Assessment of venous disease with different venous disease specific scales in Behçet's disease patients with deep vein thrombosis.

Objectives: Post-thrombotic syndrome (PTS) is a frequent and important consequence of deep vein thrombosis (DVT) for Behcet`s disease (BD) patients. Although various clinical scales are used to diagnose PTS, Villalta scale was accepted as the standard tool to diagnose and grade the severity of PTS. Poor quality of life (Qol) in the general population was defined for patients with PTS, however, studies in BD patients with PTS is limited. Our aim was to compare the performance of different scales to assess venous disease in BD patients with a history of DVT and to assess the relationship with quality of life.Methods: Patients with BD (n = 194, M/F:157/37, age:39.1 ± 9.5 years) with a DVT history were investigated. Villalta, VCSS,CEAP scale and SF 36,Veines scales were used to assess venous disease and QoL respectively.Results: Among BD patients, 120 (61.9 %) patients were classified as having PTS by Villalta and of patients 18% had severe PTS. Half of patients with CEAP score <4 were classified as having PTS. Also, 42% of patients with CEAP>4 and almost two third of VCSS classified severe CVD patients was grouped in severe PTS by Villalta scale. VCSS and Villalta classified PTS patients had decreased disease specific and general Qol scores compared to the patients without PTS. Also, severe PTS group (by VCSS) had decreased veines QoL scores and PCS compared to mild/moderate group.Conclusion: BD patients with DVT have a high risk of PTS. Our results show that both Villalta scale and VCSS should be used to assess venous disease BD patients with DVT. However, VCSS classified severity of PTS can show better correlation with venous disease -specific QoL.

Does decision tree analysis predict oral ulcer activity-related factors in patients with Behçet's syndrome?

OBJECTIVES: The study aimed to identify the interactions among treatment protocols and oral ulcer activity related factors in patients with Behçet's syndrome (BS) using the Classification and Regression Tree (CART) algorithm. METHODS: In this cross-sectional study, 979 patients with BS were included from16 centres in Turkey, Jordan, Brazil and the United Kingdom. In the CART algorithm, activities of oral ulcer (active vs. inactive), genital ulcer (active vs. inactive), cutaneous involvement (active vs. inactive), musculoskeletal involvement (active vs. inactive), gender (male vs. female), disease severity (mucocutaneous and musculoskeletal involvement vs. major organ involvement), smoking habits (current smoker vs. non-smoker), tooth brushing habits (irregular vs. regular), were input variables. The treatment protocols regarding immunosuppressive (IS) or non-IS medications were the target variable used to split from parent nodes to purer child nodes in the study. RESULTS: In mucocutaneous and musculoskeletal involvement (n=538), the ratio of IS use was higher in patients with irregular toothbrushing (ITB) habits (27.1%) than in patients with regular toothbrushing (RTB) habits (14.2%) in oral ulcer activity. In major organ involvement (n=441), male patients with ITB habits were more likely treated with IS medications compared to those with RTB habits (91.6% vs. 77.6%, respectively). CONCLUSIONS: Male BS patients on IS who have major organ involvement and oral ulcer activity with mucocutaneous and musculoskeletal involvement have irregular toothbrushing habits. Improved oral hygiene practices should be considered to be an integral part for implementing patient empowerment strategies for BS.

The assessment of presenteeism and activity impairment in Behçet's syndrome and recurrent aphthous stomatitis: a multicentre study.

OBJECTIVE: To evaluate key factors for presenteeism and activity impairment in multinational patients with Behçet's syndrome (BS) and recurrent aphthous stomatitis (RAS). METHODS: In this cross-sectional study, 364 BS patients from Jordan, Brazil, the United Kingdom and Turkey and 143 RAS patients from the United Kingdom and Turkey were included. The Work Productivity Activity Impairment (WPAI) scale was used for presenteeism and activity impairment. Mediation analyses were performed to evaluate both direct and indirect causal effects. RESULTS: Presenteeism score was higher in active patients with genital ulcers and eye involvement as well as patients with comorbidities and current smokers than the others in BS (P < 0.05). In RAS, presenteeism score was elevated by oral ulcer activity in the direct path (P = 0.0073) and long disease duration as a mediator in the indirect path (P = 0.0191). Patients with active joint involvement had poor scores in absenteeism, presenteeism, overall impairment and activity impairment compared with those of inactive patients (P < 0.05). Using mediation analysis, the activity impairment score was directly mediated by joint activity (P = 0.0001) and indirectly mediated through oral ulcer-related pain in BS (P = 0.0309). CONCLUSION: In BS, presenteeism was associated with disease activity, presence of comorbidities and being a current smoker, whereas in RAS, presenteeism was associated with oral ulcer activity and increased length of the disease. Moreover, activity impairment was adversely affected by joint activity and oral ulcer related pain in BS. Patients need to be empowered by using appropriate treatment strategies in their working environment and daily life.

