Analysis of 5-year hospitalization data of patients with systemic lupus erythematosus: Damage is a risk factor for frequent and longer stays.

OBJECTIVE: The annual hospitalization rate of patients with systemic lupus erythematosus (SLE) is approximately 10%, and hospitalizations are responsible for most of the healthcare expenses. Herein, we analyzed 5-year hospitalization data of SLE patients and determined factors leading to hospitalization. METHODS: Clinical, laboratory, and hospitalization data of SLE patients admitted to our rheumatology clinic in 2015-2020 were retrieved from our SLE database and analyzed. SLICC SLE damage index (SDI) and disease activity at admission (SLEDAI-2K) were determined. RESULTS: Among 161 hospitalized patients, 86% were females. Total rheumatologic hospitalization number was 298, and 38% of the patients were hospitalized more than once (1.85 ± 1.56). The mean hospitalization duration covering all stays for each patient was 25 ± 26.5 days. Active disease, infection, and damage-related complications were first three causes of hospitalization. Compared to patients hospitalized for active disease or damage, patients hospitalized for infection had a significantly higher number of readmissions (p < .05) and their total hospital stay was longer (p < .01).The frequency of patients with damage and the mean SDI score was significantly lower in the active disease group (68%, 1.93 ± 2.05) than hospitalizations for infection (90%, 2.68 ± 1.63) and damage-related causes (96%, 3.04 ± 1.65) (p < .05). The mean SDI score and duration (r = 0.551, p < .001) and the number of hospitalizations (r = 0.393, p < .001) were positively correlated. The mean disease activity scores of patients hospitalized for active disease, infection, and damage-related reasons were 11.03 ± 6.08, 3.21 ± 2.80, and 2.96 ± 3.32, respectively (p < .001). Renal active disease was the most common (44%), followed by hematological (34.8%), articular (21.7%), and mucocutaneous (21%) activity.Ten percent of the patients all of whom had damage were admitted to intensive care unit (ICU). Total hospitalization duration, mean SDI, antiphospholipid syndrome, lupus anticoagulant, thrombocytopenia, serositis, pulmonary hypertension, history of alveolar hemorrhage, and cardiac valve involvement were associated with ICU admission (p < .05 for all). CONCLUSION: Disease activity, infections, and damage are the leading causes of hospitalization in SLE patients. Damage prolongs hospital stay and increases hospitalization rate and ICU need. Tight control of disease activity with rational use of immunosuppressive treatment is important to reduce damage and hospitalizations.

Utility of positron emission tomography as a new tool for muscle involvement in patients with idiopathic inflammatory myositis: a controlled study.

OBJECTIVES: Idiopathic inflammatory myositis (IIM) represents a rare group of disease that can affect multiple organs in addition to the muscles. 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is an emerging scanning method that is widely used in diagnosing, staging and response to treatment in patients with cancer. In this study, we aimed to evaluate the muscle involvement in PET/CT which was performed for malignancy screening and its correlation with myositis-specific antibodies (MSA) and/or myositis-associated antibodies (MAA) in patients with IIM. METHODS: IIM patients who fulfilled 2017 EULAR/ACR classification criteria and had PET-CT scans during the active phase of myositis (within two weeks of starting steroids) were included into the study. Age and sex matched participants with history of malignancy (non-IIM patients) were defined as control group. RESULTS: Data of 160 IIM patients were evaluated and 34 patients (of 64.7% female) whose PET/CT results were available, included into the study. Fourteen patients with diagnosis of malignancy without IIM (non-IIM patients) defined as the control group. Sensitivity and specificity of a positive FDG muscle uptake were 37.1% and 100%, 65.7% and 92.9%, 91.4% and 7.1% compared to liver, mediastinum and LTM uptakes, respectively. In multivariate analysis, higher baseline CRP (p=0.017, confidence interval [CI] 95%: 1.03-1.36, OR:1.18) and LDH (p=0.029, CI 95%:1.001-1.017, OR:1.01) levels were associated with muscle PET/CT positivity. CONCLUSIONS: In patients with active IIM, median muscle FDG uptake with PET/CT was higher compared to non-IIM. PET/CT may be used for the evaluation of extent and activity in patients with IIM.

