Do functionality, strength, vascularization, inflammatory and biopsychosocial status improve by biopsychosocial model-based exercise in SSc?

OBJECTIVES: This study aimed to investigation of the effects of the Cognitive Exercise Therapy Approach (Bilissel Egzersiz Terapi Yaklasimi-BETY), a supervised biopsychosocial model-based exercise intervention, on functionality, muscle strength, vascularization, anti-inflammatory and biopsychosocial status in Systemic Sclerosis (SSc) patients. METHODS: Thirty-seven SSc patients were included. Twenty of them were recruited into the study group (SG) undergoing BETY group exercise sessions three times a week for three months and 17 were in the control group (CG) following a home exercise program. Assessments tools were the Modified Rodnan Skin Score (mRSS), Scleroderma Health Assessment Questionnaire (SHAQ), Modified Hand Mobility in Scleroderma (mHAMIS), Duruoz Hand Index (DHI), Six Minute Walk Test (6MWT), skeletal muscle strength measurements using an isokinetic dynamometer (Biodex System 3 Pro), Shear Wave Elastography (SWE), ELISA kits (for tumor necrosis factor-alpha, Interleukin-6, IL-10, serum irisin level), BETY-Biopsychosocial Questionnaire (BETY-BQ), Hospital Anxiety and Depression Scale (HADS), and Short Form-36 (SF-36). RESULTS: The SG demonstrated improvements in SHAQ, mHAMIS, 6MWT, BETY-BQ, HADS, and SF-36 values, excluding the DHI scores (p < 0.05). In contrast, CG showed worsening in SHAQ-general scleroderma symptoms and HADS scores compared to SG (p < 0.05). IL-10 and TNF-alpha increased in both groups, also various vascular parameters were significantly different changed in SG than CG (p < 0.05). Muscle strength values improved in the SG but decreased in the CG however this was statistically not significant (p > 0.05). CONCLUSIONS: BETY can be recommended as a nonpharmacologic approach to the disease management of SSc patients.

Comparison of the effects of axillary brachial plexus block, inhalation anesthesia, and total intravenous anesthesia on tourniquet-induced ischemia-reperfusion injury in upper extremity surgery.

BACKGROUND: Post-ischemia reperfusion can lead to oxidative stress and an increase in oxidative markers. Employing preventive strategies and antioxidant agents may help mitigate ischemia-reperfusion injury (IRI). The use of a tourniquet in extremity surgery has been associated with IRI. This study aims to investigate the impact of three different approaches- brachial plexus block, total intravenous anesthesia (TIVA), and inhalation anesthesia-on IRI during upper extremity surgery using a tourniquet. METHODS: Patients aged 18 to 45 with American Society of Anesthesiologists (ASA) I-II scores were randomly assigned to one of three groups: Group A received an axillary block with bupivacaine; Group I underwent inhalation anesthesia with sevoflurane; and Group T received TIVA with propofol and remifentanil infusion. Blood samples were collected to measure glucose, lactate, total anti-oxidant status (TAS), total oxidant status (TOS), and ischemia-modified albumin (IMA) levels at various time points: before anesthesia (t1), 1 minute before tourniquet release (t2), 20 minutes after tourniquet release (t3), and 4 hours after tourniquet release (t4). RESULTS: In Group I, lactate levels at t3, and glucose levels at t2 and t3, were higher compared to the other groups. Group A exhibited lower IMA levels at t2, t3, and t4 than the other groups. Additionally, Group I had lower IMA levels at t2, t3, and t4 compared to Group T. TAS levels were higher in Group I at t2, t3, and t4 compared to the other groups. TOS levels at t2 and t3 were lower in Group A than in Group I. CONCLUSION: Axillary anesthesia results in a sympathetic block, promoting better perfusion of the upper extremity. This study demonstrated lower levels of oxidative stress markers with axillary plexus block. Therefore, these results suggest that the axillary block has the potential to mitigate IRI.

Computed tomography imaging-based radiologic evaluation of pulmonary artery thrombosis in a series of patients with Behcet's disease.

