Nationwide analysis of adult hospitalizations with hematologic malignancies and systemic sclerosis
Systemic sclerosis (SSc) is a connective tissue disease with multi-system involvement and it has an increased risk of developing hematologic malignancies. This study aims to report the association between hematologic malignancies with SSc and to characterize in-hospital demographics and outcomes in patients with hematologic malignancies with and without SSc.
We performed a retrospective review of pooled data from the National Inpatient Sample (NIS) database from 2016 to 2020. Crude prevalence of hematologic malignancies among hospitalized patients with and without SSc was calculated. Logistic regression was used for statistical significance of differences in prevalence while adjusting for confounders. Demographic characteristics and outcomes of patients with hematologic malignancies with and without SSc was compared. Statistical analysis was done using chi-square and multivariate logistic regression.
Among all adult hospitalizations, the prevalence of hematologic malignancy was 1.87% compared to 2.66% among patients with SSc (adjusted odds ratio (aOR) 1.52, p <0.01). Relative to the non-SSc group, the SSc group had higher odds of in-patient mortality (OR 1.43; 95% confidence interval (CI) 1.11 – 1.87; p<0.01). The prevalence of lymphoma was 0.71% compared to 1.04% among patients with SSc (aOR 1.6, p < 0.01). Relative to the non-SSc group, the lymphoma-SSc group had similar odds of in-patient mortality (OR 0.93; 95% CI 0.55 – 1.59; p=0.80). The prevalence of leukemia was 0.79% compared to 1.28% among patients with SSc (aOR 1.74, p < 0.01). The leukemia-SSc group had higher odds of in-patient mortality (OR 1.78; 95% CI 1.29 – 2.46; p<0.01). For myeloma, there was no difference in the prevalence in adults with and without SSc (0.4 vs. 0.38%, aOR 0.96, p=0.64) and there was no difference of in-hospital mortality.
There is a positive significant association between hematologic malignancies including lymphoma and leukemia, and SSc. This association was not seen between myeloma and SSc. There is increased in-hospital mortality of patients with leukemia and SSc. Read online
Biotechnological therapeutic in Juvenile Idiopathic Arthritis: Pathophysiological implications and targeted therapies
In this retrospective cohort study, we aim to investigate the most used biological disease modifying anti-rheumatic drugs (bDMARDs) in Juvenile Idiopathic Arthritis (JIA) patients in a pediatric rheumatologic unit from a Portuguese tertiary hospital, along with their effectiveness and safety. We also intended to link their effectiveness and the pathophysiology of the disease.
The medical records of JIA patients exposed to bDMARDs, between January 2018 and January 2023, in a pediatric rheumatologic unit from a Portuguese tertiary hospital were reviewed. Therapy effectiveness was accessed based on achievement of inactive disease according to Wallace Criteria. Effectiveness of different bDMARDs in the several JIA subtypes was linked to the disease´s pathophysiology. Adverse effects were also reviewed.
Thirty-four patients were included in the study. Overall, nineteen patients (67,9%) had inactive disease at last evaluation. Six patients with missing data on inactive disease status were excluded from this analysis. Number of affected joints, ESR and CRP were significantly lower at 3, 6, 12 and 24 months after bDMARD therapy. All systemic JIA patients (n=10) were initially treated with Anakinra. Six (60%) achieved inactive disease. Two (20%) switched to Tocilizumab due to ineffectiveness in the control of articular features. Patients who switched to tocilizumab achieved inactive disease until the end of the follow-up. All patients with the other subtypes of JIA (n=24) were treated with TNF inhibitors. Inactive disease was achieved in 55,6%. Adverse effects occurred in eight patients (23,5%).
The results of the present study demonstrate the effectiveness of bDMARs in the study population. bDMARDs reduced the number of affected joints, CRP and ESR after three months of treatment, and this effectiveness was sustained over the two years of follow-up. For systemic JIA, preferred drug was Anakinra, an interleukin 1 inhibitor, and its effectiveness was consistent with previous studies. In the other JIA subtypes, TNF inhibitors were the most used bDMARDs, and showed an effectiveness consistent with previous studies. The most used bDMARDs for each JIA subtype are in line with pathophysiological differences. Our results demonstrated the safety of these drugs. Read online
Translation and cross-cultural adaptation of the modified Short QUestionnaire to Assess Health-enhancing physical activity (mSQUASH) into Turkish
The aim was to translate and cross-culturally adapt the modified Short Questionnaire to Assess Health-enhancing physical activity (mSQUASH) into Turkish
The mSQUASH was translated into Turkish and backward-translation into Dutch was performed afterwards using the Beaton method. After the Turkish version was reviewed and revised by an expert committee that included translators, two patients and the research team a pre-final version was produced. The-pre final version then entered a field-test with cognitive debriefing in 10 patients with axSpA. The final result was the Turkish mSQUASH version.
The translation process went without difficulties. Small discrepancies were either resolved during the synthesis or expert consensus meetings. Mean (SD) time to complete the mSQUASH was 6.1 (2.4) minutes in field-test procedure. The cognitive debriefing showed that the items of the Turkish mSQUASH were clear, relevant, easy to understand and easy to complete. None of the patients reported that an important aspect of physical activity was missing from the questionnaire items. Patients raised the concern that not all sport examples were culturally suitable; tennis was replaced by volleyball and basketball after the cognitive debriefing, to make it more appropriate to the Turkish culture.
The final Turkish version of the mSQUASH showed acceptable linguistic and field validity for use in both clinical practice and research. However, further assessment of the psychometric properties (validity and reliability) of the Turkish version of the mSQUASH is needed before it can be implemented.