OBJECTIVES:Subglottic stenosis (SGS) is a challenging manifestation of granulomatosis with polyangiitis (GPA), often relapsing and poorly responsive to immunosuppressive treatment. Current guidelines lack specific recommendations for SGS management and evidence is limited. This study aimed to identify features of SGS associated with a more aggressive course and to assess the efficacy of available treatments in preventing relapses. METHODS:We conducted a multicentre, retrospective cohort study including GPA patients with SGS. Patients were stratified into higher relapsers (≥2 flares) and lower relapsers (≤1 flare). Clinical and treatment features were compared across groups. Univariate and multivariate analyses were conducted to identify independent predictors of relapse and multiple flares. Kaplan-Meier curves assessed time-to-relapse across regimens. RESULTS:Eighty-nine patients were included (30% male). Forty-eight patients (54%) were higher relapsers, with a median time-to-relapse of 36 months. Systemic immunosuppressive therapy was associated with fewer relapses (82% vs 18%, P = 0.04) compared with local treatments alone. Glucocorticoids in induction regimens reduced relapse risk (86% vs 12%, P = 0.03). Cyclophosphamide (CYC) was associated with the longest relapse-free survival and reduced 5-year relapse risk (HR 0.33, P = 0.049). By contrast, glucocorticoid monotherapy in either induction or maintenance phase was associated with higher relapse rate (P = 0.006). No specific maintenance regimen was significantly protective, though rituximab and DMARDs showed a trend toward improved outcomes. CONCLUSION:Systemic immunosuppressive therapy, particularly CYC-based induction, was associated with fewer SGS relapses and prolonged relapse-free survival, while glucocorticoid monotherapy was less effective. Prospective studies are needed to optimize induction and maintenance strategies in GPA-related SGS.

To investigate the relationship between gastrointestinal symptom burden assessed by the Dudley Inflammatory Bowel Symptom Questionnaire (DISQ), fecal calprotectin (FC), a non-invasive biomarker suggestive of intestinal inflammation, disease activity, and clinical characteristics in patients with axial spondyloarthritis (axSpA). In this cross-sectional study, 174 patients with axSpA were enrolled. Gastrointestinal symptom burden was assessed using the DISQ, disease activity using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), functional status using the Bath Ankylosing Spondylitis Functional Index (BASFI), and health-related quality of life using the Short Form-36 (SF-36). FC levels were measured by enzyme-linked immunosorbent assay. Correlation analyses, receiver operating characteristic (ROC) analyses, and multivariable logistic regression models were performed. FC levels were normal (< 50 μg/g) in 52.9

Background/aim:In Behçet's disease (BD), arterial involvement represents a significant clinical challenge associated with serious complications. The present study evaluates the characteristic features, affected vessels, lesion types, treatments, and outcomes in BD patients with nonpulmonary arterial involvement. Materials and methods:Included in this retrospective study were 65 BD patients whose interventions and clinical courses were accessed from medical records. The treatments administered included immunosuppressive (IS) therapies and surgical/endovascular procedures. Fisher's exact and t-test/Mann-Whitney U test were used for statistical analysis. Results:Fifty-nine of the patients (90.8%) were male. The median (Q1-Q3) follow-up was 4 (2-12.8) years. The most commonly affected vessels were the aorta (n = 21, 32.3%) and the femoral artery (n = 19, 29.2%). Aneurysm (n = 50, 63.2%) and thrombosis (n = 18, 22.7%) were the predominant lesions; 14 (21.5%) patients experienced arterial involvement while receiving IS therapy; and 11 (16.9%) had cardiac involvement. Cyclophosphamide and azathioprine were the most commonly used IS agents. Among the 39 (60%) patients who underwent surgical and/or interventional procedures, 32 (82%) had successful outcomes. A second arterial event was experienced by 15.3% of the sample during follow-up, and a third by 3%. Active disease was noted in nine (13.8%) patients at the time of their last visit, and of the six (9.2%) that died, most had both arterial and cardiac involvement. Conclusion:Arterial involvement in BD mostly presents with aneurysms, especially in men. Surgical interventions in addition to IS therapies are required in approximately 50% of cases. As can be understood from the 15.3% relapse rate in the present study, arterial events are not uncommon, underlining the need for long-term monitoring and sustained IS treatment. Of the 9.2% of cases who died, most had both arterial and cardiac involvement.