Assessment of damage in Takayasu's arteritis.

OBJECTIVE: To assess the progression and the factors associated with damage in Takayasu's arteritis (TAK) patients during routine follow-up. METHODS: Patients diagnosed with TAK and had >6 months follow-up were enrolled in this study retrospectively. Takayasu's arteritis damage score (TADS) and vasculitis damage index (VDI) were determined at diagnosis and at the end of the follow-up and variables associated with damage scores were assessed. RESULTS: One-hundred fourteen patients (F/M: 101/13) were included in the study. The mean age at diagnosis, median symptom duration at baseline visit and mean follow-up duration were 35.3±13.3 years, 12 (0-360) months and 76.9±51.4 months, respectively. Median VDI score was 4.0 (1-8) and median TADS score was 7.0 (1-15) at baseline assessment. At the end of the follow-up, median VDI score increased to 5.0 (1-17) and TADS score to 8.0 (1-19). The median number of disease-related items were higher in TADS (8 items vs 4 items). At least one new corticosteroid (CS)-related damage item occurred in 35 patients (31%). Age at symptom-onset and cumulative CS doses were predictor factors for higher VDI score (≥5), whereas age at symptom-onset and disease duration were associated with increase in TADS (≥8). Gender and number of relapses were not associated with damage scores. CONCLUSION: Damage assessment with VDI seems to capture treatment-related damage better, whereas TADS provides some additional information on disease-related damage in Takayasu's arteritis. Older age at symptom onset, disease duration and cumulative CS dose were associated with higher damage scores. The relapse frequency did not influence the damage level in our routine-follow-up of TAK patients.

Damage assessment in adult IgA vasculitis. Cross-sectional results of a multicentre cohort.

OBJECTIVES: Immunoglobulin (Ig) A vasculitis affects children more commonly than adults and previous literature lacks any formal damage assessment. Our aim in this study is to investigate the disease course, relapse rates and prognostic factors in adult patients with IgA vasculitis and to evaluate the disease-related damage. METHODS: We assembled a retrospective cohort of adult IgA vasculitis from six tertiary Rheumatology Centres in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records. RESULTS: The study included 130 (male/female: 85/45) patients and the mean age was 42.2±17 years. Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations. One hundred thirteen patients (86.9%) were treated with oral glucocorticoids (GC). As additional immunosuppressive (IS) agents, azathioprine was given to 44 (34.9%) and pulse cyclophosphamide to 18 (12.6%) patients. Seventy-nine patients (60%) had follow-up of median 15 (IQR 7-40) months. Twelve (15%) patients relapsed during follow-up. The mean VDI score was 0.4 in the last visit. Nineteen (24.7%) patients had at least one damage item at the end of follow-up. Most frequent damage items were renal 11 (42%), ocular 4 (15%) and cardiovascular 4 (15%). CONCLUSIONS: In this cohort the most frequent damage item was renal and was related to the disease itself. Damage score was higher in patients with more severe disease and treated more aggressively. Our results suggest that more effective treatment options are needed in a subgroup of patients with IgA vasculitis to prevent the damage related with the vasculitis, especially with more severe disease.

Predictive factors for work-day loss in Behçet's syndrome: A multi-center study.

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 <5 years (74%) were higher in male patients with work-day loss (P < .05). The majority of males (90.9%) had more than four clinic visits during the previous year. Moreover, the mean work-day loss (30.8 ± 57.7 days) was higher in patients with vascular involvement (56.1 ± 85.9) than those without (26.4 ± 50.6 days) (P = .046). In addition, increased frequency of ocular involvement (25.9%) was also observed in patients with work-day loss compared to others (8.6%) (P = .059). CONCLUSION: Work-day loss was associated with both vascular and ocular involvement. Close associations were observed among male gender, early period of the disease, frequent medical visits, being a smoker and treatment with IS medications in patients with work-day loss.

