Vascular calcification in Takayasu arteritis: relationship with metabolic syndrome, left ventricular mass index, and intima-media thickness.

OBJECTIVE: The present study aims to determine the frequency of vascular calcification in Takayasu arteritis (TA) and the risk factors for it and to evaluate its relation with atherosclerotic predictors such as metabolic syndrome (MS), left ventricular mass index (LVMI) and carotid intima-media thickness (CIMT). METHODS: A cross-sectional study was conducted in patients with TA; MS was defined according to the US National Cholesterol Education Program/Adult Treatment Panel III (NCEP/ATP III) criteria. The study included 49 TA patients (22 with MS, 27 without MS) and 31 healthy controls (HCs). Non-contrast computed tomography measured calcification in coronary arteries, aorta, and branches. RESULTS: Forty-seven patients (95.9%) were female and mean age was 33.45 ± 8.53 years. Total calcification score (mean ± SD; 5223.9 ± 18041.1 AU vs. 35.87 ± 72.70 AU (p = 0.05)), CIMT, and LVMI were found to be significantly higher in TA patients than HCs (p < 0.05). While there was no significant difference between the total calcification score of MS (+) TA patients and MS (-) TA patients, in both patient groups, the total calcification score was found to be significantly higher than HCs. MS (+) and MS (-) groups were found to have significantly higher CIMT and LVMI values than the control group, in addition, MS (+) patients were found to have significantly higher LVMI and CIMT values than MS (-) group (p < 0.05). CONCLUSION: Vascular calcification, CIMT, and LVMI are elevated in all TA patients, with greater impact in the presence of MS.

Assessment and comparison of the 2023 ACR/EULAR APS criteria with the revised Sapporo criteria.

OBJECTIVE: To analyze antiphospholipid antibody (aPL)-positive patients using the 2023 American College of Rheumatology/The European Alliance of Associations for Rheumatology (ACR/EULAR) antiphospholipid syndrome (APS) classification criteria and compare the revised Sapporo criteria and the 2023 ACR/EULAR criteria and evaluate whether the 2023 ACR/EULAR criteria provide added value over the revised Sapporo criteria. METHODS: In this descriptive study, 94 aPL-positive patients (with or without APS diagnosis) were identified from two hospital-based registries (Gazi and Hacettepe University). Patients were classified into four groups to compare both criteria sets. These four groups are as follows: (1) patients classified with only the revised Sapporo criteria; (2) patients classified with only the 2023 ACR/EULAR APS criteria; (3) patients classified with both two criteria sets; and (4) patients classified with neither two criteria set. RESULTS: Of the 94 patients, 11 were classified with only the revised Sapporo criteria; one with only the 2023 ACR/EULAR APS criteria; 52 with both criteria sets; and 30 with neither set of criteria. For these 94 patients, the operating characteristics of the 2023 ACR/EULAR APS criteria, using the revised Sapporo criteria as the gold standard, the 2023 ACR/EULAR APS entry criteria demonstrated 100% sensitivity, and the 2023 ACR/EULAR APS classification criteria demonstrated 98% specificity and 82.5% sensitivity. CONCLUSION: The study emphasizes the importance of recognizing differences in clinical manifestations, such as early pregnancy loss without severe preeclampsia (PEC) and/or severe placental insufficiency (PI) and calls for a nuanced discussion on anticardiolipin (aCL) and anti-beta 2-glycoprotein-I (anti-ß2GPI) immunoglobulin G (IgG) cutoff values.

Calcineurin inhibitors experience in unplanned pregnancies with active lupus disease: A retrospective observational study.

OBJECTIVES: To evaluate the use of calcineurin inhibitors (CNIs), specifically tacrolimus, in unplanned pregnancies with active lupus disease among patients with systemic lupus erythematosus (SLE). MATERIALS AND METHODS: The study includes data from pregnancies in women diagnosed with SLE at Gazi University Hospital in Ankara, Türkiye, between January 2010 and July 2022. The study categorized pregnancies into planned and unplanned groups based on lupus nephritis presence, emphasizing the need for inactive lupus disease for at least 6 months before attempting conception in planned pregnancies. The outcomes of pregnancies involving CNIs, particularly tacrolimus, were assessed. RESULTS: In our cohort comprising 632 SLE patients, 39 individuals reported 42 pregnancies. Among the 42 pregnancies, 14 have a history of lupus nephritis. We observed that 8 of 14 patients with a history of lupus nephritis had unplanned pregnancies. Three patients used cyclosporine and 2 used tacrolimus during their pregnancy; their pregnancies were completely healthy, and no lupus flare was observed during their pregnancies. The pregnancy of 2 patients who used azathioprine and 1 last patient who used no immunosuppressive treatment ended in abortion. CONCLUSION: This study reveals that tacrolimus can be effectively used in unplanned pregnancies with active lupus disease, providing favorable maternal and fetal outcomes. The findings emphasize the importance of considering CNIs, particularly tacrolimus, in the management of SLE pregnancies, even in cases of unplanned pregnancies with a history of lupus nephritis.

