Background: Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges. Case Presentation: A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes. The patient was initially treated with corticosteroids and methotrexate but subsequently discontinued both treatment and follow-up. In September 2023, he presented with fever, sore throat, and elevated inflammatory markers. After screening for infections, methylprednisolone (MP) treatment was initiated because of AOSD activation. The following day, the patient was admitted to the intensive care unit due to an altered state of consciousness. Brain magnetic resonance imaging revealed brainstem involvement. Empirical treatments were initiated, including intravenous MP, and immunoglobulin therapy. Due to suspected macrophage activation syndrome (MAS), anakinra (ANA) infusion was initiated. Significant improvement was observed after the ANA infusion. Conclusion: This case highlights the complex management of severe AOSD complications, emphasizing the role of early recognition, aggressive therapy, and multidisciplinary care in improving outcomes.

ÖzAmaç: Geriatrik hasta (GH) tanımı 60 yaş ve üzeri bireyleri kapsamaktadır.Bu sebeple, romatologların bu ayrıcalıklı grubun tedavisini planlarken karşılaşabilecekleri sorunların farkında olmaları ve bu hastalarda,

Objectives: Destructive spondyloarthropathy (dSpA) occurs due to inflammatory destruction of axial joints due to beta2-microglobulin (beta2-M) accumulation in patients with end-stage renal disease (ESRD) receiving renal replacement therapy (RRT). This study aimed to investigate the frequency of dSpA in ESRD patients receiving RRT, by the diagnostic criteria sets of inflammatory back pain (IBP) and inflammatory classical SpA. Methods: A total of 140 patients were included. The data of the demographic informations, laboratory tests, clinical findings, HLA allele subgroups were collected. Sacroiliac and lateral feet X-rays and if necessary sacroiliac tomography were taken in patients with dSpA related pain. dSpA was investigated according to the Amor and ESSG criteria sets. Results: Fifty two patients were women (37%). The mean age was 51 ± 15 years. IBP and sacroiliitis were detected in 22 and 28 of the patients, respectively. There was a significant difference according to sacroiliitis and IBP in terms of CRP (p=0.028, p=0.043). dSpA were detected in 21 (15%) patients. Presence of HLA-A1 and duration of HD were determined as independent risk factors for dSpA. Conclusions: In the diagnosis of dSpA, the character and inflammatory origin of the pain and inflammatory markers should be investigated in addition to the radiological criteria.

INTRODUCTION:Long-term kidney transplantation (KT) results in patients with familial Mediterranean fever (FMF)-related amyloidosis are not well studied. This study reviewed the long-term survival outcomes of FMF patients who underwent KT. METHODS:We compared the outcomes of 31 patients who underwent (KT) for biopsy-proven amyloidosis secondary to FMF with 31 control patients (five with diabetes mellitus and 26 with nondiabetic kidney disease) undergoing KT between 1994 and 2021 at Başkent University Hospital. All data were recorded retrospectively from patients' files. RESULTS:THE MEDIAN AGE (QUARTILE DEVIATION: QD) at the time of KT in the FMF and control group were 31 (6.7) and 33 (11), respectively. The median follow-up period (QD) after KT was 108 (57) months in the FMF and 132 (72) months in the control group. In the FMF group, graft and patient survivals were 71% and 84% at 5 years and 45% and 48% at 10 years, respectively. In the control group, graft and patient survivals were 79% and 100% at 5 years and 63% and 71% at 10 years, respectively. Patient survival in the FMF group at 5 years was significantly lower than in the control group (p = .045). There was no statistically significant difference between the FMF and control groups in terms of graft and patient survival, and serum creatinine levels at 10 years. All patients were given triple immunosuppressive treatment with cyclosporine, mycophenolate mofetil, and prednisolone. Three patients received anakinra and one received canakinumab in addition to colchicine treatment. One FMF patient also underwent heart transplantation due to AA amyloidosis. Of the FMF patients, 11 died during follow-up. CONCLUSION:We have found that the long-term outcome of KT in patients with FMF amyloidosis is numerically worse but not statistically different from the control group. However, short- and long-term complications still need to be resolved.

