Background/Objectives: A limited number of previous studies have reported high rates of end-stage renal disease (ESRD) in adults with IgA vasculitis nephritis (IgAVN). Despite the high prevalence of the disease and the high rates of ESRD reported in the literature, no specific guidelines for adult patients have been established and there is no consensus on the management of the disease. This study aimed to prospectively investigate adults with IgAVN from a broad perspective. Methods: This investigation was designed as a prospective observational study and was conducted between 01.02.2022 and 01.10.2024. A total of 49 newly diagnosed adult (>18 years) patients with IgAVN were regularly followed up. At the end of the study, the renal remission rates, factors influencing remission, treatment data, treatment-related adverse events, and disease outcomes were determined. Results: The median follow-up time was 22 (IQR: 11–24) months. A total of 42 patients (87%) received immunosuppressive treatment in addition to the initial glucocorticoid treatment. Azathioprine (AZA) was the preferred (41%) first steroid-sparing agent. ESRD occurred in only one patient (2%), while a total of ten patients (20%) had an unfavorable outcome. The rate of nephrotic-range proteinuria (NRP) was significantly higher in the patients who did not achieve renal remission at the end of the 12-month follow-up period (9,7% vs. 60%; p = 0.02) and NRP was an independent risk factor for unfavorable outcomes [OR: 17.18; 95% CI: 1.31–224.95; p = 0.03]. A total of 16% of the patients developed an infection that required hospitalization during follow-up; these patients had a higher rate of IgAVN-associated acute kidney injury (62.5% vs. 22%; p = 0.02) and were significantly older (mean: 46 ± 15.3 vs. 65 ± 13.3; p = 0.002). One patient died of sepsis at 4 months and another died of a myocardial infarction at 32 months. Conclusions: These results suggest that adults with IgVAN do not have a high rate of ESRD if they receive effective immunosuppressive therapy. However, immunosuppressive therapy is associated with an increased risk of infection, particularly in the elderly. The presence of NRP is associated with lower long-term remission rates and has a predictive value for unfavorable outcomes.

Behcet’s syndrome is a variable vessel vasculitis characterized by recurrent oral and genital ulcers with concomitant skin, ocular, neurologic, gastrointestinal, and joint involvement. Herein, we present a patient who was diagnosed with Behcet’s syndrome, which with magnetic resonance angiography showed occlusion of the right subclavian artery at the level of the thoracic outlet and reverse flow in the right vertebral artery consistent with subclavian steal syndrome. In addition, partial narrowing was noted in the left renal artery. The distribution of arterial involvement resembled Takayasu’s arteritis, although the presence of mucocutaneous lesions, male gender, history of deep vein thrombosis, and HLA-B51 positivity favored a diagnosis of vasculo-Behçet’s syndrome. We treated the patient with methylprednisolone and cyclophosphamide. After the regression of vascular inflammation with immunosuppressive therapy, stenting was performed in the left renal artery. Cite this article as: Akkuzu G, Özgür DS, Karaalioğlu B, et al. Bahcet’s syndrome resembling takayasu’s arteritis with the distribution of arterial involvement: A case report and literature review. Eur J Rheumatol. 2023;10(2):62-66.

To evaluate the utility of elasticity imaging techniques, such as transient elastography (FibroScan), in assessing the liver and transplanted kidney in patients with AA amyloidosis (AA-A). This study was conducted in patients with AA-A, while patients with immunoglobulin light chain amyloidosis (AL-A), Familial Mediterranean Fever (FMF) without amyloidosis, and healthy controls (HC) were included as comparison groups for liver stiffness (LS) measurements. Additionally, kidney stiffness (KS) was measured in renal transplant recipients (RTRs) with transplants due to AA-A or other causes of chronic renal failure. LS evaluations were performed in 65 patients with AA-A, 14 with AL-A, 20 with FMF, and 27 with HC. LS (kPa) was significantly higher [median (IQR)] in patients with AA-A [6.4 (5.4)] and AL-A [9.8 (11)] compared to HC [4.7 (1.7)] (p < 0.001). However, the difference between AL-A and AA-A was not statistically significant. LS values were comparable in FMF-AA patients with [6.8 (6.6)] and without [5.7 (3.6)] liver involvement, and FMF patients without amyloidosis [7.15 (4.6)]. The median KS values were comparable in 19 AA-A and 16 disease controls. However, KS values were significantly higher in patients with recurrent amyloidosis in the transplanted kidney [29.3 (18.9)] compared to those without recurrence [10.9 (7.7)] (p = 0.003). In our study, increased liver stiffness measurements were observed in patients with AA-A, AL-A, and FMF compared to HC. Transient elastography using FibroScan appears to be a promising non-invasive tool for assessing liver involvement in AA-A and may aid in detecting recurrence of amyloidosis in transplanted kidneys. Further studies are needed to validate the utility of FibroScan in evaluating the involvement of the liver and kidneys in patients with AA-A.

