Serdal Ugurlu

• Istanbul University-Cerrahpaşa

Objective Assessment of the efficacy and safety of rituximab (RTX) in treating thrombocytopenia of systemic lupus erythematosus (SLE) patients unresponsive to conventional treatments, highlighting its potential as an alternative to the expensive intravenous immunoglobulin (IVIG), and investigating the optimal number of RTX cycles for complete remis...

Objectives This study investigates the impact of Familial Mediterranean Fever (FMF) and its treatment on male infertility, and the paternal effect of FMF on pregnancy outcomes or complications. Methods We enrolled 282 adult male FMF patients and excluded 102 for never attempting pregnancy. Demographic and clinical data, including MEFV mutation sta...

To investigate the fertility status, pregnancy outcomes, and disease activity during and after pregnancy in patients with juvenile idiopathic arthritis (JIA) currently being followed up at an adult rheumatology clinic. This study included 141 adult patients diagnosed with according to the International League of Associations for Rheumatology criter...

Background Spondyloenchondrodysplasia is classified as an interferonopathy resulting from recessive mutations in the ACP5 gene and manifests with various clinical features, including distinctive skeletal dysplasia, neurological abnormalities, immune dysfunction resembling systemic lupus erythematosus (SLE) and Sjogren’s syndrome. While SLE is typic...

Background Ankylosing spondylitis (AS) is a chronic inflammatory disease of the axial spine. Etanercept is a Tumor necrosis factor-alpha inhibitor (anti-TNF) that is widely used in the treatment of AS. The most common side effects of etanercept are infection, rash, and injection site reaction. Here, we reported an adverse event probably associated...

Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disorder of unknown etiology characterized by systemic inflammation, high fever, salmon-colored skin rash, arthralgia, and arthritis. Patients with AOSD may also present with elevated inflammatory markers, hyperferritinemia, anemia, leukocytosis, hepatosplenomegaly, and lymphade...

Objective Transitional care is essential to maintain the continuity of care in younger patients with rheumatic diseases. In this study, we aimed to assess the transition readiness of rheumatology patients who had already transferred from pediatric to adult care using a questionnaire. Materials and Methods We included young adult rheumatology patie...

Background Many of the familial Mediterranean fever (FMF) patients present with arthritis during attacks, which may vary in its characteristics. Aims In this study, we aimed to describe and characterise arthritis in FMF patients. Methods We used our hospital's record system to retrospectively identify FMF patients with arthritis who presented to...

To demonstrate the burden of sexual dysfunction (SD) among females with rheumatic diseases, we conducted a cross-sectional comparative study in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and Behçet’s syndrome (BS) along with suitable healthy controls (HCs). Age-matched female patients with SSc (n = 50), SLE (n = 49)...

Systemic sclerosis (SSc) poses significant challenges in clinical management, especially when complicated by scleroderma renal crisis (SRC), a rare but life-threatening manifestation. Here, we report a 41-year-old female patient with SSc who presented with SRC and concurrent thrombotic microangiopathy. Her condition persisted despite conventional t...

Familial Mediterranean fever (FMF) is a common autoinflammatory disease. The primary treatment is colchicine; however, 5-10% of patients do not respond to colchicine and are considered colchicine resistant. Colchicine resistance and disease severity are highly associated, with each used to assess and define the other. In our review, we examined the...

Objectives Treatment response may be variable across organ manifestations of Behçet syndrome (BS). We aimed to determine the frequency of de novo manifestations during adalimumab treatment. Methods We conducted a chart review of all BS patients who received adalimumab in our center between 2008 and 2023. Demographic data, reasons for initiating ad...

Analjezikler, Non-Steroid Anti-İNFLAMATUAR İlaçlar ve Kortikosteroidler Duygu KURTULUŞ Biyolojik Olmayan Hastalık Modifiye Edici Antiromatizmal ilaçlar Ali Nail DEMİR Romatolojide Biyolojik Tedavi Ajanları İsmail UÇAR İmmunsupresifler ve Diğer İlaçlar Önay GERÇİK Romatolojide İntravenöz İmmünoglobulin Uygulamaları Hüseyin Ali ÖZTÜRKAlper YILDIRIM R...