Oral ulcer activity assessment with the composite index according to different treatment modalities in Behçet's syndrome: a multicentre study.

OBJECTIVES: The aim of this multicentre study was to understand patients' needs and to evaluate the oral ulcer activity with the Composite Index (CI), according to different treatment modalities in Behçet's syndrome (BS). METHODS: BS patients (n=834) from 12 centres participated in this cross-sectional study. Oral ulcer activity (active vs. inactive) and the CI (0: inactive vs. 1-10 points: active) were evaluated during the previous month. The effects of treatment protocols [non-immunosuppressive: non-IS vs. immunosuppressive: (ISs)], severity (mild vs. severe), disease duration (<5 years vs. ≥5 years) and smoking pattern (non-smoker vs. current smoker) were analysed for oral ulcer activity. RESULTS: Oral ulcer activity was observed in 65.1% of the group (n=543). In both genders, the activity was higher in mild disease course with non-IS treatment group compared to severe course with ISs (p<0.05). As a resistant group, patients with mild disease course whose mucocutaneous symptoms were unresponsive to non-IS medications were treated with ISs in a limited period and achieved the highest CI scores in females. Oral ulcer activity and poor CI score were associated with disease duration less than 5 years compared to others in male patients (p<0.05). CONCLUSIONS: Oral ulcer activity pattern is affected by both the combination of disease course, treatment protocols and disease duration. CI scores reflected the oral clinical activity and CI might be a candidate scale to evaluate the efficacy of treatments during the follow-up of oral ulcer activity in BS.

Does illness perception associate with disease symptoms in Behçet's disease?

This study aims to assess the relationship between illness perception and disease course and symptoms in Behçet disease (BD). One hundred ten consecutive BD patients (F/M 50/60, mean age 38.5 ± 9.88 years) and 57 patients with Psoriasis as a disease control group (F/M 28/29, mean age 48.12 ± 15.52) are included in this cross-sectional study. Illness perception is evaluated using a revised version of the Illness Perception Questionnaire (IPQ-R). In IPQ-R, the identity score reflecting the number of symptoms is higher in BD patients with musculoskeletal involvement than the others (6.77 ± 2.91 vs. 5.08 ± 3.3, respectively, p = 0.007). The consequences score for musculoskeletal involvement (19.52 ± 7.03) and timeline (acute/chronic) score for eye involvement (26.67 ± 4.32) are also higher compared to patients without them (16.37 ± 5.82 and 22.09 ± 8.68) (p = 0.011 and p = 0.038), reflecting negative beliefs about the illness. The score of psychological attribution is higher in patients with psoriasis than BD (p = 0.039), whereas the other subgroup scores are lower in patients with psoriasis compared to those of BD (p < 0.05). This study provides a patient's perspective in the disease management process of BD using the IPQ-R questionnaire. A patient's own personal beliefs and emotional responses to their symptoms might affect the outcome measures, especially with musculoskeletal symptoms and eye involvement in BD. However, psychological attribution is found to be a prominent issue in psoriasis.

The assessment of contributing factors to oral ulcer presence in Behçet's disease: Dietary and non-dietary factors.

OBJECTIVE: The aim of this study was to assess the contributing factors for oral ulcer activity in Behçet's disease (BD). METHODS: Ninety-two patients with BD (F/M: 42/50, mean age: 38.7±10.02 years) participated in this cross-sectional study. Data regarding disease-related factors, smoking patterns, and self-reported dietary/non-dietary triggering factors for oral ulcer activity were collected by a questionnaire. Treatment protocol was categorized as mild and intensive groups associated with organ involvements. RESULTS: A mild treatment protocol was more common in females (52.4% vs 20%) than in males (p=0.002). During the last three months, the number of oral ulcers in female patients was higher in the mild treatment group (6.4±6.5) than in the intensive treatment group (3.3±4.9) (p=0.045). In patients with active oral ulcers (n=63), rate of being a non-smoker was also higher in females (86.7% vs 63.6%) than in males. Daily frequency of tooth brushing was 1.2±0.8 in patients with BD, and was higher in females (1.5±0.9 vs 0.9±0.6) (p=0.001). Stress and fatigue (78.3%) were reported as the most frequent triggering factors for oral ulcer presence in patients with BD. A total of 148 different dietary factors associated with oral ulcer presence were reported in the study. CONCLUSION: A mild treatment protocol and being a non-smoker were found to be the contributing factors associated with oral ulcer activity in patients with BD. Being motivated for oral hygiene and being non-smokers were positive health behaviors observed in females. Irrespective of gender, stress and fatigue were defined as the most common self-reported triggering factors for oral ulcer presence in BD. In addition, the roles of some dietary factors were also reported.