Colchicine-intolerant familial mediterranean fever patients: A comparative study between different colchicine doses and IL-1 inhibitor monotherapy.

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Colchicine is the primary treatment for FMF, although some patients do not respond well or are unable to tolerate it. For these patients, the addition of interleukin-1 (IL-1) antagonists is the preferred option. However, the impact of colchicine treatment alongside the use of IL-1 antagonists remains unclear. METHODS: We recruited adult FMF patients who satisfied the Eurofever and Pediatric Rheumatology International Trials Organization classification criteria and were receiving IL-1 antagonist treatment from our FMF cohort. All the patients exhibited colchicine intolerance or resistance. As per the FMF cohort protocol, the patients were longitudinally followed up, including assessments of their C-reactive protein, erythrocyte sedimentation rate, autoinflammatory disease activity index (AIDAI), and autoinflammatory damage index (ADDI). RESULTS: Among the 125 patients (68 female and 57 male), 96 received a combination of IL-1 antagonists and the maximum tolerated dose of colchicine, whereas 29 were treated exclusively with IL-1 antagonists due to colchicine intolerance. The patients' inflammatory markers, AIDAI activity, and ADDI damage scores did not differ significantly between the two groups during the follow-up period. Notably, the drug retention rates were significantly higher in the patients treated solely with IL-1 antagonists. CONCLUSION: While the typical approach is to maintain colchicine treatment alongside the use of IL-1 antagonists, for patients who cannot tolerate effective colchicine doses, IL-1 antagonists alone may effectively control FMF disease activity.

Is compressed colchicine tablet superior to other colchicine preparations in patients with familial Mediterranean fever?

AIMS: The aim of our study is to evaluate the differences in effectiveness, dosage, and side effect profiles in the use of colchicine preparations and evaluate the superiority of compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine tablets. MATERIALS AND METHODS: Patients who were diagnosed with FMF according to the Tel Hashomer criteria, aged 18 years and older, and switched from compressed colchicine to coated colchicine tablets in the rheumatology clinic of Gazi University were identified. The daily colchicine dose and FMF attack frequency before and after switching from coated colchicine tablets to compressed colchicine tablets were compared. RESULTS: The study included 43 female (72.9%) and 16 male patients (27.1%), and the mean age was 34.54 ± 8.3 years. The number of attacks per year was significantly reduced after switching to compressed colchicine tablets, and daily colchicine doses were lower after switching to compressed colchicine tablets (1.97 ± 0.23 vs 1.78 ± 0.39 mg, p < 0.001). CONCLUSION: Compressed colchicine tablets were shown to be superior to other colchicine preparations and compressed colchicine tablets to be a useful treatment option before initiating biological agents in patients who were unresponsive to coated colchicine.

Diffuse generalized venulitis as the primary pathology of Behçet's disease: A comprehensive magnetic resonance venography study.

OBJECTIVE: Behçet's Disease (BD) is a chronic multisystem vasculitis that manifests with destructive inflammation affecting the eyes, central nervous system, and blood vessels. The pathology of vein involvement in BD is poorly characterized. Magnetic resonance (MR) venography gives more comprehensive information about deep veins and adjacent tissues. In this study, we aimed to characterize vein involvement and evaluate the diagnostic utility of MR venography in BD. METHODS: Sixty-five BD patients who fulfilled the International Study Group (ISG) criteria and 20 healthy control subjects were enrolled. Inferior vena cava (IVC), common iliac veins (CIV), external (EIV) and internal iliac veins (IVV), common femoral veins (CFV), femoral veins (FV), and greater saphenous veins (GSV) of BD patients and healthy controls were evaluated with MR venography and ultrasonography for the presence pathologic features, luminal thrombi, vessel wall changes, and perivascular abnormalities. RESULTS: 33 vascular and 32 non-vascular BD patients (mean age 39.3 ± 11.3 years and 48 [73.8%] male) were enrolled. MR venography revealed diffuse concentric thickening of the walls of IVC, CIV, EIV, IIV, CFV, FV, and GSV in BD (healthy controls vs. BD p<0.05 for all vein segments). MR venography provided additional information about veins and perivascular tissues like contrast enhancement, enlarged lymph nodes, and seminal vesicle vascularization, which were remarkably more frequent in vascular BD than non-vascular BD and healthy controls. CONCLUSION: The results of our study suggest that the involvement of the venous system is diffuse and generalized in BD, and demonstration of venulitis might help diagnose the disease.