Background During the Coronavirus disease (COVID-19) pandemic, one of the biggest concerns of rheumatologists and rheumatology patients has been whether the risk or severity of the disease will increase with immunosuppressive therapy. Some drugs have been reported to be associated with adverse Covid19 outcomes [1,2]. Cyclophosphamide (CYC) is a drug that has been used in rheumatology practice for many years. There is not enough data in the literature on the frequency or consequences of COVID-19 while receiving CYC therapy. Objectives The aim of this study is to examine the frequency and outcomes of Covid19 in patients who received CYC therapy during the Covid19 pandemic. Methods The files of patients who received CYC therapy protocol between March 2020 and March 2022 at Başkent University Faculty of Medicine Ankara Hospital, Rheumatology outpatient clinic were retrospectively reviewed. In our clinic, CYC therapy is administered as an intravenous treatment protocol of 500 mg three times every 10 days, then 500 mg every two weeks. Although the cumulative dose varies depending on the disease and the patient, it is usually planned to be at least three gram. The diagnosis of Covid 19 was made in the patients with clinically compatible radiology and SARS-CoV-2 PCR test results. Results A total of 36 patients received CYC during the specified period. CYC indications were ANCA-associated vasculitis in 12 patients, interstitial lung disease associated with undifferentiated connective tissue disease in 5 patients, SLE in 5 patients, scleroderma in 4 patients, Sjögren's syndrome in 4 patients, Behçet's disease in 1 patient, vasculitis associated with sarcoidosis in 1 patient, rheumatoid vasculitis in 1 patient, leukocytoclastic vasculitis in 1 patient, polymyositis in 1 patient and Takayasu disease in 1 patient.The median age (q1-q3) was 62 (52-68) years. Covid19 infection was detected in only 3 patients (8%) during the CYC therapy protocol. The median cumulative CYC dose for these patients was 3.5 g. One out of 3 patient was hospitalized for Covid 19 pneumonia. There was no death due to Covid19. Conclusion In this study, it has been shown that CYC therapy was safe during the Covid19 pandemic period. References [1]Samanta J, Naidu G, Deo P, Mittal S, Prasad CB, Das D, Dhir V, Sharma SK, Ramachandran R, Rathi M, Nada R, Minz RW, Jain S, Sharma A. Managing ANCA-associated vasculitis during COVID-19 pandemic: a single-center cross-sectional study. Rheumatol Int. 2022 Dec;42(12):2159-2166.[2]Singh N, Madhira V, Hu C, Olex AL, Bergquist T, Fitzgerald KC, Huling JD, Patel RC, Singh JA. Rituximab is associated with worse COVID-19 outcomes in patients with rheumatoid arthritis: A retrospective, nationally sampled cohort study from the U.S. National COVID Cohort Collaborative (N3C). Semin Arthritis Rheum. 2023 Feb;58:152149. Acknowledgements: NIL. Disclosure of Interests None Declared.

