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Introduction: We aimed to evaluate the characteristics of the patients with a rheumatologic disease who underwent TPE., Method: A single-center, retrospective study was conducted between January 2016 and June 2023., Results: Twenty patients with a median age of 51 years received a median of 6 TPE sessions. Concurrently, immunosuppressive therapy was administered to 18 (90%) of them. During the follow-up period, 9 patients (45%) died. Creatinine (p = 0.001), C-reactive protein (p = 0.001), sedimentation rate (p = 0.002), leukocyte (p = 0.003), thrombocyte (p = 0.003), and neutrophil (p = 0.003) counts was decreased after TPE. Similarly, in the ROC analysis of post TPE laboratory parameters, urea, creatinine, CRP, hemoglobin, platelets, and lymphocytes predicted mortality with areas under the curve values ranging from 0.747 to 0.869. In the Cox regression analysis for mortality, creatinine was predictive for mortality (p = 0.030), HR 1.59 (95% CI: 1.05-2.41)., Conclusion: In rheumatologic conditions, TPE is beneficial to fill the gap until the effects of immunosuppressants become apparent., (© 2024 International Society for Apheresis and Japanese Society for Apheresis.)

Background: Idiopathic granulomatous mastitis (IGM) is a benign and rare chronic inflammatory disease of the breast. Although there are various treatment modalities, an ideal treatment algorithm has not been defined., Aims: We designed this study to evaluate the clinical status, duration of remission, remission rates, and treatment algorithm in patients with IGM during a 5-year follow-up period after immunosuppressive therapy., Methods: This study was planned retrospectively in the rheumatology outpatient clinic including 63 patients with biopsy-proven IGM. Demographic characteristics, clinical findings, treatment options, and drug-free remission periods after treatment were obtained from the patient's records., Results: The mean age of female patients with IGM was 36.4 ± 6.1 years. Remission was achieved in all patients receiving immunosuppressive treatment and the median remission period was 13.9 months. After 5 years of follow-up, the median remission time without medication was 46.1 months. There was a significant improvement in the laboratory parameters and clinical findings of the patients. The most preferred immunosuppressive agent in all patients was methotrexate, and the second was azathioprine., Conclusion: During the 5-year follow-up period, no recurrence after immunosuppressive therapy was detected in IGM patients. As seen in the treatment management chart, methotrexate provided remission in the majority of patients., Competing Interests: Declarations. Ethics approval: This study was granted approval by the Ethics Committee of Health Sciences University, Gulhane Training and Research Hospital within the framework of the Declaration of Helsinki (Date: 13.09.2023 and Decision number: 2023/201). Consent to participate: Informed consent (verbal due to nature of the study) has been obtained from all participants. Consent for publication: Not applicable. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