Psoriasis is a common multisystem inflammatory disease, and arthritis is an essential component of the disorder, requiring early diagnosis and prompt treatment for successful management. In this study, we aimed to investigate the relationship between nail and scalp involvement and other covariates with psoriatic arthritis (PsA). This cross-sectiona...

Familial mediterranean fever (FMF) is characterized by inflammatory attacks due to overactivation of pyrin inflammasome. This study aimed to investigate the reliability of S100A8/A9, neopterin, and matrix metalloproteinase 3 (MMP3) at monitoring subclinical inflammation and disease activity, and at differentiating FMF attacks from appendicitis, the...

Objectives Still’s disease is a rare autoinflammatory disorder characterized by systemic inflammation, fever, rash, and arthritis. The term “Still’s disease” covers the pediatric subtype systemic Juvenile Idiopathic Arthritis (sJIA) and adult-onset Still’s disease (AOSD), which affects adults. Biological drugs, including anti-interleukin-1 agents a...

This case study describes a 67-year-old female patient who presented with shortness of breath and quick exhaustion. In 2018, initial symptoms led to a diagnostic thoracic CT scan, which confirmed sarcoidosis using endobronchial ultrasound (EBUS). Despite the diagnosis, the patient did not receive treatment. A 2023 bone marrow biopsy revealed that t...

Tumor necrosis factor type 1A receptor‐associated periodic syndrome (TRAPS) and cryopyrin‐associated autoinflammatory syndrome (CAPS) are rare monogenic autoinflammatory diseases (AIDs) mainly caused by pathogenic variations in the TNFRSF1A and NLRP3 genes, respectively. Here, we describe a unique patient presenting with symptoms overlapping both T...

Several possible factors are hypothesized to trigger familial Mediterranean fever (FMF) attacks; however, there is no consensus on this matter. We aimed to identify these triggering factors and analyze their relationship with the Mediterranean fever gene mutation status. We prepared a questionnaire that included the most commonly mentioned possible...

Objective Lupus nephritis (LN) is challenging, especially in treatment-resistant cases. This retrospective study evaluates rituximab’s effectiveness in LN patients at our clinic. Methods We retrospectively analyzed LN patients who received rituximab at our clinic from 2010 to 2022. We measured key parameters before and after rituximab, including c...

Objective To highlight Mendelian inheritance in adult-onset systemic lupus erythematosus with a unique case of spondyloenchondrodysplasia-immune dysregulation and explore the potential of baricitinib as a promising treatment. Methods We present a case of spondyloenchondrodysplasia-immune dysregulation in an adult patient, born to nonconsanguineous...

Objective Systemic lupus erythematosus is a prevalent autoimmune disease, with 20 to 40 percent of cases exhibiting thrombocytopenia. Conventional treatments involve corticosteroids and disease-modifying antirheumatic drugs, yet effectively managing thrombocytopenia remains challenging.¹ This study evaluates rituximab’s efficacy, side effects, and...

Objectives Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disorder of unknown etiology characterized by systemic inflammation, high fever, salmon-colored skin rash, arthralgia, and arthritis. Patients with AOSD may also present with elevated inflammatory markers, hyperferritinemia, anemia, leukocytosis, hepatosplenomegaly, a...

Objectives: This study compared the secukinumab treatment responses and adverse effects in psoriatic arthritis patients who received secukinumab as second-line with those that received secukinumab after two or more tumor necrosis factor-alpha (TNF-α) inhibitors. Patients and methods: The retrospective study included 68 psoriatic arthritis patients...

Idiopathic recurrent pericarditis (IRP) is defined by recurring episodes of pericardial inflammation without a known cause. This study investigates the safety and efficacy of anakinra, an interleukin‑1 inhibitor, as a successful therapy for IRP in cases resistant to conventional treatment. A retrospective evaluation of patients treated at our autoi...

Introduction: Familial Mediterranean Fever (FMF) is the most common autoinflammatory disease that has mainly been treated with colchicine since 1972. A significant portion of patients do not respond to colchicine and require further treatment, mainly IL-1β antagonists such as anakinra, canakinumab and rilonacept as IL-1β has a crucial role in path...

Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disorder predominantly characterised by periodic fever, abdominal pain, and joint manifestations. It can exhibit various atypical presentations. However, cases of FMF concurrently presenting with chronic thrombosis and myositis have not been previously reported. A 41-year-old male...

Dear Editor, We read with great interest the article by Patt et al. [1] in which they aimed to evaluate the incidence and risk of osteoporosis and femoral neck fracture in patients with FMF compared with the general population. The authors concluded that patients with FMF were at increased risk of developing osteoporosis and femoral neck fractures....

Familial Mediterranean fever (FMF) is a systemic autoinflammatory disease that requires lifelong treatment and is associated with several comorbidities, including mental health disorders such as anxiety and depression. FMF and mental health necessitate further research; hence, this study aims to observe anxiety and depression and their relationship...

Dear Editor, We read the recently published systematic review by Hirahara et al. [1] with great interest. We would like to thank the authors for undertaking this comprehensive study on the association between FMF and pregnancy, adhering to the principles of evidence-based medicine. The findings of the meta-analysis part of the systematic review ind...

Objectives FMF is the most common hereditary monogenic fever syndrome marked by recurrent attacks of fever and polyserositis. Colchicine is the current recommended first-line treatment for FMF. However, a small portion of FMF patients are unresponsive or intolerant to colchicine. Anti-IL-1 agents are alternative treatment options for colchicine-res...

Deficiency of adenosine deaminase 2 (DADA2), caused by recessive mutations in the ADA2 gene, results in cutaneous or systemic vasculitis with variable clinical manifestations. There is only one other case in literature carrying both ADA2 and MEFV gene pathogenic variants. Here we report the second case that carries both ADA2 and MEFV pathogenic var...

Study design: This was a single-blinded randomized clinical trial. Introduction: Systemic sclerosis (SSc) is an autoimmune connective tissue disease that progresses with fibrosis. Patients with SSc need to be protected against epidemic diseases and provided for in terms of rehabilitation needs. Purpose of the study: To compare the effects of r...

Objectives: Digital ulcers (DUs) are associated with a significant burden in systemic sclerosis (SSc) by leading to severe pain, physical disability, and reduced quality of life. This effort aimed to develop recommendations of the Turkish Society for Rheumatology (TRD) on the management of DUs associated with SSc. Methods: In the first meeting h...

Systemic autoinflammatory diseases have always been one of the most striking and challenging aspects of the art of medicine. Among this fascinating constellation of diseases, familial Mediterranean fever (FMF) is the most common. FMF involves the reproductive system and may cause fertility problems. With the start of the interleukin (IL)-1 inhibito...

Objective: Vascular involvement is an important cause of morbidity and mortality in patients with Behçet's syndrome (BS). We aimed to survey the efficacy and safety of infliximab (IFX) in BS patients with vascular involvement followed in a dedicated tertiary center. Methods: Charts of all BS patients who used IFX for vascular involvement between...

Aim: To investigate the clinical and radiological outcomes and glucocorticoid-sparing effect of rituximab therapy in 13 patients with retroperitoneal fibrosis (RPF). Methods: We analyzed the data of both glucocorticoid-naive and glucocorticoid-resistant RPF patients who were treated with rituximab. Demographic features, positron emission tomogra...

Purpose: COVID-19, which has affected people in various ways, has also shown to cause various limitations on the lives of Scleroderma patients. The current study aims at evaluating the upper extremity functions, sleep quality, fatigue, and health-related quality of life in Scleroderma patients and to determine the factors affecting upper extremity...

Background: In this study, we aimed to investigate different types of celiac antibodies in psoriasis patients and to see if the presenceof the antibodies was associated with other variables. Methods: We included patients with plaque psoriasis who were followed up in our dermatology clinic between February 2019 and February 2021 and added a healt...

urpose. The primary aim of our study was to compare the changes in physical activity, sleep, fatigue, pain, and quality of life levels between before confinement and during confinement in patients with Behçet and FMF. The secondary aim of the present study was to determine the exercise behavior of patients with Behçet and FMF during confinement. Me...