AIM: Pulmonary artery involvement is a severe complication of Behcet's disease (BD). Although venous thrombosis is common in BD, pulmonary embolism is considered to be rare because the inflammatory nature makes the thrombi strongly adherent to the venous walls. This study aimed to define the radiological characteristics of pulmonary artery thrombosis (PAT) on computed tomography (CT) imaging in BD patients. METHODS: We retrospectively evaluated 165 BD patients with vascular involvement. Among the patients with venous involvement (n = 146), we identified 65 patients who had undergone thorax CT imaging previously. Fourteen patients who were diagnosed with PAT were included in the study. Expert radiologists re-evaluated the patients' initial and control thorax CT scans, classified the PAT as acute or chronic based on their radiological features. RESULTS: The patients' median age was 35 (min-max: 15-60) years at the time of the initial CT scan, and nine were male. Twelve (85.7%) patients were symptomatic at the time of CT evaluation. Upon re-evaluating the thorax CTs, acute PAT was diagnosed in six (42.8%); chronic PAT was detected in eight (57.1%) patients. Two patients with chronic PAT also had acute PAT. Pulmonary artery aneurysms were present in three (21.4%) patients, and intracardiac thrombus was found in three (21.4%) patients. CONCLUSION: A significant number of BD patients with venous involvement had radiological findings consistent with acute PAT potentially due to pulmonary emboli in this study. The clinical importance of these lesions has to be defined with future studies.

Assessing the quality of forced vital capacity measurement in patients with systemic sclerosis.

INTRODUCTION: Forced vital capacity (FVC) is an important tool for monitoring lung functions in patients with systemic sclerosis (SSc). However, several disease manifestations may influence the quality of FVC test in SSc. We aimed to assess the quality of FVC measurements according to current guidelines in patients with SSc and determine the factors that may affect results. METHOD: In this cross-sectional study, SSc patients and age/sex matched controls underwent spirometry. Quality of FVC measurements were graded according to updated American Thoracic Society (ATS) and European Respiratory Society (ERS) guidelines. Demographics, clinical features and parameters that may affect FVC test quality were compared between SSc patients with high and low quality FVC test. RESULTS: 98 SSc patients (90 female) and 100 controls were included. The rate of high quality FVC measurement in SSc patients was significantly lower in SSc patients compared to controls. (80 % vs 60.2 % p = 0.002). Among SSc patients; diffuse disease, ILD, anti-topoisomerase 1 antibody positivity, immunosuppressive use, flexion contractures of hands, reduced mouth opening and decreased chest expansion were more frequent in patients with low quality FVC (p < 0.05 for all). Patients with muscle weakness and medium/high risk of malnutrition were also numerically higher in low quality FVC group. Presence of more than one condition that may affect FVC quality was significantly higher among patients with low quality FVC. CONCLUSION: A significant percent of SSc patients had low quality FVC measurement. Physicians should be aware of this point while interpreting FVC test results especially in SSc patients with more than one condition that may affect the quality of the test.

Clinical characteristics and disease course before and after SARS-CoV-2 infection in a large cohort of systemic sclerosis patients.

Background/aim: The objective of this study is to evaluate the clinical presentations and adverse outcomes of Coronavirus Disease 2019 (COVID-19) in patients with systemic sclerosis (SSc) and assess the impact of SSc features on the clinical course of COVID-19. Materials and methods: In this multicenter, retrospective study, SSc patients with COVID-19 were included. Clinical features of SSc, along with detailed COVID-19 data, were extracted from medical records and patient interviews. Results: The study included 112 patients (mean age 51.4 ± 12.8 years; 90.2% female). SSc-associated interstitial lung disease (ILD) was evident in 57.1% of the patients. The findings revealed hospitalization in 25.5%, respiratory support in 16.3%, intensive care unit admission in 3.6%, and a mortality rate of 2.7% among SSc patients with COVID-19. Risk factors for respiratory failure, identified through univariate analysis, included ILD (OR: 7.49, 95% CI: 1.63-34.46), ≥1 comorbidity (OR: 4.55, 95% CI: 1.39-14.88), a higher physician global assessment score at the last outpatient visit (OR 2.73, 95% CI: 1.22-6.10), and the use of mycophenolate at the time of infection (OR: 5.16, 95 %CI: 1.79-14.99). Notably, ≥1 comorbidity emerged as the sole significant predictor of the need for respiratory support in COVID-19 (OR: 5.78, 95% CI: 1.14-29.23). In the early post-COVID-19 period, 17% of patients reported the progression of the Raynaud phenomenon, and 10.6% developed new digital ulcers. Furthermore, progression or new onset of dyspnea and cough were detected in 28.3% and 11.4% of patients, respectively. Conclusion: This study suggests a potential association between adverse outcomes of COVID-19 and SSc-related ILD, severe disease activity, and the use of mycophenolate. Additionally, it highlights that having comorbidities is an independent risk factor for the need for respiratory support in COVID-19 cases.

