Kenan Aksu

Objectives: Behçet's disease (BD) is a chronic, multisystem immune‐mediated vasculitis. A significant number of patients with BD have inflammatory comorbid (IC) disease that can affect the clinical course of the disease. In the current literature, there is no study which investigated IC disease in BD. It was aimed to show the prevalence and clinical spectrum of IC disease in patients with BD. Methods: A total of 1591 patients diagnosed with BD and followed at five tertiary healthcare facilities were enrolled in the study. Patients were divided according to the presence of IC disease. Demographic characteristics, clinical manifestations, and laboratory parameters were systematically noted from medical records. The activity of the disease was evaluated by Behçet's Disease Current Activity Form (BDCAF). Results: Inflammatory comorbid diseases were identified in 246 (15.3%) patients. The most common comorbidities were axial spondyloarthritis ( n = 115, 7.2%), familial Mediterranean fever (FMF) ( n = 38, 2.4%), and hidradenitis suppurativa ( n = 37, 2.3%). Patients with IC disease had a greater use of anti‐TNF treatment ( p = 0.03). In the multivariate analysis, older age, and longer disease duration were found to be significantly associated with IC disease in BD ( p < 0.05). Conclusion: BD can be associated with other inflammatory conditions. Furthermore, patients with inflammatory comorbidities were more likely to receive anti‐TNF therapy, although this may partly reflect treatment indications for the comorbid conditions themselves. Therefore, clinicians should remain vigilant for inflammatory comorbidities, as their recognition may have implications for therapeutic decision‐making.

Background/aim: In Behçet’s disease (BD), arterial involvement represents a significant clinical challenge associated with serious complications. The present study evaluates the characteristic features, affected vessels, lesion types, treatments, and outcomes in BD patients with nonpulmonary arterial involvement. Materials and methods: Included in this retrospective study were 65 BD patients whose interventions and clinical courses were accessed from medical records. The treatments administered included immunosuppressive (IS) therapies and surgical/endovascular procedures. Fisher’s exact and t-test/Mann–Whitney U test were used for statistical analysis. Results: Fifty-nine of the patients (90.8%) were male. The median (Q1–Q3) follow-up was 4 (2–12.8) years. The most commonly affected vessels were the aorta (n = 21, 32.3%) and the femoral artery (n = 19, 29.2%). Aneurysm (n = 50, 63.2%) and thrombosis (n = 18, 22.7%) were the predominant lesions; 14 (21.5%) patients experienced arterial involvement while receiving IS therapy; and 11 (16.9%) had cardiac involvement. Cyclophosphamide and azathioprine were the most commonly used IS agents. Among the 39 (60%) patients who underwent surgical and/or interventional procedures, 32 (82%) had successful outcomes. A second arterial event was experienced by 15.3% of the sample during follow-up, and a third by 3%. Active disease was noted in nine (13.8%) patients at the time of their last visit, and of the six (9.2%) that died, most had both arterial and cardiac involvement. Conclusion: Arterial involvement in BD mostly presents with aneurysms, especially in men. Surgical interventions in addition to IS therapies are required in approximately 50% of cases. As can be understood from the 15.3% relapse rate in the present study, arterial events are not uncommon, underlining the need for long-term monitoring and sustained IS treatment. Of the 9.2% of cases who died, most had both arterial and cardiac involvement.

Background/aim: Emerging evidence suggests that dysregulated angiogenesis may contribute to the pathogenesis of Behçet’s disease (BD). This study primarily aimed to investigate serum angiopoietin-1 (Ang-1) and angiopoietin-2 (Ang-2) levels in Turkish patients with BD and compare them with healthy controls. The secondary aims were to compare angiopoietin levels based on clinical manifestations, disease activity, and medication use and to investigate their correlations with age, disease duration, and inflammatory markers. Materials and methods: This cross-sectional study included 56 consecutive patients with BD, diagnosed according to International Study Group criteria, and 56 age- and sex-matched healthy controls. Physical characteristics, clinical manifestations, time since symptom onset, time since diagnosis, medications, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels were recorded. Serum Ang-1 and Ang-2 levels were measured by enzyme-linked immunosorbent assay. Comparisons between the two groups were analyzed using the Student t-test or Mann–Whitney U test, and correlations were assessed using Pearson or Spearman correlation coefficients, depending on the data distribution. Results: Serum Ang-1 and Ang-2 levels were significantly higher in patients with BD compared to healthy controls (p < 0.001 and p = 0.031, respectively). Patients with genital ulcers had significantly lower serum Ang-1 levels compared to those without (p = 0.029). Additionally, Ang-2 levels showed moderate positive correlations with CRP (r = 0.518, p < 0.001) and ESR (r = 0.459, p < 0.001). Conclusion: The elevated expression of Ang-1 and Ang-2 in Turkish patients with BD supports the growing evidence of their possible roles in BD pathogenesis.

