Fertility, pregnancy outcomes, and disease activity during pregnancy in patients with juvenile idiopathic arthritis: a descriptive study.

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 criteria, who are now monitored at an adult rheumatology clinic. Data on demographics, disease characteristics, medication history, fertility status, and pregnancy outcomes were collected through medical records and patient interviews.

A rare case of late-onset spondyloenchondrodysplasia with immune dysregulation presenting as adult-onset monogenic lupus.

Background: Spondyloenchondrodysplasia is classified as an interferonopathy resulting from recessive mutations in the 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 typically considered multifactorial and more prevalent in adulthood, a subset of approximately 10%-25% of childhood cases arise from monogenic form. Among these, spondyloenchondrodysplasia accounts for only a rare fraction of monogenic lupus cases, with only 22 reported instances in the literature.

MEF-AlloSite: an accurate and robust Multimodel Ensemble Feature selection for the Allosteric Site identification model.

A crucial mechanism for controlling the actions of proteins is allostery. Allosteric modulators have the potential to provide many benefits compared to orthosteric ligands, such as increased selectivity and saturability of their effect. The identification of new allosteric sites presents prospects for the creation of innovative medications and enhances our comprehension of fundamental biological mechanisms.

Measuring Transition Readiness of Patients After Transfer from Pediatric to Adult Care in Rheumatology.

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. We included young adult rheumatology patients who had already transferred to adult rheumatology care.

Anakinra in the management of adult-onset still's disease: a single-center experience.

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 lymphadenopathy. Glucocorticoids and biological disease-modifying anti-rheumatic drugs, including the anti-interleukin-1 agent anakinra, are used in the management of AOSD.

Characteristics of arthritis in patients with familial Mediterranean fever.

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 our clinic between 2005 and 2020.

Sexual dysfunction among female patients with rheumatic diseases.

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), and BS (n = 54), along with 52 female HCs were included in this study between April and October, 2021. Sociodemographic features were recorded, and psychometric tests, i.

Targeting complement dysregulation: eculizumab in scleroderma renal crisis management-a case-based review.

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 treatments such as plasma exchange and renin-angiotensin-aldosterone system blockade.

Severity scores, damage indices, and the concept of the colchicine-resistant patient in familial Mediterranean fever.

Familial Mediterranean fever 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.

Relationship of psoriatic arthritis with nail and scalp involvement in Turkish psoriasis patients: Multicentered cross-sectional study.

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-sectional study, conducted from June 2021 through December 2021, included 763 patients from 11 different centers in Turkey.

Exploring S100A8/A9, neopterin, and MMP3 in familial Mediterranean fever.

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 most common misdiagnosis among FMF patients. Blood samples (n = 75), comprising from FMF patients during an attack (n = 20), the same FMF patients during the attack-free period (n = 14), patients with appendicitis (n = 24), and healthy volunteers (n = 17) were obtained.

Efficacy and safety of biologic drugs in Still's disease: a systematic review and network meta-analysis of randomized controlled trials.

Objectives: Still's disease is a rare autoinflammatory disorder characterized by systemic inflammation, fever, rash and arthritis. The term 'Still's disease' covers the paediatric subtype systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), which affects adults. Biologic drugs, including the anti-IL-1 agents anakinra, canakinumab and rilonacept and the IL-6 antagonist tocilizumab, are used in the management of Still's disease.

Secukinumab after first-line tumor necrosis factor-alpha inhibitor therapy in psoriatic arthritis: A real-world retrospective cohort study.

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 followed up between October 2018 and October 2021. The patients were divided into two groups according to their anti-TNF-α treatment history.

A rare case of uncharacterized autoinflammatory disease: Patient carrying variations in NLRP3 and TNFRSF1A genes.

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 TRAPS and CAPS, without known pathogenic variants in the respective genes. The patient harbored the p.

Factors triggering familial mediterranean fever attacks, do they really exist?

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 trigger factors of familial Mediterranean fever.

Anakinra in idiopathic recurrent pericarditis: a comprehensive case series and literature review.

Objective: 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.

Methods: A retrospective evaluation of patients treated at our autoinflammatory center between 2011 and 2023 was conducted.

Cobdock: an accurate and practical machine learning-based consensus blind docking method.

Probing the surface of proteins to predict the binding site and binding affinity for a given small molecule is a critical but challenging task in drug discovery. Blind docking addresses this issue by performing docking on binding regions randomly sampled from the entire protein surface. However, compared with local docking, blind docking is less accurate and reliable because the docking space is too largetly sampled.

The clinical role of anakinra in the armamentarium against familial Mediterranean fever.

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 pathogenesis of FMF. This review summarizes the current approach to treating FMF and discovers the pharmacological and clinical utility of IL-1 blocking agents based on accumulated evidence with a focus on anakinra.

Unprecedented coexistence of autoinflammatory myositis and chronic thrombosis with heterozygotic M694V mutation: An atypical presentation of familial Mediterranean fever.

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.