BackgroundGeriatric population (GP) includes people over 65 years of age. GP can also be divided into the 65-75 age group and >75 age group. In the last 50 years, the proportion of the elderly in the society has increased (1). Meanwhile, GP has higher frequency of rheumatological disorders. While managing the treatment of this special patient group, some physiological differences of the elderly should be considered. Elderly patients are at increased risk for adverse drug reactions as a result of age-related changes in pharmacokinetics and pharmacodynamics. Polypharmacy is contributing to increased risk of clinically significant drug interactions. In addition, alterations in cognitive functions may impair drug compliance. On the other hand, clinicians may hesitate to apply more effective treatment due to safety concerns. (2).Rheumatologists should be aware of the problems they may encounter when planning the treatment of this privileged group.ObjectivesWe reviewed rheumatology guidelines to assess rheumatology organizations’ perspective on GP.MethodsGuidelines published by EULAR, ACR and the British Society for Rheumatology (BSR) for adult patients from 1970-2022 were reviewed by two rheumatologists to assess the existence of specific recommendations for the treatment of adult patients over 65 years of age.ResultsIn total, 58 guidelines were reviewed. None of the guidelines grouped patients by age. Seven (12%) guidelines had recommendations or statements about elderly patients (Table 1). As we observe, there are no satisfactory recommendations for the GP with rheumatological diseases. The most propable reason for this result is the lack of studies in the rheumatology literature to lead to guideline recommendations.Table 1.Characteristics of guidelines reviewedNumber of guidelines (n)Guidelines with specific recommandation for GPEULAR381-2-3-4-5-6ACR116BSR971EULAR points to consider for the management of difficult-to-treatrheumatoidarthritis(recommendation 6)2. Points to consider for the development, evaluation and implementation of mobile health applications for self-management in patients with rheumaticdiseases (points to consider 8)3. 2019 update of EULAR recommendations for Vaccination in AdultPatientswithAutoimmuneInflammatoryRheumatics4. EULAR Recommendations for prevention and management of osteoporoticfractures(recommendation 6)5. EULAR Points to consider for monitoring (detection/prevention) comorbidities in inflammatoryrheumaticdiseases (point to consider 10)6. 2015 Recommendations for the management of polymyalgiarheumatica: a European League AgainstRheumatism/American College of Rheumatologycollaborativeinitiative (recommendation 9)7. BSR guidelines for the management of polymyalgiarheumatica (recommendation 6)ConclusionCurrent prominent rheumatology guidelines have insufficiently addressed the management of rheumatological diseases in GP. Additional studies ares needed to delineate specific guidelines for the management of geriatric patients with rheumatological diseases.References[1]OECD (2022), Elderlypopulation (indicator). doi: 10.1787/8d805ea1-en (Accessed on 27 January 2022)[2]Tutuncu Z, Reed G, Kremer JA. Do patients with older-onsetrheumatoidarthritisreceive less aggressivetreatment? Ann Rheum Dis. 2006 Sep;65(9):1226-9.Disclosure of InterestsNone declared

ABS TRACT Psoriatic arthritis (PsA) is a multisystemic inflammatory condition that can lead to disability and impaired quality of life. Anti-tumor necrosis factors (TNFs) are approved drugs for PsA with good control of symptoms and inhibition of disease progression. Latent tuberculosis infection reactivation is one of the important complications observed after treatment with TNF- α inhibitors. Here we present a case of PsA that was treated with 2 different types of anti-TNF drugs who was diagnosed with tuberculosis lymphadenitis even though receiving a 9-month preventive therapy. This case report emphasizes the importance of close follow-up and careful examination of the patients with rheumatological diseases using anti-TNF treatment.

Background To investigate the prevalence of radiographic ischial entheseal lesions in patients with psoriatic arthritis (PsA) compared to patients with rheumatoid arthritis (RA). Patients and Methods Thirty-eight patients with PsA and 46 patients with RA were included. Anteroposterior radiographs of the pelvis and lateral foot were evaluated for entheseal lesions. The following entheseal sites were reviewed: os ischium, bilateral Achilles tendon and inferior calcaneus. Abnormalities such as cortical erosions and enthesophytes (irregular bony proliferation) were recorded. Results The frequency of enthesopathic changes in the ischial region was found to be statistically significantly higher in PsA patients compared with RA patients (50 and 28.3%, respectively, p=0.04). Enthesopathic changes of the calcaneus and Achilles tendon also occurred more frequently in PsA patients than in RA patients. Conclusion Radiographic entheseal lesions in the ischial region are more prevalent in PsA patients compared with RA patients with symptoms in that region. Furthermore, such enthesopathic changes in the ischium are observed as frequently as changes in the Achilles tendon. These findings regarding structural entheseal lesions in the pelvic region contribute to the knowledge of entheseal involvement in PsA.