Objectives: Takayasu arteritis (TAK) may lead to arterial stenosis/occlusion and aneurysms, occasionally requiring vascular interventions. We evaluated outcomes, restenosis, and complications after vascular interventions in TAK and explored factors associated with restenosis. Methods: We retrospectively included TAK patients who underwent extracardiac endovascular or surgical vascular interventions before or after TAK diagnosis across eight tertiary centers (1995–2024). Indications, disease activity status at the time of intervention, procedural characteristics, early/late outcomes, major complications, and restenosis were recorded. Restenosis was defined as > 70% stenosis on follow‐up imaging performed ≥ 3 months after the procedure. Restenosis analyses were restricted to steno‐occlusive lesions. Results: We included 119 patients with 201 treated lesions (185 steno‐occlusive lesions; 16 aneurysms/dissections). Of all procedures, 61.2% ( n = 123) were performed after TAK diagnosis under immunosuppressive therapy. Follow‐up imaging was available for 170/185 steno‐occlusive procedures, and restenosis status was evaluable in 168 procedures; restenosis occurred in 82/168 (48.8%) with a median time to restenosis of 19.5 months. In exploratory Kaplan–Meier analysis, 1‐, 3‐, and 5‐year restenosis‐free patency rates were 76.8%, 63.6%, and 56.5%, respectively. In analyses restricted to post‐diagnosis steno‐occlusive procedures, crude restenosis was significantly less frequent under biologic therapy than without biologic therapy (30.8% vs. 53.1%; Fisher's exact p = 0.040); however, biologic therapy was not associated with time to restenosis in Cox regression (HR 1.01, 95% CI 0.51–1.99; p = 0.97) or patient‐clustered Cox analysis (HR 1.01, 95% CI 0.47–2.18; p = 0.98). Major complication rates were 3.9% for endovascular procedures and 12.5% for surgical procedures. Overall mortality was 11.8%, with two deaths attributed to procedure‐related complications. Conclusions: Vascular interventions in selected patients with Takayasu arteritis were associated with acceptable major complication rates. Among post‐diagnosis steno‐occlusive procedures, biologic therapy was associated with lower restenosis rates in crude analyses; however, this association was not sustained after accounting for restenosis timing. Therefore, the potential role of biologic therapy in improving post‐interventional vascular outcomes should be interpreted cautiously and requires confirmation in prospective studies with standardized imaging surveillance.

PurposeTo report demographic characteristics and ophthalmological manifestations of patients with granulomatosis with polyangiitis (GPA) in a tertiary eye care center in Turkey.MethodsMedical records of patients with GPA-related ocular manifestations evaluated between 2013 and 2023 were included. Demographic and clinical characteristics of the patients including nature of systemic involvement, ophthalmologic symptoms and signs, laboratory investigations and treatment modality were reviewed.ResultsTwelve eyes of 10 patients (5 female/5 male) were included. The mean age was 57.2 +/- 12.2 (35-71) years. Five (50%) patients were already diagnosed with GPA. Ocular involvement was the first manifestation of GPA in 3 patients. The remaining 2 patients had simultaneous systemic and ocular symptoms at presentation. Conjunctival hyperemia (9 eyes) and pain (7 eyes) were the most frequent presenting symptoms followed by blurred vision (3 eyes). The frequencies of ocular manifestations were as follows:episcleritis (3 eyes), isolated peripheral ulcerative keratitis (PUK) (3 eyes), scleritis (3 eyes), simultaneous PUK and scleritis (2 eyes) and periorbital mass (1 eye).ConclusionOphthalmological manifestations can be the initial findings in GPA. Since GPA can affect different structures of the eye, it sometimes might be challenging for ophthalmologists. Therefore, it is crucial for ophthalmologists to be well-informed about GPA-related ocular findings and to have a high index of suspicion for GPA. Although PUK associated with scleritis is highly suggestive for GPA, isolated cases of PUK or scleritis can be seen in GPA. Therefore, it is important to adopt a multidisciplinary approach, consider GPA in differential diagnosis, and benefit from accurate diagnostic tests.

Objective: Metabolic syndrome (MetS) is one of the preventable risk factors for cardiovascular disease (CVD). The aim of this study was to investigate the effect of MetS on CVD and cumulative organ damage in a multi‐center, large cohort of patients with Takayasu arteritis (TAK).Methods: This is a cross‐sectional study involving 192 consecutive TAK patients from seven tertiary rheumatology centers in Turkey. Clinical data of TAK patients fulfilling the 1990 American College of Rheumatology classification criteria were collected from medical records. They were evaluated for risk factors of CVD, disease activity, damage, and MetS at their last visits.Results: A total of 192 consecutive TAK patients were included in this study. One hundred and fifty‐eight (82%) were female, the mean age was 43.3 ± 13 years, and mean disease duration was 13.5 ± 9.3 years. MetS was detected in 50 (26%) of the patients and CVD was detected in 28 (14.6%). The presence of MetS was detected as an independent risk factor for CVD (P < 0.001). In addition, the mean vasculitis damage index of the group with MetS was significantly higher than in the other patients (4.5 ± 3.3 vs 3.2 ± 2.2, respectively, P = 0.004).Conclusion: The presence of MetS in TAK is associated with increased CVD and disease damage. Awareness and management of MetS can improve disease prognosis in patients with TAK.