Assessment of the frequency of cardiovascular risk factors in patients with Takayasu's arteritis.

Objectives: The prevalence of atherosclerotic risk factors and disease in Takayasu's arteritis (TAK) has not been well defined. We aimed to assess the frequency of cardiovascular (CV) risk factors and the incidence of CV events (CVEs) in patients with TAK from two ethnically different populations. Methods: Patients with TAK followed at Mayo Clinic, Rochester, MN, USA and Marmara University, Istanbul, Turkey were included in this retrospective study. Patients with TAK were compared with age-, sex- and calendar year-matched controls from the same geographical region without TAK. The 2008 Framingham 10-year general CV risk score (FRS) was used for the evaluation of CV risk at the time of TAK incidence/index date. Results: In total, 191 patients with TAK and 191 non-TAK controls were included. Hypertension and the prevalence of lipid-lowering treatments were significantly more frequent in TAK. Prior to the incidence/index date, occurrence of CVE was significantly higher in TAK. The FRS was significantly higher in TAK compared with non-TAK at incidence/index date. The cumulative incidence of CVE was 15.4% at 10 years in TAK vs 5.8% in non-TAK; the risk of CVE was increased among patients with TAK (hazard ratio = 4.36; 95% CI: 1.25, 15.13). Conclusion: CV risk factors are more common in patients with TAK, particularly hypertension. The FRS is higher in patients with TAK at the time of diagnosis. The cumulative incidence of CVE was also significantly higher during follow-up in TAK. Our results suggest that patients with TAK should undergo careful assessment of CV risk factors, and an aggressive risk modification approach is warranted.

Limited reliability of radiographic assessment of spinal progression in ankylosing spondylitis.

Objectives: Conventional radiography is key to assessing AS-related spinal involvement and has become increasingly important given that spinal fusion may continue under biologic therapy. We aimed to compare the reliability of radiographic scoring of the spine by using different approaches to understand how different readers agree on overall scores and on individual findings. Method: Six investigators scored 68 plain radiographs of the cervical and lumbar spine of 34 patients with a 2-year interval, for erosions, sclerosis, squaring, syndesmophytes and ankyloses using the Spondyloarthritis Radiography (SPAR) module. The intraclass correlation coefficients were calculated compared with two gold standards. The reproducibility of each finding in 1632 vertebral corners and new syndesmophytes in each corner was calculated by kappa analysis and positive agreement rates. Results: The intraclass correlation coefficients mostly revealed good to excellent agreement with the gold standards (0.69-0.95). The kappa analysis showed worse agreement, being relatively higher for syndesmophytes (0.163-0.559) and ankylosis (0.48-0.95). Positive agreement rates showed that erosions were never detected at the same vertebral corner by two readers (positive agreement rate: 0%). The mean (range) positive agreement rates were 10.1% (0-27.7%) for sclerosis and 19.2% (0-59.7%) for squaring, and were higher for syndesmophytes [38.8% (21.4-62.5%)] and ankylosis [77.3% (64-95.3%)]. Conclusion: Our results show that there is a poor agreement on the presence of grade 1 lesions included in the Modified Stoke Ankylosing Spondylitis Spine Score-mostly for erosions and sclerosis-which may increase the measurement error. The currently used definitions of reliability have a risk of overestimating reproducibility.

The assessment of work productivity and activity impairment in Behçet's disease.

BACKGROUND/AIM: The aim of this study was to assess the relationships between the course of Behçet's disease (BD), disease-specific fears, and work productivity and activity impairment. MATERIALS AND METHODS: In this cross-sectional study, 110 consecutive BD patients were included. The Work Productivity and Activity Impairment questionnaire was used. RESULTS: In the group of employed patients, 30.41% had missed work during the previous week. The mean percentages of daily activity impairment were higher in patients with musculoskeletal involvement (39.81 ± 33.61%) compared to those without (23.48 ± 32.45%) (P = 0.008). A greater decrease in working hours was observed in patients with eye involvement (45.52 ± 15.29 h) compared to those without (54.15 ± 15.29 h) (P = 0.007). More of the male patients (67.8%) were afraid of losing their jobs compared to females (30%) (P = 0.000). CONCLUSION: The highest levels of lost productivity and the most severe effects on daily life are consequences of eye and musculoskeletal involvement in the study population. More effective therapeutic approaches are required to improve the working lives of patients with BD. Moreover, male patients had a higher fear of losing their jobs, suggesting a match between the expected clinical course and the predictions of BD patients.