Survival in systemic sclerosis associated pulmonary arterial hypertension in the current treatment era-results from a nationwide study.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). This nationwide study aims to describe real world treatment characteristics and assess survival rates of patients with SSc-PAH. METHODS: In this retrospective cohort study, patients with SSc-PAH were identified from Turkish Ministry of Health National Electronic Database (from January 2016 to September 2022), using ICD-10 codes. Data on demographics, treatment characteristics, and death was collected. Kaplan-Meier curves were used to calculate cumulative probabilities of survival at 1, 3, and 5 years. RESULTS: Five hundred forty-seven patients (90.7% female) with SSc-PAH were identified. Median age at PAH diagnosis was 59.9 (50.0-67.4) years. During a median follow-up duration of 3.2 (1.5-4.8) years, 199 (36.4%) deaths occurred. Estimated survival rates at 1, 3, and 5 years were 90.2%, 73.2%, and 56.6%, respectively. Survival was similar among patients with and without interstitial lung disease (p = 0.20). Patients who used immunosuppressives had better survival than those who did not (p < 0.001). No difference was observed in survival rates according to initial PAH-specific treatment regimen (monotherapy or combination) (p = 0.49). CONCLUSION: Compared to most of historical cohorts, higher survival rates for SSc-PAH were observed in this study. Early diagnosis of PAH may have contributed to these findings. The impact of immunosuppressive therapy on prognosis of SSc-PAH needs to be further investigated in prospective studies. Key Points • Early diagnosis is pivotal for better outcomes in SSc-PAH. • Implementation of PAH treatment guidelines in routine clinical practice is still poor and should be improved. • Effect of immunosuppressive therapies on disease course has to be defined in SSc-PAH.

Factors that predict development of chronic kidney disease in patients with rheumatoid arthritis receiving biologic DMARDs and mortality rates.

OBJECTIVES: We aimed to determine the choice of biologic/targeted synthetic disease-modifying anti-rheumatic drugs (b/ts-DMARDs), factors associated with the development of chronic kidney disease (CKD), and mortality in RA patients with CKD receiving b/ts-DMARDs. METHODS: Two thousand one hundred forty-one RA (79.4% female) patients were included in the analysis from the HUR-BIO prospective registry. Patients were divided into the CKD group and the non-CKD group. Age and gender-matched patients were selected from the non-CKD group, and then three main groups were determined. CKD was staged according to the glomerular filtration rate criteria. The clinical characteristics of the patients, disease activities, treatment choices, drug retention rate, and mortality rates were compared between the groups. RESULTS: CKD was detected in 90/2141 (4.2%) RA patients on b/ts-DMARDs. Forty patients (2.3%) developed CKD during follow-up after the initiation of b/ts-DMARDs. In the CKD group, anti-TNF agents were chosen as the first-line b/ts-DMARDs therapy in 64.4% of patients, with etanercept leading in 31 (34.4%) patients. In multivariate analysis, age at the start of treatment, DAS-28-ESR at last visit, amyloidosis, hypertension, and history of smoking were the factors associated with the development of CKD in RA patients receiving b/ts-DMARDs. The mortality rate in RA-CKD patients until the onset of the pandemic was 15.41 per 1000 patient years, whereas it was 85.9 per 1000 patient years after the pandemic. CONCLUSION: Comorbidities and control of disease activity are critical in the development of CKD in RA patients receiving b/ts-DMARDs. While there was no significant difference in mortality rate between CKD and non-CKD patients, the overall mortality rate increased after the COVID-19 pandemic duration in both groups.

