Rheumatology International最新文献

Giant cell arteritis (GCA) is a medium and large vessel vasculitis. Vision loss is considered the most serious complications traditionally attributed to untreated disease. Urgent corticosteroids (CS) therapy is the standard of care and is considered adequate to prevent blindness. However, in some rare cases blindness may occur despite implementation of the appropriate treatment. We aimed to find patients who went blind despite high dose CS therapy and identify unique features of them together with conducting narrative review of previous case reports to characterize this group in search of common potential risk factors. Cases of blindness prior to treatment induction were not analyzed here. We identified 2 patients in our records who went blind despite high dose CS therapy. We found some repeated common features in patients that went blind in spite of treatment: advanced age, preexisting pronounced arteriosclerosis, thrombocytosis, contraindication to full dose CS therapy resulting in lower doses of CS within the recommended ranges. Defining the subgroup of GCA patients that went blind in spite of proper CS treatment requires further attention as they might potentially benefit from more aggressive therapy (e.g. early introduction of disease-modifying antirheumatic drugs).

Inflammatory rheumatic diseases (IRDs) compromise vascular integrity through systemic inflammation and (auto) immune reactions, which are associated with an increased risk of vascular complications. Venous stasis, endothelial dysfunction, and coagulation imbalance are the primary pathophysiological factors behind thrombotic events in these individuals. The incidence of venous thromboembolism (VTE) in IRDs is higher than in the general population, although the magnitude of this increase varies across diseases. Inflammatory damage to vessel walls, diminished elasticity, and compromised muscle pump function may contribute to the formation of varicose veins (VVs). A sedentary lifestyle, decreased muscular strength, and weight gain worsen the condition, particularly by adversely affecting venous return in the lower extremities. Consequently, the prevention of vascular issues in IRDs should be facilitated by both pharmaceutical interventions and rehabilitation with lifestyle modifications. A multidisciplinary rehabilitation strategy-encompassing regular physical activity, compression therapy, inflammation management, weight control, and patient education-enhances venous return, mitigates thrombosis risk, and improves quality of life.

The aim of this study is to identify factors associated with kinesiophobia in patients with systemic sclerosis (SSc). A total of 72 adult patients diagnosed with SSc were included in this cross-sectional study. Clinical parameters reflecting disease severity, organ involvement, and inflammatory status were recorded. Kinesiophobia level was assessed using the Tampa Scale for Kinesiophobia (TSK). The Berg Balance Scale (BBS), Y Balance Test (YBT), Timed Up and Go Test (TUG), and 10-Meter Walk Test (10MWT) were used to evaluate balance and functional capacity. Factors associated with kinesiophobia was analyzed using multiple linear regression analysis. Kinesiophobia (TSK ≥ 37) was identified in 26 patients (36.1%). Disease related parameters, including disease duration, disease activity, comorbidity burden, skin and other organ involvement (lung, gastrointestinal) were higher in patients with kinesiophobia (p < 0.05, for all). Patients with kinesiophobia had significantly lower BBS score, slower walking speed, longer TUG duration, and were unable to perform the YBT, compared to those without kinesiophobia (all p < 0.001). In the multivariable regression analysis, (β = - 1.26, CI - 1.59 to - 0.94, p < 0.001) and TUG (β = 1.24, CI 0.04-2.43, p = 0.043) showed to be associated with kinesiophobia severity independently. The multivariable model accounted for 65% of the variance in kinesiophobia (adjusted R² = 0.65). In patients with SSc, static imbalance and decreased mobilization capacity are closely associated with kinesiophobia. Monitoring these parameters should be prioritized for the management of kinesiophobia in patients with SSc.

Fibromyalgia is a widespread chronic pain condition affecting quality of life. Emotional distress and loneliness are factors commonly related to pain and suffering. However, no study has analyzed how all these variables interplay in individuals living with fibromyalgia. The study objective was to empirically test the relationships among loneliness, anxiety, depression, pain, and suffering in this population. Descriptive, observational, cross-sectional study. Structural equation modeling (SEM) was used to assess the relationships between loneliness, anxiety, and depression to the experience of pain and suffering in 317 fibromyalgia patients who attended a specialized pain management facility. Loneliness was positively associated with anxiety, pain, depression, and suffering. Moreover, in our sample, anxiety, depression, suffering, and pain demonstrated significant covariation. We also found that depression is positively related to pain and suffering. Our study demonstrates that loneliness in fibromyalgia is closely linked to anxiety, depression, pain, and suffering. Regular, comprehensive interventions that incorporate social support strategies may help reduce this burden.

IgG4-related disease (IgG4-RD) is a rare immune-mediated, fibroinflammatory disease with heterogenous presentations and no standardized treatment recommendations. This study investigates long-term efficacy and safety of current therapeutic strategies with a focus on rituximab. A retrospective analysis was conducted on 24 patients diagnosed with IgG4-RD at a German tertiary center (2010-2020) using the 2020 revised comprehensive diagnostic criteria. Patients were recruited from Rheumatology and Nephrology. Data included organ involvement, laboratory results, histology, treatments, relapses, therapy-related damage, and comorbidities. The cohort included 12 males and 12 females, median age at diagnosis 53 (95%CI 37.0; 61.0) years. Males had more affected organs (2.4 vs. 1.6; p = 0.036) and higher IgG4 levels (>5× upper limit: 33.3% vs. 16.7%; p = 0.036). Immunosuppressive therapy was initiated in 87.5% of patients, with glucocorticoids (GC) universally included. Rituximab was administered to 71.4%, mainly as a 4 x 375 mg/m² regimen (77.3%), with a median follow-up post first rituximab of 51.0 months (95%CI 27.0; 63.0). Adverse events were not more frequent with rituximab compared to other regimens. At last visit, 47.6% were off immunosuppressives and 38.1% remained on GC. Active organ involvement declined, though 16.7% showed organ damage progression. Relapses were frequent (81.0%), but less common upon rituximab initiation (26.7%). This representative IgG4-RD cohort demonstrates that long-term treatment with rituximab as maintenance therapy is generally effective and safe regardless of therapeutic regimen. Despite this glucocorticoid use remains high, highlighting the need for guidelines to standardize the use of glucocorticoids and DMARDs like rituximab in both induction and maintenance therapy.