Background: This study aimed to examine fetomaternal outcomes in pregnant women in a large Turkish Takayasu arteritis (TAK) cohort and to evaluate the effects of pregnancy on the disease in those patients.Methods: This is a cohort study involving 296 pregnancies of 112 TAK patients from 8 tertiary rheumatology centers in Turkey. Pregnancies were divided into 2 groups as pre‐d (before disease onset) and post‐d (after disease onset). In addition, post‐d pregnancies were further divided into 2 subgroups according to fetomaternal complications (FMC) development status. Finally, patients were grouped into those with and without a history of pregnancy after disease onset.Results: In post‐d pregnancies, rates of worsening hypertension, new‐onset hypertension, and preeclampsia were higher than in pre‐d pregnancies (0.9% vs 16%, P < .001, 0.5% vs 5.3%, P = .012, and 0% vs 4%, P = .013, respectively). Patients with FMC were more likely to have renal artery involvement (65% vs 21%, P = .003). The patients who had post‐d were younger, had longer disease duration, and had more relapses number than other patients (P < .001, P = .028, P = .016, respectively). Vasculitis Damage Index (VDI) results were similar in patients with or without post‐d pregnancies.Conclusion: Pregnancies after disease onset were found to be associated with HT and preeclampsia/eclampsia. HT‐related FMCs are increased in TAK, and patients with renal artery involvement are at higher risk. The number of relapses increases in patients who become pregnant after disease onset, but pregnancy was not an independent risk factor for relapse. Pregnancy after the onset of disease had no negative effect on VDI.

The aim of the present study was to examine the effects of age on mucocutaneous activity by using moderation analysis in Behçet’s syndrome (BS). In this cross‐sectional study, 887 BS patients (female : male, 481:406; mean age, 38.4 ± 10.9 years) followed in 13 tertiary centers in Turkey were included. Mucocutaneous activity was evaluated by using the Mucocutaneous Index (MI) according to sex and disease course. Moderation analysis was performed to test the effect of age on mucocutaneous activity. A moderator variable is a third variable and affects the relationship between independent and outcome variables. Age was chosen as a potential moderator variable (interaction effect), MI score as the outcome variable and sex as an independent variable in the analysis. The moderation analysis tested the effects of age in three steps: whole BS patient group, patients without systemic involvement and those with systemic involvement. The moderation model was only significant in BS patients with systemic involvement (P = 0.0351), and a significant relationship was observed between female sex and MI score (P = 0.0156). In addition, the interaction plot showed that female patients had increased MI scores compared with male patients, especially in the 28‐year‐old age group (P = 0.0067). Moreover, major organ involvement was newly diagnosed in the majority of these young female BS patients. Our results suggest that the relationship between sex and mucocutaneous activity was moderated by age in the systemic involvement group. Also, increased mucocutaneous activity may be associated with new major organ involvement in young female BS patients with systemic involvement.

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 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.

Objective: Pulmonary hypertension (PH) may occur in Takayasu arteritis (TA), mostly due to pulmonary arteritis, but also due to left heart disease and/or chronic thromboembolism (CTE). Using transthoracic echocardiography (TTE), we investigated the frequency of PH caused by pulmonary arteritis. Methods: This cross-sectional study included 70 patients with TA fulfilling the 1990 ACR criteria, 68 healthy controls, and 67 patients with systemic sclerosis (SSc) fulfilling the 1980 ACR criteria representing the disease control group. Patients with severe left heart disease or CTE were excluded. The ESC-ERS guideline definition was considered for diagnosis of PH. Results: The mean systolic pulmonary artery pressure (SPAP) values in TA, SSc, and healthy control groups were 20.93±6.06, 31.57±12.75, and 18.88±5.39 mmHg, respectively. While the SPAP values were similar between TA and healthy groups, the SPAP values in the SSc group were significantly higher than in other groups. Based on conventional and/or magnetic resonance angiography findings, pulmonary arteritis was present in 4 out of 70 TA patients; however, PH was not detected in any patients with TA, including those with pulmonary arteritis. Conclusion: The TTE findings suggested that the frequency of PH was not increased in TA. However, a low frequency of pulmonary arteritis in our series might have affected our results. Cite this article as: Kalfa M, Emmungil H, Musayev O, Soysal Gündüz Ö, Yılmaz Z, İnal V, et al. Frequency of pulmonary hypertension in transthoracic echocardiography screening is not increased in Takayasu arteritis: experience from a single center in Turkey. Eur J Rheumatol 2018; 5: 249-53.