Patients with ANCA-associated vasculitis (AAV) are at increased risk of osteoporosis due to multiple disease- and treatment-related factors. However, real-world data on osteoporosis screening practices in this population remain limited. The aim of this study was to assess osteoporosis screening practices and the prevalence of reduced bone mineral density (BMD) in patients with AAV using a nationwide registry, and to identify factors associated with osteoporosis. This nationwide, retrospective, web-based study was conducted using the Turkish Vasculitis Study Group (TRVaS) database, including patients diagnosed with AAV between January 2000 and December 2023. Patients were evaluated for osteoporosis screening using dual-energy X-ray absorptiometry (DXA) and categorized according to BMD status. Clinical characteristics and potential risk factors were analyzed, and multivariable logistic regression was performed to identify independent predictors of osteoporosis. A total of 528 patients were included (median age 56 years [interquartile range 43–66]; 57.8

Objective SLE is a heterogeneous systemic autoimmune disease with diverse clinical manifestations. We aimed to identify clinical subgroups of SLE patients using cluster analysis at diagnosis and at the last follow-up and to evaluate their prognostic implications for disease activity, organ damage and mortality.Methods In this single-centre retrospective cohort study, 199 patients with SLE who fulfilled the 1997 American College of Rheumatology (ACR) classification criteria and were regularly followed at the Rheumatology Clinic of Kocaeli University Hospital were included. Patients were divided into clusters based on their clinical involvement at diagnosis and the last visit using the Gower distance and the Partitioning Around Medoids algorithm. Differences between clusters were assessed using one-way analysis of variance, Kruskal-Wallis and χ2 analyses. Analyses were performed using SPSS V.29.0 (IBM Corp, Armonk, New York, USA) and R V.4.3.0.Results At diagnosis, three clusters were identified: Cluster 1 (n=108) with predominant arthritis; Cluster 2 (n=49) with renal and haematological involvement and highest disease activity (SLE Disease Activity Index 2000, p=0.004); Cluster 3 (n=42) with mucocutaneous manifestations, often accompanied by haematological involvement. After a median follow-up of 168 months, four clusters were identified. Cluster 4′, a heterogeneous group with mucocutaneous, articular and renal involvement, had significantly higher Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index (SDI) scores (median 4.0 (0–8), p<0.001), indicating poor prognosis. During follow-up, 29.6% of Cluster 1, 22.4% of Cluster 2 and 42.9% of Cluster 3 transitioned to Cluster 4′. Patients with mucocutaneous-predominant disease at diagnosis had a twofold increased risk of transitioning to the poor-prognosis cluster (OR 2.05, 95% CI 1.01 to 4.16, p=0.046). Mortality did not differ significantly between clusters.Conclusion Cluster analysis based on clinical involvement in SLE can identify homogeneous patient subgroups and predict long-term outcomes. Patients with mucocutaneous-predominant onset, often receiving less intensive treatment, may have a worse prognosis, highlighting the importance of individualised monitoring and management strategies.

Objection: Data on the clinical course of COVID-19 and subsequent humoral immune responses in patients with rheumatic diseases remain limited. This study aimed to compare SARS-CoV-2 antibody responses between patients with rheumatic diseases and healthy controls and to identify clinical and treatment-related factors associated with antibody levels. Methods: This prospectively designed, single-center cross-sectional study included unvaccinated adults with rheumatoid arthritis, systemic lupus erythematosus, or ankylosing spondylitis who had a previous COVID-19 infection, along with age- and sex-matched healthy controls. COVID-19 was identified by PCR confirmation or compatible thoracic CT findings, and anti-spike and anti-nucleocapsid SARS-CoV-2 antibodies were assessed 1-6 months after infection using Elecsys immunoassays. Results: PCR-confirmed infection (P< .001) and several COVID-19-related symptoms, including headache, dyspnea, nausea/vomiting, fatigue, sore throat, and arthralgia, were significantly more frequent in the patient group (all P < .05). However, hospitalization and pulmonary involvement were each significantly more common in the control group (both P< .001). Intensive care admission was similarly low in both groups. Anti-S seropositivity was significantly higher in patients than in controls (P < .001), while anti-N seropositivity and anti-N/anti-S antibody titers were comparable between groups. Antibody levels did not differ significantly among rheumatic disease subgroups. However, both anti-N and anti-S titers were significantly higher in patients not receiving biologic therapy (both P < .001). Conclusion: Humoral immune responses following COVID-19 appeared to be largely preserved in patients with rheumatic diseases. Although anti-S seropositivity was higher in the patient group, antibody titers were lower among those receiving biologic therapy, suggesting a potential treatment-related effect on humoral immunity. Larger studies are warranted to confirm these findings.