Do we consider enough the presence of triggering factors in the evaluation of patients with FMF? Triggering factors are highly prevalent in colchicine-resistant FMF patients.

The aim of the study is to investigate the frequency of triggering factors in colchicine-resistant and -responsive Familial Mediterranean Fever (FMF) patients as well as the effect of  interleukin (IL)-1 antagonist treatment on the triggering factors. Both colchicine-resistant (patients on IL-1 antagonist treatment) and colchicine-responsive (patients on colchicine who had ≤ 3 attacks in the last year) patients were questioned for the presence of 12 different triggering factors, including exposure to cold, emotional stress, fatigue, physical activity, menstruation (for females), sleeplessness, prolonged standing, long-duration travel, high-fat diet intake, starvation, infection, and trauma. Colchicine-resistant patients were questioned for the presence of triggering factors for two time periods, before and after treatment with IL-1 antagonists. We studied 28 colchicine-resistant and 35 colchicine-responsive patients. Overall 77.8% of patients had at least one triggering factor. Triggering factors were associated with 28.5% of the total number of attacks. More than half of the patients (57.1%) declared that they had avoided these conditions. The frequency of triggering factors was higher in the colchicine-resistant group as compared to the colchicine-responsive group (89.3% vs 68.6%; p = 0.04). In colchicine-resistant FMF patients, the frequency of triggering factors (89.3% vs 32.1%) and the percentage of attacks initiated by triggering factors (27.8 vs 14.4%; p < 0.001) were decreased after treatment with IL-1 antagonists. In this study, triggering factors were more frequent in colchicine-resistant patients as compared to colchicine-responsive patients. Treatment with IL-1 antagonists seems to increase the endurance of colchicine-resistant patients in stressful conditions.

Extended autoantibody panel in Turkish patients with early-stage systemic sclerosis: Coexpressions and their influences on clinical phenotypes.

BACKGROUND/AIM: To investigate the frequency and clinical relevance of an extended autoantibody profile in patients with systemic sclerosis (SSc). MATERIALS AND METHODS: In this cross-sectional study, serum from 100 consecutive patients was subjected to indirect immunofluorescence (IIF) (HEp-20-10/primate liver mosaic) and Systemic Sclerosis Profile by EUROIMMUN to evaluate anti-nuclear antibodies (ANA) and autoantibodies against 13 different autoantibodies in patients with SSc less than 3 years. RESULTS: Ninety-three of 100 patients were positive for ANA by IIF. Fifty-three patients showed single positivity, 26 anti-topoisomerase antibodies (anti-Scl70 ab), 16 anticentromere antibodies (ACAs), six anti-RNA polymerase III antibodies (anti-RNAPIII ab), one anti-Ku antibody, one anti-PM/Scl100 antibody, two anti-PM/Scl75 antibodies, one anti-Ro52 antibody, whereas 32 patients had multiple autoantibody positivities. Among classic SSc-specific autoantibodies, anti-Scl70 and anti-RNAPIII abs showed the highest cooccurrence (n = 4). One patient was simultaneously positive for anti-RNAPIII ab and ACA, and one was positive for ACA and anti-Scl70 ab. The clinical features were not statistically different between single and multiple autoantibody-positivity for classic SSc-specific autoantibodies (ACA, anti-Scl70 ab, and anti-RNAPIII ab), except for digital ulcer in the multiantibody positive ACA group (p = .019). CONCLUSION: Based on our results, coexpression of autoantibodies is not uncommon in SSc patients. Although autoantibodies specific to SSc in early disease show generally known clinical features, it remains to be investigated how the coexpression of autoantibodies will affect clinical presentation.

Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension.