AA Amyloidosis: A Contemporary View.

PURPOSE OF REVIEW: Amyloid A (AA) amyloidosis is an organ- or life-threatening complication of chronic inflammatory disorders. Here, we review the epidemiology, causes, pathogenesis, clinical features, and diagnostic and therapeutic strategies of AA amyloidosis. RECENT FINDINGS: The incidence of AA amyloidosis has declined due to better treatment of the underlying diseases. Histopathological examination is the gold standard of diagnosis, but magnetic resonance imaging can be used to detect cardiac involvement. There is yet no treatment option for the clearance of amyloid fibril deposits; therefore, the management strategy primarily aims to reduce serum amyloid A protein. Anti-inflammatory biologic agents have drastically expanded our therapeutic armamentarium. Kidney transplantation is preferred in patients with kidney failure, and the recurrence of amyloidosis in the allograft has become rare as transplant recipients have started to benefit from the new agents. The management of AA amyloidosis has been considerably changed over the recent years due to the novel therapeutic options aiming to control inflammatory activity. New agents capable of clearing amyloid deposits from the tissues are still needed.

Evaluation of baseline F-18 FDG positron emission tomography in the diagnosis and assessment of giant cell arteritis.

Background/aim: The aim of this study is to evaluate the baseline F18-FDG PET/CT findings of individuals diagnosed with giant cell arteritis (GCA) and to explore its association with clinical findings and classification criteria. Materials and methods: We analysed data from patients who underwent F18-FDG PET/CT scans to investigate large vessel (LV) involvement between 2010 and 2019. Only patients with a clinical diagnosis of GCA and at least 6 months of follow-up were included. We compared initial clinical features and laboratory findings based on the presence of LV vasculitis on PET/CT and the maximum standard uptake value (SUVmax) of vascular territories. Results: Twenty-nine patients (median age at diagnosis: 70, F/M: 24/5) were included in the study. Among them, 21 patients (72.4%) presented with cranial symptoms, while 8 patients (27.5%) had isolated LV-GCA. Twenty-two patients (75.9%) met the ACR/EULAR 2022 GCA classification criteria. LV vasculitis was detected on PET/CT in 23 patients (79.3%). A positive correlation was observed between SUVmax in the thoracic aorta and both CRP and ESR levels (r = 0.50, p = 0.026 and r = 0.63, p = 0.002, respectively). PET/CT positive patients were found to be younger (p = 0.016) and more frequently female (p = 0.017). They also exhibited fewer headaches (56.5% vs. 100%, p = 0.04), experienced fewer flares during follow-up (p = 0.03), and had a lower cumulative glucocorticoid dose at the 6th month (p = 0.036). Comparison of PET/CT-positive patients (n = 23) based on the fulfilment of the ACR/EULAR 2022 classification criteria revealed that patients who met these criteria were older (p = 0.02) and had significantly lower CRP levels at diagnosis (p = 0.02). Conclusion: The performance of F18-FDG PET/CT in diagnosing LV involvement in GCA is favourable, and the severity of FDG uptake in the vessel wall correlates with the acute phase response. Patients with extracranial involvement on PET/CT exhibit distinct features, including a younger age and female predominance. Additionally, these patients appear to experience fewer relapses and require lower doses of glucocorticoids. However, the clinical significance of PET/CT in patients who met ACR/EULAR classification criteria, predominantly consisting of patients with ischemic cranial symptoms, could not be determined in our study.

Serum and urine interferon-inducible protein 10, galectin-9, and SIGLEC-1 as biomarkers of disease activity in systemic lupus erythematosus.