Purpose: Methotrexate (MTX) is still the main treatment option for rheumatoid arthritis (RA). There is no consensus on drug administration routes and dosage when administered alone or in combination with other drugs. This study aims to identify the effects of early administration of a combination therapy containing high dose subcutaneous (SC) MTX on RA management. Materials and Methods: Forty-five patients with RA who newly diagnosed were divided into two groups randomly. The patients who took 12.5 mg SC MTX per week in the first 4 weeks defined as a "low dose group". The patients who took 25 mg SC MTX per week in the first 4 weeks defined as a "high dose group". Then, patients of both groups continued with 12.5 mg oral MTX per week. Clinical and laboratory findings, disease activity scores and response rates of the patients were recorded at the beginning, 3rd months and 6th months. Results: There was no significant difference between the two groups at 3rd month’s values. There were statistically significant improvements at 6th month’s values. Values were as follows in low and high dose groups: mean DAS28-CRP (3.5 vs 2.7), VAS pain score (3.3 vs 1.6), and TJC28 (3 vs 1.5), respectively. Conclusion: Early administration of high-dose SC MTX effectively controls disease activity and increases the quality of life in RA patients.

Objective: Fibromyalgia syndrome (FMS) is a chronic disorder characterized by widespread, unexplained pain in the muscles, including the head, neck, and sides of the hips, and fatigue. We aimed to evaluate the familiarity of physical medicine and rehabilitation and rheumatology physicians with fibromyalgia syndrome (FMS) in Turkey by means of a survey and to determine if these physician groups followed the 1990 FMS diagnostic criteria and 2010 FMS classification criteria for diagnosis. Material and Methods: The survey questions consisted of two parts; the first part consisted of 10 questions about demographics and professional experience, as well as the number of patients who had been diagnosed, treated, and followed up with in the prior 3 months by physicians. The second part consisted of 15 questions about perspectives on the 1990 FMS diagnostic criteria and 2010 FMS classification criteria. Results: One hundred and seventy one physicians participated in this survey. The majority of physicians 105 (99.1%) from physical medicine and rehabilitation and 59 (90.8%) rheumatologists could diagnose FMS. The rate of diagnosis and the rate of follow-up for FMS patients were significantly higher with physical medicine and rehabilitation specialists than with rheumatologists (p= 0.013 and p = 0.000; respectively) and were statistically significant. Conclusion: Differences in the awareness and descriptions of as well as approaches to FMS by physical medicine rehabilitation physicians and rheumatologists were examined in this study.

Background and aim This study aimed to determine the frequency of relapse, the risk factors for relapse, and the correlation of relapse with immunosuppressive regimens in patients with granulomatosis polyangiitis (GPA). Materials and methods The demographic characteristics, the clinical, laboratory, and radiological findings, the immunosuppressive treatment regimens, and the remission and relapse rates of 50 patients with GPA were obtained retrospectively from medical records. Results : The mean relapse-free survival rates at years 1, 3, and 5 were 82%, 60%, and 50%, respectively. Increased relapse rates were observed in patients who had cavitary lung lesions (52.2% vs. 22.2%, p = 0.04) and in those who had elevated serum creatinine levels (1.8 vs. 0.9, p = 0.00). The patients received two different types of remission induction therapies; 36% of them received the combination therapy involving cyclophosphamide (CYC) and rituximab (RTX), and 62% received CYC alone. Relapse was observed in 22.3% of the patients who received the combination remission induction therapy and in 61.3% of the patients who received CYC alone (P = 0.003). Conclusion An increased risk of relapse was observed in patients who had cavitary lung lesions and in those who had elevated serum creatinine levels. The combined use of RTX and CYC for the remission therapy in GPA reduced the relapse rates compared with the use of CYC alone