The primary systemic vasculitides are rare diseases characterized by vessel wall inflammation. Isolated pulmonary vasculitis, large-vessel vasculitis, and Behçet's disease are mimickers of chronic thromboembolic pulmonary hypertension (CTEPH); group IV pulmonary hypertension (PH) can occur as a devastating complication in the course of these diseases. Pulmonary endarterectomy, balloon angioplasty, anticoagulation and pulmonary vasodilator agents are the main treatment options for CTEPH. There is no specific recommendation for the treatment of patients having group IV PH due to primary systemic vasculitides. We reviewed herein data about group IV PH due to primary systemic vasculitides.

Treatment of systemic sclerosis-associated digital ulcers: recommendations of the Turkish Society for Rheumatology.

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 held in December 2020 with the participation of a task force consisting of 23 rheumatologists the scope of the recommendations and research questions were determined. A systematic literature review was conducted by 5 fellows and results were presented to the task force during the second meeting. The Oxford system was used to determine the level of evidence. The preliminary recommendations were discussed, modified, and voted by the task force and then by members of TRD via e-mail invitation allowing personalised access to a web-based questionnaire [SurveyMonkey®]. RESULTS: A total of 23 recommendations under 7 main headings were formulated covering non-pharmacological measures for the prevention of DUs and pharmacological treatments including vasodilators, anti-aggregants, antibiotics, wound care, pain control, and interventions including sympathectomy, botulinum toxin, and surgery. Risk factors, poor prognostic factors, prevention of DU and adverse effects of medical treatments were reported as 4 overarching principles. CONCLUSIONS: These evidence-based recommendations for the management of SSc-associated DUs were developed to provide a useful guide to all physicians who are involved in the care of patients with SSc, as well as to point out unmet needs in this field.

Pulmonary hypertension in Behçet's disease: echocardiographic screening and multidisciplinary approach.

BACKGROUND: Little is known about the prevalence and causes of pulmonary hypertension (PH) in Behçet's disease (BD). This study was conducted to determine the prevalence and causes of PH in BD. METHODS: In this descriptive study, we screened 154 patients with BD for PH using transthoracic echocardiography between February 2017 and October 2017. An estimated systolic pulmonary arterial pressure (sPAP ≥ 40 mmHg) was used as the cutoff value to define PH. Patients with BD were categorized into 5 groups according to organ involvement including mucocutaneous/ articular, ocular, vascular, gastrointestinal, and neurologic involvement. Additional laboratory and imaging results were obtained from hospital file records to determine the causes of PH. RESULTS: PH was detected in 17 (11%) patients. Nine (52.9%) of these patients had group II PH (due to left heart disease), 4 (23.5%) had IV PH (due to pulmonary arterial involvement), and 1 had III PH (due to chronic obstructive lung disease). The frequency of PH was higher in BD patients with vascular involvement than those without (52.9% vs 28.5%; p = 0.04). Among 10 patients with pulmonary artery involvement (PAI) 4 (40%) had PH. Although the vascular BD group had the highest rate of PH, we observed no statistically significant difference in the frequency of PH between the predefined BD subgroups. DISCUSSION: : PH is not rare in patients with BD. The majority of BD patients with PH are in group II or IV PH. Patients with vascularinvolvement carry a higher risk for the development of PH. Monitoring BD patients with PAI should be considered for the development of group IV PH.

Hematologic Malignancy Risk in Inflammatory Arthritis Patients Treated with TNF Inhibitors: The Real-Life Data from the HUR-BIO Registry.

INTRODUCTION: This study aimed to assess the incidence of hematologic malignancy (HM) among inflammatory arthritis (IA) patients receiving tumor necrosis factor inhibitors (TNFi) compared with the general Turkish population. METHODS: HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a single-center biological disease-modifying anti-rheumatic drug (bDMARD) registry since 2005. Patients with IA, including rheumatoid arthritis, spondyloarthritis, or psoriatic arthritis who had at least one visit after the TNFi were screened from 2005 to November 2021. Standardized incidence rates (SIR) were calculated after adjustment for age and gender and compared with the 2017 Turkish National Cancer Registry (TNCR). RESULTS: Of the 6139 patients registered in the HUR-BIO, 5355 used any TNFi at least once. The median follow-up duration was 2.6 years for patients receiving TNFi. Thirteen patients developed a HM on follow-up. In these patients, the median age at the IA onset was 38 (range, 26-67), and the median age at the HM diagnosis was 55.5 (range, 38-76). Patients using TNFi had an increased HM incidence (SIR 4.23, 95% confidence interval (CI) 2.35-7.05). Ten patients with HM were under 65 years of age. In this group, there was a higher incidence of HM in both men (SIR 5.15, 95% CI 1.88-11.43) and women (SIR 4.76, 95% CI 1.74-10.55). CONCLUSIONS: The risk of HMs in inflammatory arthritis patients receiving TNFi was four times higher than in the general Turkish population.