Aim: Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection may have a more severe course in patients with underlying disease or who have had immunosuppression. In this study, it was aimed to determine the frequency of coronavirus disease 2019 (COVID-19) and the mortality rates related to COVID-19 among patients with rheumatic disease., Methods: The patients who were followed up with rheumatic disease in the rheumatology outpatient clinic in a tertiary hospital were retrospectively assessed if they had COVID-19 infection or not between March 2020 and January 2022., Results: A total of 10 682 patients were evaluated. There were 2928 (27.4%) COVID-19-positive and 7754 (72.6%) COVID-19-negative patients. The mean age of COVID-19-positive patients was 46.2 ± 14.6 years, and 65.8% were female. Forty-two (1.4%) patients died due to COVID-19. Among COVID-19-negative patients, 192 patients died. The most common rheumatic disease among patients with COVID-19 was spondyloarthritis (SpA) (30.4%). Corticosteroids were the most common treatment agent in COVID-19-positive patients regardless of mortality. Thirty-one (73.8%) patients were receiving corticosteroids, and 35 (83.3%) patients were receiving immunosuppressive agents among patients with mortality. According to the logistic regression analysis, older age, male gender, and receiving corticosteroid, hydroxychloroquine, mycophenolate mofetil, tofacitinib, rituximab, and cyclophosphamide were found to be related to increased mortality., Conclusion: COVID-19 is a serious infection and the current study emphasized that patients with rheumatic diseases had increased mortality rates, particularly in patients who were old, male, and on immunosuppressive treatments., (© 2024 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objectives: Behçet's disease (BD) is a multisystem disease and frequently occurs during the second-fourth decades of life, although disease onset may be seen at any age. This study aimed to analyze the influence of the age of onset on clinical manifestations of BD., Materials and Methods: This retrospective study analyzed two cohorts (paediatric and adult) to determine the association between the age of onset and clinical features in BD. Patients were classified into four groups to analyze the clinical characteristics according to the age of fulfilling the BD diagnostic criteria as follows: childhood onset (<12 years), adolescent onset (13-17 years), adult onset (18-39 years), and late onset (>40 years)., Results: The study included 801 patients with BD. Male predominance, pathergy test positivity, aphthosis (oral or genital), and skin and ocular involvements were higher among adult patients than paediatric patients. The presence of positive family history for BD, neuro-BD, and epididymitis were observed significantly common in the paediatric group., Conclusion: There may be differences in clinical manifestations with regard to the age of disease onset. Disease presentations may differ from adult patients, and clinicians should be aware of the high familial aggregation rate of BD, especially in countries where the disease is endemic., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state which is characterized by seizures, headache, visual disturbances, paresis, and altered mental status. Golimumab is anti-tumor necrosis factor-α inhibitor (anti-TNF-α) that can be used in the treatment of rheumatologic diseases. Here, we present a patient who had developed PRES after golimumab treatment for ankylosing spondylitis (AS). A 45-year-old female patient was admitted to the emergency service with a newly onset severe headache, loss of vision in both eyes, and two generalized tonic-clonic seizures that lasted for 3 to 4 min. The patient had the diagnoses of AS for 12 years and hypertension for 3 years and receiving golimumab and carvedilol. The patient was diagnosed with PRES based on the current clinical and diffusion cranial magnetic resonance imaging (MRI) findings. On suspicion of being the trigger of this situation, golimumab was stopped. After starting anti-convulsant therapy and controlling blood pressure, the neurological findings recovered rapidly and no seizures were seen. Control MRI images, in the first month's visit, were normal. Although chemotherapeutic agents are well-known causes of PRES, there are few reported cases with anti-TNF-α agents in the literature. To our knowledge, this is the first case that developed PRES after golimumab. Demyelinating diseases are the most frightening neurologic complication of anti-TNF-α treatment; however, PRES should come to mind in patients presenting with neurological symptoms., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Background: Idiopathic granulomatous mastitis (IGM) is a rare inflammatory breast disease, in which there is no clear established treatment algorithm. Several physicians keep away from using immunosuppressive (IS) treatments in routine clinical practice., Aims: This study aimed to evaluate the rates of drug-free remission of the patients with IGM in a period of 3-year follow-up., Methods: This retrospective study conducted with 55 biopsy-proven IGM patients, who were followed up between February, 2011, and November, 2021, in rheumatology outpatient clinic of Gulhane Training and Research Hospital. The demographic and clinical characteristics of the patients were obtained from patients' files. The 3-year follow-up data were assessed for long-term outcome analyses., Results: There were 55 female patients with a mean age of 36.8 ± 6.3 years. Fifty-four (98.1%) patients were in drug-free remission at the end of 3 years. The median duration of drug-free remission in patients receiving methotrexate (MTX), only corticosteroid (CS), and azathioprine was 19.7, 32.9, and 14.7 months, respectively. The drug-free remission duration for the patient who received cyclosporine A as IS was 28.3 months. The median duration of IS treatment was 15.8 months, and the median duration of treatment with CS and other IS combination was 6.7 months. Recurrence was observed in 4 (80%) patients without IS therapy after surgery, of whom MTX was used in 3 (75%) patients and achieved remission., Conclusions: IS agents provide high rate of prolonged drug-free remission and should be considered a part of routine medical care of the patients with IGM., (© 2023. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

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.