Introduction and objectives: This study aimed to evaluate the efficacy of secukinumab (SEC) in axial spondyloarthropathy (axSpA) in anti-TNFα naïve and anti-TNFα experienced patients. It also focused on the duration of SEC treatment and its side effects. Patients and methods: The patients with axSpA treated with SEC and followed up in our outpat...

Behçet disease (BD) and familial Mediterranean fever (FMF) are two inflammatory disorders that share many features including historical background, ethnical distribution and inflammatory characteristics. Several studies suggested that BD and FMF might occur in the same individual more commonly than expected. Additionally, the pathogenic MEFV gene v...

Aim: This study aimed to show the effectiveness of interleukin (IL)-23 inhibitors in psoriatic arthritis (PsA) at weeks 12 and 24 in a real-world setting. Materials and methods: Forty-three patients with active PsA were enrolled in this study. These patients were treated with either guselkumab (n = 20) or risankizumab (n = 23). Treatment respons...

Objectives: Rheumatoid arthritis (RA) is an autoimmune disease involving synovial joints, and it is known that extra-articular manifestations that may affect the central and peripheral nervous systems may develop during its course. Rheumatoid meningitis is very rare among all neurological involvements. In this study, cases diagnosed as rheumatoid...

Objective: The presence of familial Mediterranean fever (FMF) cases without MEFV pathogenic variants led us to search for other genes' involvement in the disease development. Here, we describe the presence of genetic heterogeneity in a three-generation family with an FMF/mevalonate kinase deficiency(MKD)-overlapping phenotype without MEFV/MVK path...

Taking the responsibility for their own health should be an important goal for the patients in the health care transition process. The Transition Readiness Assessment Questionnaire (TRAQ) is an effective tool to assess the competencies of the patients in five domains, which is usually administered before transitioning to adult care. For the first t...

Introduction Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by polyserositis and arthritis attacks, which are mediated by increased plasma levels of cytokines. Our hypothesis was that the increase in specific cytokines can also lead to portal hypertension, even in the absence of overt hepatic steatosis. Methods We...

Background/Purpose: Recurrent fever and serositis are the hallmarks of Familial Mediterranean fever (FMF). Colchicine is known for yielding high success in disease control by obviating attacks; however, management of the disease may be challenging in the case of colchicine resistance. In this specific group, which consists 5-10% of FMF patients, in...

Purpose It is known that morbidity and mortality risks are higher in systemic lupus erythematosus-related (SLE) intestinal pseudo-obstruction (IPO) cases. Therefore, it is crucial to recognize and treat these cases promptly. Here, we present a case of SLE-related IPO in a 41-year-old female patient. Methods Laboratory tests and radiodiagnostic met...

Introduction and objectives This study aimed to evaluate the efficacy of secukinumab (SEC) in axial spondyloarthropathy (axSpA) in anti-TNFα naïve and anti-TNFα experienced patients. It also focused on the duration of SEC treatment and its side effects. Patients and methods The patients with axSpA treated with SEC and followed up in our outpatient...

Background: Giant cell arteritis (GCA) is a large vessel vasculitis that may cause significant morbidity in the elderly population. We aimed to evaluate presentation characteristics, treatment, and outcome in a cohort of patients with GCA diagnosed and followed in a single center. Methods: A retrospective chart review revealed 84 (41 M/43 F) reg...

It is assumed that in candidates for TNF-alpha inhibitor (TNFi) treatment, tuberculin skin test (TST) may be unreliable, since BCG vaccination causes false positive and drugs cause false negative results, favoring the use of Quantiferon or T-spot assays. However, these tests may not be readily available in all parts of the world. We aimed to determ...

Objective: To investigate markers of carotid atherosclerosis and oxidized low density lipoprotein (OxLDL) levels in patients with Familial Mediterranean Fever (FMF) who have no risk factors for cardiovascular disease. Method: It was included 44 patients (25 F/19 M; mean age: 33.5±7.5) with FMF in attack free period and gender and age matched 44 hea...

Objective A decline in the frequency of AA amyloidosis secondary to RA and infectious diseases has been reported. We aimed to determine the change in the frequency of AA amyloidosis in our Behçet’s syndrome (BS) patients and to summarize the clinical characteristics of and outcomes for our patients, and also those identified by a systematic review....