Background/aim: In this prospective study, we aimed to investigate the association of serum (s) and urine (u) IP-10, galectin-9, and SIGLEC-1 with disease activity in patients with systemic lupus erythematosus (SLE). Materials and methods: Sixty-three patients with active SLE (31 renal, 32 extrarenal) were included. Thirty patients with inactive SLE (15 renal, 15 extrarenal), 17 with renal active AAV, and 32 healthy volunteers were selected as control groups. Serum and urine IP-10, galectin-9, and SIGLEC-1 were tested using ELISA. Results: Levels of sIP-10 (p = 0.046), uIP-10 (p < 0.001), sGalectin-9 (p = 0.03), and uSIGLEC-1 (p = 0.006) were significantly higher in active SLE group compared to the inactive SLE; however, no differences were detected in the comparison of uGalectin-9 (p = 0.18) and sSIGLEC-1 (p = 0.69) between two groups. None of the biomarkers discriminated patients with active renal SLE from active extrarenal SLE. ROC analyses revealed an AUC of 0.63 (0.52-0.73) for sIP-10, 0.78 (0.68-0.86) for uIP-10, 0.64 (0.53-0.74) for sGalectin-9, and 0.68 (0.57-0.77) for uSIGLEC-1 in discriminating disease activity in SLE, which did not outperform C3 (0.75, 0.64-0.84) and C4 (0.72, 0.61-0.82). sIP-10 (p = 0.001), uIP-10 (p = 0.042), and uGalectin-9 (p = 0.009) were significantly increased in patients with active renal SLE compared to active renal AAV. sGalectin-9 (p < 0.001) and sIP-10 levels (p = 0.06) were decreased after 8 (5-22.5) months of treatment. Conclusion: sIP-10, uIP-10, sGalectin-9, and uSIGLEC-1 reflect global disease activity in SLE but do not outperform C3 and C4. sIP-10 and uIP-10 may be specific for active SLE compared to active AAV. sIP-10 and sGalectin-9 might be valuable in monitoring response after treatment.

Thrombotic risk assessment in antiphospholipid syndrome: do noncriteria antibodies contribute?

Background/aim: In this cross-sectional study, it was aimed to test the predictive value of noncriteria antiphospholipid antibodies (aPL) in addition to the global antiphospholipid syndrome score (GAPSS) in predicting vascular thrombosis (VT) in a cohort of patients with APS and aPL (+) systemic lupus erythematosus (SLE). Material and methods: This study included 50 patients with primary APS, 68 with SLE/APS, and 52 with aPL (+) SLE who were classified according to VT as VT ± pregnancy morbidity (PM), PM only or aPL (+) SLE. Antiphospholipid serology consisting of lupus anticoagulant (LA), anticardiolipin (aCL) immunoglobulin G (IgG)/IgM/IgA, antibeta2 glycoprotein I (aß2GPI) IgG/IgM/IgA, antiphosphatidylserine/prothrombin (aPS/PT) IgG/IgM and antidomain-I (aDI) IgG was determined for each patient. The GAPSS and adjusted GAPSS (aGAPSS) were calculated for each patient, as previously defined. Logistic regression analysis was carried out with thrombosis as the dependent variable and high GAPSS, aCL IgA, aß2GPI IgA, and aDI IgG as independent variables. Results: The mean GAPSS and aGAPSS of the study population were 11.6 ± 4.4 and 9.6 ± 3.8. Both the VT ± PM APS (n = 105) and PM only APS (n = 13) groups had significantly higher GAPSS and aGAPSS values compared to the aPL (+) SLE (n = 52) group. The patients with recurrent thrombosis had higher aGAPSS but not GAPSS than those with a single thrombotic event. The computed area under the receiver operating characteristic curve demonstrated that a GAPSS ≥13 and aGAPSS ≥10 had the best predictive values for thrombosis. Logistic regression analysis including a GAPSS ≥13, aCL IgA, aß2GPI IgA, and aDI IgG showed that none of the factors other than a GAPSS ≥13 could predict thrombosis. Conclusion: Both the GAPSS and aGAPSS successfully predict the thrombotic risk in aPL (+) patients and aCL IgA, aß2GPI IgA, and aDI IgG do not contribute to high a GAPSS or aGAPSS.