OBJECTIVES:Takayasu arteritis (TAK) may lead to arterial stenosis/occlusion and aneurysms, occasionally requiring vascular interventions. We evaluated outcomes, restenosis, and complications after vascular interventions in TAK and explored factors associated with restenosis. METHODS:We retrospectively included TAK patients who underwent extracardiac endovascular or surgical vascular interventions before or after TAK diagnosis across eight tertiary centers (1995-2024). Indications, disease activity status at the time of intervention, procedural characteristics, early/late outcomes, major complications, and restenosis were recorded. Restenosis was defined as > 70% stenosis on follow-up imaging performed ≥ 3 months after the procedure. Restenosis analyses were restricted to steno-occlusive lesions. RESULTS:We included 119 patients with 201 treated lesions (185 steno-occlusive lesions; 16 aneurysms/dissections). Of all procedures, 61.2% (n = 123) were performed after TAK diagnosis under immunosuppressive therapy. Follow-up imaging was available for 170/185 steno-occlusive procedures, and restenosis status was evaluable in 168 procedures; restenosis occurred in 82/168 (48.8%) with a median time to restenosis of 19.5 months. In exploratory Kaplan-Meier analysis, 1-, 3-, and 5-year restenosis-free patency rates were 76.8%, 63.6%, and 56.5%, respectively. In analyses restricted to post-diagnosis steno-occlusive procedures, crude restenosis was significantly less frequent under biologic therapy than without biologic therapy (30.8% vs. 53.1%; Fisher's exact p = 0.040); however, biologic therapy was not associated with time to restenosis in Cox regression (HR 1.01, 95% CI 0.51-1.99; p = 0.97) or patient-clustered Cox analysis (HR 1.01, 95% CI 0.47-2.18; p = 0.98). Major complication rates were 3.9% for endovascular procedures and 12.5% for surgical procedures. Overall mortality was 11.8%, with two deaths attributed to procedure-related complications. CONCLUSIONS:Vascular interventions in selected patients with Takayasu arteritis were associated with acceptable major complication rates. Among post-diagnosis steno-occlusive procedures, biologic therapy was associated with lower restenosis rates in crude analyses; however, this association was not sustained after accounting for restenosis timing. Therefore, the potential role of biologic therapy in improving post-interventional vascular outcomes should be interpreted cautiously and requires confirmation in prospective studies with standardized imaging surveillance.

To evaluate longitudinal renal trajectories and predictors of kidney function in patients with rheumatoid arthritis (RA) receiving biologic therapies, using serial estimated glomerular filtration rate (eGFR) measurements during follow-up. As a secondary objective, renal outcomes were compared between anti-tumor necrosis factor (anti-TNF) agents and other biologic therapies. This single-center retrospective cohort study included 141 patients with RA fulfilling the 2010 ACR/EULAR classification criteria and receiving the same biologic therapy for at least one year. Renal parameters, including eGFR, creatinine, and urea, were assessed at baseline, every 6 months up to 24 months, and at final follow-up. Chronic kidney disease (CKD) was defined according to the 2024 KDIGO criteria. Propensity score matching (PSM) was performed to balance baseline characteristics between anti-TNF and non-TNF groups. Longitudinal renal changes were evaluated using linear mixed-effects models. Baseline CKD prevalence was 5.0

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by autoantibody production and immune complex deposition. Antiphospholipid syndrome (APS) is frequently associated with SLE and is characterized by arterial or venous thrombosis and pregnancy morbidity in the presence of persistent antiphospholipid antibodies. The coexistence of ankylosing spondylitis (AS) with SLE and APS is extremely rare. A 38-year-old woman was admitted to our clinic with gangrene of the right fifth toe and swelling of both knees. She had a previous diagnosis of AS based on inflammatory low back pain and radiographic evidence of bilateral Grade 4 sacroiliitis. Laboratory investigations revealed positive antinuclear antibody, anti-double-stranded DNA, and antiphospholipid antibodies, including lupus anticoagulant and anti-cardiolipin IgG. Renal biopsy performed due to proteinuria demonstrated mesangioproliferative lupus nephritis. Based on clinical, laboratory, and histopathological findings, the patient was diagnosed with coexisting AS, SLE, and APS. Considering the coexistence of these autoimmune diseases and the patient’s previous exposure to anti-tumor necrosis factor therapy, treatment with the interleukin-17 inhibitor secukinumab was initiated. The patient showed significant improvement in inflammatory back pain and arthritis during follow-up. To our knowledge, this is one of the rare reported cases of coexistence of AS, SLE, and APS treated with secukinumab.