Objectives: Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease affecting both genders in reproductive age. In this study, we aimed to investigate the relation between FMF and pregnancy on both maternal and fetal aspects. Material and methods In this retrospective, single-center, descriptive study we analysed total of 95 pregnancies of 40 FMF patients. Clinical and demographic data were obtained from patients’ records. To prevent recall bias, only the last pregnancy of each patient was evaluated for disease activity and use or revision of medications during pregnancy. Results: The median age of the patients at diagnosis was 22 and the first pregnancy age was 26 years. The median duration of FMF at last pregnancy was 8 (0–23) years. Eight (20%) patients had at least 1 pregnancy via assisted reproductive techniques (IVF), while 34 (85%) patients had at least 1 spontaneous pregnancy. While 32 patients were in remission (80%) before pregnancy, 8 were clinically active (20%). Improvement in clinical course and attack frequency during pregnancy was observed in 23 patients (57.5%), stable course in 10 (25.0%), and worsening in 7 (17.5%). The rate of live birth was 70.0%, abortus was 28.9%, preterm labor was 8.1%, pre-eclampsia was 5.0%, and only 1 achondroplasia as congenital fetal abnormality was observed. Conclusion: FMF did not constitute a contraindication for pregnancy. The most important obstetric problems, complications, and negative fetal outcomes in the course of pregnancy are increased IVF requirement, abortion, and cesarean rates. There is no increase in the risk of congenital malformations due to FMF itself or use of colchicine.

The aim of this study is to evaluate the baseline F18-FDG PET/CT findings of individuals diagnosed with giant cellarteritis (GCA) and to explore its association with clinical findings and classification criteria.Materials and methods: We analysed data from patients who underwent F18-FDG PET/CT scans to investigate large vessel (LV)involvement between 2010 and 2019. Only patients with a clinical diagnosis of GCA and at least 6 months of follow-up were included.We compared initial clinical features and laboratory findings based on the presence of LV vasculitis on PET/CT and the maximumstandard uptake value (SUVmax) of vascular territories.Results: Twenty-nine patients (median age at diagnosis: 70, F/M: 24/5) were included in the study. Among them, 21 patients (72.4%)presented with cranial symptoms, while 8 patients (27.5%) had isolated LV-GCA. Twenty-two patients (75.9%) met the ACR/EULAR2022 GCA classification criteria. LV vasculitis was detected on PET/CT in 23 patients (79.3%). A positive correlation was observedbetween SUVmax in the thoracic aorta and both CRP and ESR levels (r = 0.50, p = 0.026 and r = 0.63, p = 0.002, respectively). PET/CTpositive patients were found to be younger (p = 0.016) and more frequently female (p = 0.017). They also exhibited fewer headaches(56.5% vs. 100%, p = 0.04), experienced fewer flares during follow-up (p = 0.03), and had a lower cumulative glucocorticoid dose at the6th month (p = 0.036). Comparison of PET/CT-positive patients (n = 23) based on the fulfilment of the ACR/EULAR 2022 classificationcriteria revealed that patients who met these criteria were older (p = 0.02) and had significantly lower CRP levels at diagnosis (p = 0.02).Conclusion: The performance of F18-FDG PET/CT in diagnosing LV involvement in GCA is favourable, and the severity of FDGuptake in the vessel wall correlates with the acute phase response. Patients with extracranial involvement on PET/CT exhibit distinctfeatures, including a younger age and female predominance. Additionally, these patients appear to experience fewer relapses and requirelower doses of glucocorticoids. However, the clinical significance of PET/CT in patients who met ACR/EULAR classification criteria,predominantly consisting of patients with ischemic cranial symptoms, could not be determined in our study.