Efficacy of rituximab on disease activity, severity, and disease-related damage in patients with immunosuppressive-resistant systemic sclerosis.

Background/aim: B-cell depletion with rituximab (RTX) is widely used as a rescue therapy in patients with systemic sclerosis (SSc). The aim herein was to analyze the progress of disease-related outcomes after RTX therapy in severe SSc patients. Materials and methods: Included in this study were 27 SSc patients who were followed-up between 2012 and 2020 and received at least 1 cycle of RTX for active disease, despite receiving standard immunosuppressives (ISs). In addition to the European Scleroderma Study Group and European Scleroderma Trials and Research Group activity scores, Medsger's severity, and the recently developed Scleroderma Clinical Trials Consortium Damage Index values were evaluated initially and at 1 year after the first infusion. The progress of individual organ damage was also assessed at the end of the follow-up period (at least 6 months after the last infusion) using the data extracted from the medical records. Results: Disease activity and severity-improved and disease-related overall damage worsened after the first year of RTX therapy (p < 0.001, p = 0.008, and p = 0.005). Some of the disease-related organ damage had improved at the end of the follow-up period, indicating its reversibility. Overall damage scores ≥11 after the first year of RTX therapy were found to be associated with mortality (p = 0.035). Conclusion: RTX contributed to reducing the activity and severity in SSc patients with severe disease, nonetheless the efficacy related to the damage was limited. High damage scores in the first year were found to be associated with mortality. Spontaneous progress of manifestations requiring a longer period to improve and irregular consecutive RTX courses might lead to difficulties in differentiation between activity and damage.

Parental attitudes in children with persistent developmental stuttering: a case-control study

Abstract Background Clinical experiences emphasize the possible role of parental attitudes and behaviours in shaping stuttering behaviors however, the number of studies in this area is still insufficient. Objective Our aims were to compare parental attitudes in children with and without stuttering and to determine the effect of parental attitudes on stuttering severity. Methods We used an age and gender matched case control design with 24 children with stuttering and 22 healthy school children. Demographic information form and Parental Attitude Research Instrument (PARI) were enrolled by the mothers. Results According to our results; there was a statistically significant difference in parental attitudes of children with and without stuttering. Our results showed that excessive maternal control of the child and the expectations of obedience from the child more frequently observed in parents of the children with stuttering. Also there was a significant positive correlation with the severity of stuttering and excessive maternal control of the child, the expectations of obedience from the child and marital conflict. Discussion In conclusion, there was an important difference in parental styles of study group and this difference was related to the severity of stuttering. Clinicans should address parental attitudes in this samples.

How women with intimate partner violence (IPV) reason about other's intentions: effect of IPV on counterfactual inferences among healthy high socioeconomic level women from Turkey

Abstract Background: Counterfactual thinking (CFT) is a specific type of human thought involving mental representations of alternatives to past situations by perceiving the immediate environment from an imagined perspective. CFT problems and deficits in counterfactual inference ability are related to psychopathologies. Objective: We aimed to assess the CFT in a sample of high sociocultural-healthy women with and without intimate partner violence (IPV) exposure to determine whether exposure to different types of IPV has effects on CFT. Methods: Three hundred thirty-six women recruited the study. Data was collected by Violence Exposure Questionnaire and Counterfactual Inference Test. Results: Compared with non-victims, physical IPV victims significantly generate fewer counter-factual thoughts when faced with a simulated scenario. In addition, the reaction of rumination (judgemental) in response to a temporal nearly happened event was significantly lower among both physical and emotional IPV victims. Among victims, deficits in the CIT is positively correlated with the number of physical, emotional and economic abuses but the degree of correlations were weak. Discussion: We demonstrated that IPV exposure is severe in healthy women at the high socioeconomic level and is associated with the decrease in CFT ability.