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare type of ANCA associated Vasculitis (AAVs). Cyclophosphamide (CYC) is generally recommended for the induction of remission in life/organ threatening AAVs in combination with glucocorticoids. However, due to its rarity, randomized controlled trials regarding the efficacy of treatment modalities in EGPA are hard to perform. Therefore, the level of evidence for the use of CYC in the treatment of EGPA is lower when compared to other AAVs (1). Objectives: The aim of this study is to investigate common therapeutic agents used for the treatment of patients with EGPA. Methods: Medical records of patients who were followed-up with the diagnosis of EGPA between 2007-2020, in rheumatology clinics of Ankara and Adana Hospitals of Başkent University, were analyzed retrospectively. Treatment outcomes were assessed. Results: Records of 11 patients (six females) were analyzed. The median age was 47 (19-77) years. The median follow-up time of the patients was 24 (9-156) months. Six patients were diagnosed with asthma. The median time between the diagnosis of asthma and EGPA was 4.5 (1-3) years. Five patients had tissue biopsies. Biopsy locations were terminal ileum, lung, myocardium and nerve. The most common forms of involvement were asthma, eosinophilic pneumonia and / or nodule, cardiovascular involvement, mononoritis multiplex, vasculitic skin rash, arthritis and bowel involvement, respectively. P-ANCA was positive in 8 patients. Three patients had myocarditis and cardiomyopathy, and two patients had isolated valve problems. The median BVAS value at the time of diagnosis and the third month of treatment was 17 (6-27) and 4 (2-7), respectively. Nine patients used oral 1mg/ kg methylprednisolone (MP) and 500mg CYC every two weeks as an induction therapy. The cumulative median CYC dose was 4.5 g (1.5-8). Neither of the patients developed CYC related side effects. MP was tapered to 2 mg in five patients, and was quited in two patients. Azathioprine (AZA) was used in remission treatment following CYC therapy. Rituximab (RTX) therapy 1 g twice, 2 weeks apart was initiated in two patients due to unresponsiveness to CYC. While RTX was effective in one patient, newly developed renal involvement was detected after the third cycle of RTX therapy in other patient. Two patients had pregnancy plan therefore they used AZA plus MP as induction. A patient had mycophenolate mofetil plus MP due to AZA allergy. All patients are currently in remission except one patient. Conclusion: In seven out of 11 EGPA patients, long-term remission was achieved with CYC treatment. CYC appears to be an effective and inexpensive method of first-line treatment for organ threatening EGPA. References: [1]Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016 Sep;75(9):1583-94. Disclosure of Interests: None declared

BACKGROUNDRheumatoid arthritis (RA) is one of the most common chronic inflammatory diseases. It mainly involves the joints and also affects directly or indirectly nearly all organ systems. The question whether RA causes exocrine pancreatic disfunction remains unclear. The purpose of this study is to evaluate whether or not RA contributes to pancreatic exocrine insufficiency. This was done by ruling out seconder Sjögren's syndrome (SjS) by using Schirmer's test.METHODSA total of 60 patients (20 RA, 20 RA + SjS and 20 SjS) and 20 healthy volunteers were included in the study. Patients with RA who had not undergonethe Schirmer's test in the last 6 months and all healthy volunteers included in the study underwent the Schirmer's test at an outpatient clinic. Random fecal samples were taken from all participants and fecal pancreatic elastase was measured to evaluate pancreatic exocrine functions.RESULTSIn the study, a statistically significant difference was found between the control group,SjS and RA+SjS groups. But there was no significant difference between the control group and RA group. In RA group, fecal elastase levels were statistically significantly higher compared to the SjS group. But there was no significant difference between RA+SjS and SjS groups.CONCLUSIONFecal elastase significantly decreased in SjS compared to the normal population while pancreatic exocrine functions are considered to be impaired in SjS. There are also impaired pancreatic exocrine functions in the secondary SjS associated with RA. Consequently, pancreatic exocrine dysfunction, which can be seen in patients with RA, may be thought to be caused by secondary SjS associated with RA (Tab. 6, Fig. 1, Ref. 19).