Most of the published data relate to classical forms of rheumatic diseases (RD) and information on rare inflammatory disorders such as Behçet’s syndrome (BS) and familial Mediterranean fever (FMF) is limited. We studied the frequency of side effects and disease flares after COVID-19 vaccination with either Pfizer/BioNTech or Sinovac/CoronaVac in 25...

A previously healthy 24-year-old male patient was referred to our clinic with bilateral lower extremity pain and dark urine, which developed two weeks after receiving the second dose of BNT162b2 vaccine against SARS-CoV-2. Laboratory tests indicated rhabdomyolysis. Lower extremity magnetic resonance imaging was compatible with myositis. Myositis-re...

Introduction: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by polyserositis and arthritis attacks, which are mediated by increased plasma levels of cytokines. Our hypothesis was that the increase in specific cytokines can also lead to portal hypertension, even in the absence of overt hepatic steatosis. Methods:...

Introduction: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by poly-serositis and arthritis attacks, which are mediated by increased plasma levels of cytokines. Our hypothesis was that the increase in specific cytokines can also lead to portal hypertension, even in the absence of overt hepatic steatosis. Methods: W...

Introduction: The current study aimed to investigate whether patients regularly using colchicine or hydroxychloroquine (HCQ) have an advantage of protection from COVID-19 or developing less severe disease. Methods: Patients who were taking colchicine or HCQ regularly for a rheumatic disease including Familial Mediterranean Fever, Behçet's syndro...

Giriş: • Aralık 2019’da Çin’de ortaya çıkan SARS-CoV-2 (Şiddetli Akut Solunum Sendromu – Koronavirus-2) ilişkili doğrulanmış COVID-19 olgu sayısı şubat 2022 sonu itibariyle dünya çapında 430 milyona ulaşmış, ölüm sayısı ise beş milyon 900 bini aşmıştır [1]. • Amerikan Hastalık Kontrol ve Korunma Merkezleri (CDC) 14 Ağustos 2020’de çocuk ve adolesa...

Familial Mediterranean fever (FMF) is a hereditary, autoinflammatory disease characterized by recurrent fever and serositis attacks. The disease onset occurs before 20 years of age in 90% of patients and rarely after the 4th decade. The aim of this study is to screen our FMF patient pool for patients with disease onset after age of 40 and to compar...

Initial case series of small number of patients at the beginning of the pandemic reported a rather guarded prognosis for Behçet’s syndrome (BS) patients infected with SARS-CoV-2. In this prospective study, we describe the incidence, clinical characteristics, disease course, management, and outcome in a large cohort of BS patients with laboratory-co...

Objectives Infliximab (IFX) is being increasingly used for the treatment of severe manifestations of Behçet syndrome (BS). However, emergence of new manifestations has also been occasionally reported during IFX treatment. We aimed to assess the frequency of new manifestations in our BS patients treated with IFX. Methods A chart review was conducte...

IntroductionThere is a lack of information about the frequency of obstructive sleep apnea (OSA) in primary Sjogren’s syndrome (pSS). Using all-night polysomnography (PSG), this study aimed to investigate the frequency of OSA in pSS and the factors affecting the frequency of OSA in this condition. Methods Consecutive patients with pSS who presented...

Background/aim: Giant cell arteritis (GCA) is a large vessel vasculitis that may cause significant morbidity in the elderly population. We aimed to evaluate presentation characteristics, treatment, and outcome in a cohort of patients with GCA diagnosed and followed in a single center. Materials and methods: A retrospective chart review revealed...

Hemophagocytic Lymphohistiocytosis (HLH) is a lethal complication of several infections especially viral origin. M.Tuberculosis infection can also lead HLH, yet it is an uncommon trigger. Considering role of increased cytokines in HLH, autoinflammatory conditions as Familial Mediterrean Fever (FMF) might contribute to development of it. Nevertheles...

Introduction Idiopathic granulomatous mastitis (IGM) is a chronic inflammatory breast disease of unknown etiology, and erythema nodosum (EN) is a rare extramammary manifestation of this entity characterized by reddish, tender nodules of the lower legs. We aimed to investigate whether the association of IGM with EN has a role as a prognostic indicat...