The effect of the endotracheal tube cuff shape on post-operative sore throat in surgeries longer than 120 min in supine position.

BACKGROUND: This study aimed at comparing the effects of cylindrical- and conical-cuff endotracheal tubes (ETTs) inflated with saline on cuff pressures, post-operative sore throat, and post-operative analgesic consumption in surgeries taking longer than 120 min. OBJECTIVE: This study aimed at comparing the effects of cylindrical- and conical-cuff endotracheal tubes (ETTs) inflated with saline on cuff pressures, post-operative sore throat, and post-operative analgesic consumption in surgeries taking longer than 120 min. METHODS: The study was performed on a total of 100 patients, aged 18-65 years, in the ASA I-III risk group, with cylindrical cuff (Group C, n = 50) and conical cuff (Group T, n = 50) ETT patients. Cuff pressure values of all patients were recorded. RESULTS: The cuff pressure values at all measurement times and maximum cuff pressure value were significantly lower in Group T than in Group C (p < 0.05). In the post-operative 24-h period, sore throat and total analgesic consumption were significantly lower in Group T than in Group C (p < 0.05). CONCLUSION: conical cuff ETTs prevent the increase in intraoperative cuff pressures, reduce the incidence of post-operative sore throat, and accordingly reduce post-operative analgesic consumption compared to cylindrical cuff ETTs.

ANTECEDENTES: El objetivo de este estudio fue comparar los efectos de los tubos endotraqueales (ETT) de manguito cilíndrico y cónico inflados con solución salina sobre las presiones del manguito, el dolor de garganta postoperatorio y el consumo de analgésicos postoperatorios en cirugías de más de 120 min de duración. OBJETIVO: Comparar los efectos de los tubos endotraqueales de manguito cilíndrico y cónico inflados con solución salina sobre las presiones del manguito, el dolor de garganta posoperatorio y el consumo de analgésicos posoperatorios en cirugías de más de 120 minutos de duración. MÉTODO: El estudio se realizó en 100 pacientes con edades entre 18 y 65 años, con riesgo ASA I-III, con tubo endotraqueal de manguito cilíndrico (grupo C, n = 50) o manguito cónico (grupo T, n = 50). RESULTADOS: Los valores de presión del manguito en todos los tiempos de medición y el valor máximo de presión del manguito fueron significativamente más bajos en el grupo T que en el grupo C (p < 0.05). En el período de 24 horas del posoperatorio, el dolor de garganta y el consumo total de analgésicos fueron significativamente menores en el grupo T que en el grupo C (p < 0.05). CONCLUSIONES: Los tubos endotraqueales de manguito cónico previenen el aumento de las presiones intraoperatorias del manguito, reducen la incidencia de dolor de garganta posoperatorio y, en consecuencia, disminuyen el consumo de analgésicos posoperatorios en comparación con los de manguito cilíndrico.

The impact of antiphospholipid antibodies in Takayasu arteritis.