Objectives: Smoking has a well-established detrimental effect on the prognosis and treatment success in patients with ankylosing spondylitis. It is important to encourage and convince patients to quit smoking. We aimed to evaluate the contribution of rheumatologists to smoking cessation in patients with ankylosing spondylitis., Methods: This single-center cross-sectional study was conducted in a tertiary research hospital between March 2022 and June 2022. The data related to demographics, smoking status, duration of smoking, average number of cigarettes smoked per day, reasons for quitting smoking, and methods of quitting smoking were obtained through face-to-face interviews., Results: This study was carried out on 308 patients. A total of 102 ankylosing spondylitis patients quit smoking. Of the patients who quit smoking, 39 (38.3%) patients quit smoking with a recommendation of a rheumatologist and 29 (28.4%) patients quit because of their concerns related to ankylosing spondylitis disease. The most commonly used methods for quitting smoking were herbal supplements in 40 (39.2%) patients and medication for smoking cessation in 40 (39.2%) patients., Conclusions: It has been shown that about one-fifth of ankylosing spondylitis patients are not questioned by a rheumatologist about smoking. On the other hand, it was seen that the factor with the greatest effect on those who quit smoking was the rheumatologist. Therefore, rheumatologists should question all ankylosing spondylitis patients about smoking and encourage smokers to quit in order to achieve better outcomes in the long term., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Kaya et al.)

Objectives: Rheumatoid arthritis associated interstitial lung disease (RA-ILD) is a major concern in RA. These patients have been included in clinical trials and in the post-marketing setting of RA patients using tofacitinib. We aimed to assess the real-life efficacy and safety of tofacitinib in patients with RA-ILD., Methods: RA patients with ILD diagnosis based on the HRCT images of the lungs from eight different centres recruited to study. As a control group, RA patients without ILD under tofacitinib were included. Demographic data, patients' characteristics, available pulmonary function tests regarding RA and RA-ILD at the visit in which tofacitinib was initiated and for the last follow-up visit under tofacitinib were recorded. Reasons for tofacitinib discontinuation were also recorded. Drug retention rates were compared by log-rank test. p-value <0.05 was considered statistically significant., Results: A total of 47(42.6% male) RA patients with RA-ILD and a control group of 387 (17.8% male) patients without RA-ILD were included in analysis. After the median of 12 (9-19) months follow-up, mean FEV1%; 82.1 vs. 82.8 (pre/post-treatment, respectively, p=0.08), mean FVC%; 79.8 vs. 82.8 (pre/post-treatment, respectively, p=0.014) were stable and worsening was observed in 2/18 (11.1%) patients. Retention rates were similar (p=0.21, log-rank). In RA-ILD group, most common cause of drug discontinuation was infections (6.3 vs. 2.4 per 100 patient-years)., Conclusions: Treatment strategy of RA-ILD patients is still based on small observational studies. A high rate of discontinuation due to infections was observed in RA-ILD patients under tofacitinib; however, RA-ILD patients were older than RA patients without ILD.

Objectives: The present study aims to evaluate the relationship between Behçet's uveitis and lymphangiogenesis by determining levels of Vascular endothelial growth factor-C (VEGF-C, its receptors sVEGFR-2, sVEGFR-3 and lymphangiogenesis markers podoplanin (PDPN) and lymphatic vessel endothelial hyaluronan receptor 1(LYVE-1), and C-type lectin domain family 1 member B (CLEC2)., Materials and Methods: 55 patients with BD uveitis and 31 healthy control subjects were enrolled in the study., Results: sVEGFR-2, sVEGFR-3, VEGF-C/sVEGFR-2 ratio, PDPN and LYVE-1 levels were higher in the patient group. A positive correlation was found between LYVE-1 and hsCRP levels. PDPN had a strong predictive value for progression with a cut-off value of 2 pg/mL, with 69% sensitivity and 68% specificity (p = 0.001)., Conclusion: LYVE-1 and PDPN can be good representatives of the ongoing inflammatory processes in BD uveitis and point out that the disease can be related to lymphangiogenesis.