Background Idiopathic inflammatory myopathies (IIM) are essentially treated aiming to improve muscle function and extra muscular disease manifestations. Rituximab is potentially a glucocorticoid-sparing agent which was reviewed in multiple studies with small sample sizes due to the rarity of the disease. Higher statistical power can enhance the tru...

Objectives Vaccination against COVID-19 emerges as an effective strategy for combating the pandemic. While many of our patients with rheumatic diseases (RD) wonder whether it is safe to get the vaccine, vaccine hesitancy is rising among the general population. We assessed the willingness to get vaccination and its probable predictors among patients...

Bisalbuminemia is rare finding of bifid albumin bands in serum electrophoresis. The protein abnormality can be sporadic and inherited. So far, this albumin variant was not found to cause or coexist with a specific pathology. This is a case of bisalbuminemia in 43-year-old female patient with a past medical history of Rheumatoid Arthritis (RA) and S...

Background The course of novel coronavirus disease 2019 (COVID-19) has been of special concern in patients with inflammatory rheumatic diseases (IRDs) due to the immune dysregulation that may be associated with these diseases and the medications used for IRDs, that may affect innate immune responses. Objective In this cohort study, we aimed to rep...

Pathogenic variants in nucleotide-binding oligomerization-like receptor protein 12 (NLRP12) have been recently suggested as possible causes of autoinflammatory syndromes and should be considered for the differential diagnosis in the patients presenting with symptoms of autoinflammatory diseases. Here we report a very rare case of NLRP12-associated...

Objectives Anakinra is proven to be effective in controlled trials in terms of attack frequency and subclinical inflammation in colchicine-resistant patients. The objective of this study was to review the patients followed in our single centre with FMF who received anakinra because of insufficient colchicine response. Methods The study was conduct...

Background: There are few data on disease characteristics and outcome of coronavirus 2019 (COVID-19) among patients with inflammatory rheumatic diseases (IRDs). In this cohort study, we aimed to report the disease characteristics and variables associated with COVID-19 outcome among patients with IRDs. Methods: Between April and June, 2020, 167 adul...

The subacute cutaneous lupus erythematosus (SCLE) is a distinct subtype of lupus erythematosus (LE) representing specific clinical and serological features. Almost 20%‐30% of the cases with SCLE are predicted to associated with medications. Thiazide diuretics, terbinafine, antiepileptic, and proton pump inhibitors are the best‐known drugs to induce...

As the inflammation research improves year by year, so does our understanding of the autoinflammatory conditions. Over the past years, the number of monogenic autoinflammatory conditions snowballed thanks to our understanding of basic immunology and genetics. Familial Mediterranean Fever (FMF), being the entrance to this fascinating world, still ha...

Objectives: This study aims to investigate the coexistence of familial Mediterranean fever (FMF) and Behçet's disease (BD). Patients and methods: This cross-sectional study was conducted between May 2009 and August 2009. The study included 14,881 randomized children (7,741 males, 7,140 females; mean age 13.0 years; range, 12 to 14 years) from si...

Objective: The main devastating complication of FMF is AA amyloidosis. Approximately 10-15% of the patients are either intolerant or have an insufficient response to colchicine treatment. The most promising alternative treatment approach is anti-IL-1 agents. The aim of this study was to evaluate the efficacy and safety of anti-IL-1 therapy in FMF...

It has been reported that multiple sclerosis (MS) is more common among patients with familial Mediterranean fever (FMF) than expected in normal population. Also, an association between MEFV gene variants and disease severity in MS has been described. Excessive production of interleukin-1 (IL-1) beta is responsible for FMF pathogenesis, and anti-IL-...

Objectives: Triggering Receptor Expressed on Myeloid cells-1 (TREM-1) is a monocyte and neutrophil receptor functioning in innate immunity. TREM-1 activity has been studied in various autoimmune diseases such as RA and SLE but there is no data in autoinflammatory pathologies. We studied soluble TREM-1 (sTREM-1) activity in Familial Mediterranean Fe...