BACKGROUND: The significance of antiphospholipid antibodies (aPL) is controversial in Takayasu arteritis (TA). This study was conducted to explore the frequency of aPL and their association with disease-related complications in TA. METHODS: : This cross-sectional study was conducted to investigate the presence of anti-cardiolipin (aCL), anti-beta 2 glycoprotein- 1(aß2G1) antibodies, and lupus anticoagulant (LA) in TA patients. TA patients admitted to the Department of Rheumatology of Hacettepe University Faculty of Medicine between December 2015 and September 2016 who fulfilled the American College of Rheumatology (ACR) classification criteria for TA were consecutively enrolled in the study. Patients were grouped according to aPL positivity and compared in terms of disease manifestations, type of vascular involvement at diagnosis, and vascular complications/interventions attributable to TA. RESULTS: Fifty-three TA (49 female) patients were enrolled in the study. We detected 9 (16.9%) patients with IgM and/or IgG aß2G1 and/or LA positivity. There were no patients with positive aCL. All aß2G1 titers were low. There were no differences in terms of symptoms, signs, type of vascular involvement, the number of patients with disease-related complications or vascular interventions/surgery between aPL (+) and aPL(-) groups (p > 0.05 for all). The number of patients with thrombotic lesions was similar between the groups (p > 0.05). There were no patients with a history of venous thrombosis or on anticoagulant treatment in the aPL(+) group. Only 1 patient with IgM aß2G1 (+) had a history of pregnancy loss. DISCUSSION: Our results indicate that aPL positivity is not rare in TA. On the other hand, all aPL titers were low and no differences were found in the frequency of disease-related complications between aPL(+) and aPL(-) patient groups. Only TA patients with atypical manifestations with high suspicion of aPL-related complications should be considered to be investigated for aPL.

Association between oxygen delivery and digital ulcers in systemic sclerosis.

OBJECTIVE: Tissue hypoxia due to microvasculopathy is the main cause of digital ulcers (DUs) in systemic sclerosis (SSc). Reduced oxygen delivery (DO2) to the tissues may also contribute to the development of DU. This study was conducted to investigate the association between DO2 and DUs in patients with SSc. METHODS: In all, 111 patients and 30 healthy controls were enrolled. DO2 was calculated by using the formula; DO2 = Cardiac output × arterial oxygen saturation (SpO2) × serum haemoglobin level × 1.39 × 10. Both right index finger SpO2 measurements (index-SpO2) and highest value of SpO2 (maximum SpO2) obtained among the fingers of the subjects were used for the calculations and DO2 results were adjusted both for weight and body surface area (BSA). RESULTS: Mean DO2 was lower in SSc patients as compared to controls in all 4 different calculations but the difference was only statistically significant when using index-SpO2 and adjusting for BSA (498 mL/min/m2 vs 549 mL/min/m2, p = 0.03). There was a strong positive correlation between cardiac output and DO2 calculated by using the index-SpO2 (r = 0.903; p < 0.001). Of the SSc patients, 46 (41.4 %) had DUs within the last 12 months. Patients with DUs had higher mean mRSS, lover mean FVC and more frequently diffuse disease, interstitial lung disease, anti-SCL70 antibody positivity (p < 0.05 for all). No difference was observed in DO2 among DU positive or DU negative groups by any calculation (p > 0.05 for all). CONCLUSIONS: DO2 in SSc patients seems to be lower than healthy controls. However, DO2 is similar between the patients with and without DUs. Our results suggest that the contribution of DO2 is negligible to the development of DU and support the major role of microvasculopathy in SSc patients with DUs.

Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease.

BACKGROUND: To explore the frequency and clinical associations of radiologic pleuroparenchymal fibroelastosis (PPFE) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: In this single-center retrospective study, high resolution computed tomography (HRCT) images of 105 patients with SSc-ILD were examined for the presence of PPFE. Demographic, clinical, laboratory, and pulmonary function test (PFT) data of patients with and without PPFE were compared. RESULTS: PPFE was detected in 19 (18.1%) patients ('definite PPFE' in 13 and 'consistent with PPFE' in 6 patients). Patients with PPFE had higher age and longer disease duration than PPFE (-) patients (p < 0.05 for both). Radiologic usual interstitial pneumoniae (UIP) pattern was more frequent (26.3% vs. 4.7%, p = 0.01) and median force vital capacity (FVC) was lower in patients with PPFE (64% vs. 82%, p = 0.005). Spontaneous pneumothorax developed in one patient with PPFE. More deaths occured in PPFE (+) group during follow-up (31% vs. 11%, p = 0.04).