OBJECTIVES:Anti-topoisomerase I antibody (ATA) is typically associated with diffuse cutaneous systemic sclerosis (dcSSc). However, subset of limited cutaneous SSc (lcSSc) patients also present with ATA positivity. Emerging data suggest that ATA-positive lcSSc may represent a distinct or intermediate clinical phenotype. This study aimed to compare the demographic, clinical, and treatment features of early ATA-positive lcSSc patients with those of ACA-positive lcSSc and ATA-positive dcSSc patients. METHODS:Patients were recruited from the multicentre Turkish SOLAR cohort (Systemic sclerOsis Longitudinal Assessment Registry). Among 295 SSc patients screened, 172 were included: 74 ACA-positive lcSSc, 55 ATA-positive lcSSc, and 43 ATA-positive dcSSc. Demographic, clinical, and treatment-related variables were analysed and compared across groups. RESULTS:ATA-positive lcSSc patients were younger at the onset of Raynaud's phenomenon (RP) (p=0.042), the first non-RP symptom (p=0.016), and at diagnosis (p=0.018) compared with ACA-positive lcSSc patients. Interstitial lung disease (ILD) was significantly more frequent in ATA-positive lcSSc (74.5%) than ACA-positive lcSSc (8.1%, p<0.001) and was comparable to ATA-positive dcSSc. Modified Rodnan skin scores were highest in ATA-positive dcSSc but were also significantly elevated in ATA-positive lcSSc (p<0.001). Pitting scars were more frequent in dcSSc. Among patients with ILD, ATA-positive lcSSc and ATA-positive dcSSc showed similar HRCT patterns. ATA-positive lcSSc patients were more frequently treated with glucocorticoids and mycophenolate mofetil than ACA-positive lcSSc, whereas cyclophosphamide was highest in dcSSc. CONCLUSIONS:ATA-positive lcSSc patients exhibit a clinically distinct phenotype characterized by a substantial risk of internal organ involvement, despite having less extensive skin disease. Their overlap with dcSSc and divergence from ACA-positive lcSSc highlight the importance of incorporating both skin involvement and serologic subtyping into the early management and risk stratification of SSc.

Subclinical and overt inflammatory bowel disease (IBD) is frequently observed in patients with axial spondyloarthritis (ax-SpA), traditionally identified through invasive ileocolonoscopic evaluation. Validated non-invasive assessing instruments are therefore needed to facilitate early detection. This study aimed to validate the Turkish version of the Dudley Inflammatory Bowel Symptom Questionnaire (DISQ) as a tool for assessing gastrointestinal symptom burden suggestive of intestinal involvement in patients with axial spondyloarthritis. In this cross-sectional validation study, 174 patients fulfilling the 2009 Assessment of SpondyloArthritis international Society (ASAS) criteria for axial SpA were enrolled. Convergent validity was assessed by examining correlations between DISQ scores and disease activity (Bath Ankylosing Spondylitis Disease Activity Index [BASDAI]), functional status (Bath Ankylosing Spondylitis Functional Index [BASFI]), and health-related quality of life (Short Form-36 [SF-36]). Discriminant validity was evaluated by comparing DISQ scores in patients with and without gastrointestinal symptoms suggestive of IBD. Test–retest reliability was assessed by re-administration of the DISQ after one month. Internal consistency was evaluated using Cronbach’s alpha. DISQ scores demonstrated moderate positive correlations with BASDAI (r = 0.432, p < 0.001) and BASFI (r = 0.248, p = 0.001), and a strong inverse correlation with SF-36 scores (p < 0.001), supporting convergent validity. Patients reporting diarrhea, abdominal pain, oral aphthae, or mucus in stool had significantly higher DISQ scores than those without these symptoms (all p ≤ 0.008), confirming discriminant validity. The Turkish version of the DISQ demonstrated acceptable internal consistency (Cronbach’s α = 0.70) and good test–retest reliability (ICC = 0.76; 95

This study aims to investigate the relationship between sleep hygiene and sleep quality in patients with systemic sclerosis (SSc) and to compare the sleep hygiene and sleep quality outcomes across three distinct groups: SSc patients, rheumatoid arthritis (RA) patients, and healthy controls (HC). This study employed an observational, cross-sectional, and parallel group design. SSc-related and RA-related variables, depression and anxiety were assessed. Physical function and quality of life, pain and fatigue of SSc patients were also evaluated. Sleep quality using the Pittsburg Sleep Quality Index (PSQI) and sleep hygiene using the Sleep Hygiene Index (SHI) were evaluated for all participants. Linear regression analysis was performed to show the relationship between the SHI scores and the other variables. Total PSQI and SHI scores were found to be significantly higher in SSc patients than in RA patients and HC. Fatigue, smoking, all SF-36 domains, depression and anxiety scores were associated with SHI scores in SSc patients. In the univariate logistic regression analysis, SSc patients exhibited 4.50 times higher odds (95