Behcet's disease is a chronic multisystemic disease with remissions and relapses. Several studies have shown that immune mechanisms play an important role in the development of the disease. In order to assess the association of disease activity with IL-17A/F, IL-23, IL-12/23 (p40) and IL-35 expression, we aimed to investigate production of these cytokines in peripheral blood mononuclear cells (PBMCs) from Behcet's patients and normal controls. Furthermore, we included Systemic Lupus Erythematosus (SLE) as disease control to evaluate the specificity of our data for immunopathogenesis of BD. Totally 15 active, 15 inactive Behcet's patients, 12 active and 12 inactive SLE patients and 12 healthy volunteers were enrolled in the study. Peripheral blood mononuclear cells were separated, lymphocyte cultures were performed and IL-17A/F, IL-12/23 p(40), IL-23, IL-35 cytokine levels were measured by ELISA in culture supernatants in the presence or absence of phytohemagglutinin (PHA) on time-dependent manner. IL-17 A/F levels increased parallel to IL-23 levels in Behcet's and SLE patients. Compared to healthy controls, IL-17 A/F levels were higher in active Behcet's and SLE patients; on the contrary, levels of IL-35 were lower. IL-17A/F, IL-12/23 (p40) and IL-23 levels were detectable most frequently in active Behcet's patients followed by active SLE patients. Our results indicate that IL-17 A/F, IL-23 and IL-12/23 (p40) may play role in the immunopathogenesis of BD so as Th17 and Th1 cell responses. Since IL-35 levels were lower in active Behcet's patients compared to inactive patients and healthy controls, there may be a plasticity between Th17 and Treg cells according to the state of disease activity.

Objectives: To investigate the association between endothelial progenitor cells (EPCs) and Takayasu arteritis (TA). Subjects andMethods: A total of 39 subjects were included in this study: 12 subjects had been diagnosed with active TA, 11 had active Behçet disease (BD), and 16 were healthy controls. The EPCs, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels of all the subjects were measured. MedCalc 15.8 software (MedCalc, Belgium) was used for all statistical analyses. Results: The level of EPCs was higher in TA patients (4.25 ± 2.56) than in the BD group (2.27 ± 2.0) and the healthy controls (2.12 ± 1.2) (p = 0.015). TA patients with acrotism (n = 4) had higher levels of EPCs compared to TA patients without acrotism (n = 8) (6.50 ± 1.73 vs. 3.12 ± 2.16, p = 0.02). A positive correlation was found between EPCs and the ESR (r = 0.723, p = 0.0079) and between EPCs and CRP in patients with TA (r = 0.769, p < 0.0034). Conclusion: High levels of circulating EPCs were correlated with the CRP level and the ESR in patients with TA. These cells could be a marker for acrotism and inflammation in patients with TA.

OBJECTIVES:To investigate corneal, scleral, choroidal, and foveal thicknesses in female patients with rheumatoid arthritis (RA) and compare them with healthy subjects.MATERIALS AND METHODS:This prospective study included consecutive female patients diagnosed with RA and healthy subjects. Corneal, scleral, choroidal, and retinal (foveal) thicknesses were obtained by using optical coherence tomography and a comparison was performed between groups for all outcome measures.RESULTS:Thirty-six eyes of 36 female patients diagnosed with RA (group 1) and 36 eyes of 36 healthy female volunteers (group 2) were included. Mean corneal, scleral, choroidal thicknesses and retinal thickness at the fovea of group 1 were 543.3±33.7 µm, 343.7±42.2 µm, 214.6±50, and 213.5±18.9 µm, respectively; in group 2, these values were 549.9±29.6 μm, 420.9±42.4 μm, 206.4±41.9 μm, and 222±15.5 μm, respectively. The comparison between group 1 and 2 with respect to corneal, choroidal, and foveal thicknesses did not reveal statistical significant differences (p>0.05). On the contrary, there was a statistically significant difference with respect to scleral thickness between the groups, with the RA patients demonstrating a thinner scleral layer (p<0.001).CONCLUSION:Female patients with RA seem to demonstrate statistically significant scleral thinning when compared with healthy subjects, while there was no difference concerning corneal, choroidal, and foveal thickness.