Assessment of the New 2012 EULAR/ACR Clinical Classification Criteria for Polymyalgia Rheumatica: A Prospective Multicenter Study.

OBJECTIVE: To assess the performance of the new 2012 provisional European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) polymyalgia rheumatica (PMR) clinical classification criteria in discriminating PMR from other mimicking conditions compared with the previous 5 diagnostic criteria in a multicenter prospective study. METHODS: Patients older than 50 years, presenting with new-onset bilateral shoulder pain with elevated acute-phase reactants (APR), were assessed for the fulfillment of the new and old classification/diagnostic criteria sets for PMR. At the end of the 1-year followup, 133 patients were diagnosed with PMR (expert opinion) and 142 with non-PMR conditions [69 rheumatoid arthritis (RA)]. Discriminating capacity, sensitivity, and specificity of the criteria sets were estimated. RESULTS: Discriminating capacity of the new clinical criteria for PMR from non-PMR conditions and RA as estimated by area under the curve (AUC) were good with AUC of 0.736 and 0.781, respectively. The new criteria had a sensitivity of 89.5% and a specificity of 57.7% when tested against all non-PMR cases. When tested against all RA, seropositive RA, seronegative RA, and non-RA control patients, specificity changed to 66.7%, 100%, 20.7%, and 49.3%, respectively. Except for the Bird criteria, the 4 previous criteria had lower sensitivity and higher specificity (ranging from 83%-93%) compared with the new clinical criteria in discriminating PMR from all other controls. CONCLUSION: The new 2012 EULAR/ACR clinical classification criteria for PMR is highly sensitive; however, its ability to discriminate PMR from other inflammatory/noninflammatory shoulder conditions, especially from seronegative RA, is not adequate. Imaging and other modifications such as cutoff values for APR might increase the specificity of the criteria.

Assessment of Patients with Takayasu Arteritis in Routine Practice with Indian Takayasu Clinical Activity Score.

OBJECTIVE: To assess the Indian Takayasu Clinical Activity Score (ITAS2010) in followup of Takayasu arteritis (TA). METHODS: ITAS2010 forms were filled in prospectively (n = 144). Clinical activity was assessed with physician's global assessment (PGA) and criteria defined by Kerr, et al. RESULTS: ITAS2010 was significantly higher in patients with active disease. Total agreement between ITAS2010 and PGA was 66.4%, and between ITAS2010 and Kerr, et al was 82.8%. During followup, 14 of 15 patients showing vascular progression with imaging were categorized as having inactive disease according to ITAS2010. CONCLUSION: ITAS2010 was discriminatory for activity during the followup, but the agreement between PGA and ITAS2010 was moderate. Future work should include the incorporation of advanced vascular imaging and demonstration of ITAS2010 as a scalable measure and not simply a dichotomous measure of activity/flare versus remission.

Advances in the diagnosis, assessment and outcome of Takayasu's arteritis.

Takayasu's arteritis (TAK) is a rare, chronic large-vessel vasculitis (LVV) that predominantly affects aorta, its major branches, and the pulmonary arteries. Segmental stenosis, occlusion, dilatation, or aneurysm formation may occur in the vessel wall during the course of the disease. The vascular involvement can be shown with different imaging modalities to make the diagnosis of TAK. Conventional angiography, the gold standard method for initial diagnosis, seems to be replaced with the new imaging modalities such as magnetic resonance angiography (MRA) and (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) in recent years. The data coming from the new studies support that MRA and FDG-PET are also promising for the assessment of disease activity. Prognosis is possibly getting better with lower mortality in recent years; however, it is difficult to assess the widely different vascular intervention rates among the clinical series. Leflunomide, TNF-α antagonists, and tocilizumab are new options in patients resistant to conventional therapies. There is a clear need to develop a validated set of outcome measures for use in clinical trials of TAK. The OMERACT Vasculitis Working Group has taken on this task and aims to develop a core set of outcomes for LVV.