quantified by modified Stoke AS Spine Score (mSASSS) was 3 times higher in AS patients with MBL deficiency. Disease activity, clinical picture, and therapies were not associated with MBL levels.In AS patients with MBL deficiency, there was a tendency towards a more severe radiographic progression detected by mSASSS.