Rheumatology and immunology research最新文献

Background and objectives: The functional disability status of Indian children with juvenile idiopathic arthritis is unidentified. In this cross-sectional study functional capacity of 60 juvenile idiopathic arthritis patients was assessed by the Childhood Health Assessment Questionnaire.

Methods: A total of 60 juvenile idiopathic arthritis patients aged ranges from 1 to 12 years were recruited from a teaching hospital in eastern India. A childhood health assessment questionnaire was used to assess the functional health of children. Pain, patient's/parent's global assessment of general well-being, and physician's global assessment were assessed.

Results: Childhood health assessment questionnaire disability index for oligoarticular juvenile idiopathic arthritis differed significantly from polyarticular juvenile idiopathic arthritis (P < 0.001), systemic-onset juvenile idiopathic arthritis (P = 0.018) and undifferentiated juvenile idiopathic arthritis (P < 0.001). There was a good to a strong positive correlation between the childhood health assessment questionnaire disability index with pain score, patient's/parent's global assessment score, and physician global assessment score for the total juvenile idiopathic arthritis cohort. regarding juvenile idiopathic arthritis subtypes, significant correlations were noted between the childhood health assessment questionnaire disability index with the patient's/parent's global assessment and physician's global assessment (except for enthesitis-related arthritis).

Conclusions: Assessment and documentation of the functional health status of juvenile idiopathic arthritis patients will improve the management of the disease.

Background and objectives: Left ventricular systolic dysfunction (LVSD) is a cardiac involvement that is the leading cause of death among patients with systemic sclerosis (SSc). We aimed to define the clinical course and predictors of LVSD among SSc patients.

Methods: We conducted a cohort study among adult patients with SSc who were followed up from 2013 to 2020. Semiparametric Cox regression analysis with robust clustering by cohort identification number was used to evaluate the predictors of LVSD.

Results: Among the 3, 987 person-years, LVSD was defined in 35 of 419 SSc patients for an incidence of 0.88 per 100 person-years. The median duration of the disease was 8.5 (interquartile range (IQR) 4.9-12.9) years. Every 1-point increase in the modified Rodnan skin score (mRSS) and salt and pepper skin were strong predictors of LVSD, with a respective adjusted hazard ratio (HR) of 1.05 and 3.17. During follow-up, 26 cases (74.3%) had unimproved LVSD. The strong predictors of the unimprovement of LVSD were every 1-point increase in mRSS (HR 1.05), every 1 mg increase in prednisolone treatment (HR 1.05), and every 1 U/L increase in creatine kinase (CK) (HR 1.001). Mycophenolate treatment was a protective factor against the unimprovement of LVSD in SSc (HR 0.15).

Conclusions: LVSD was frequently found in patients with diffuse cutaneous SSc, and in most cases, it remained unimproved during follow-up. High mRSS, steroid use, and high CK levels were predictors of unimproved LVSD, whereas mycophenolate treatment might prevent the progression of LVSD. Steroids should be prescribed with caution in patients with longer disease duration.

Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), even though often underdiagnosed. SSc-pHI has been recently defined as cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications. SSc-pHI represents a major determinant of mortality in SSc, accounting alone for about 12% of disease-related deaths; its early recognition and promptly therapeutic interventions are therefore crucial. Both perfusion defects and myocardial inflammation contribute to the occurrence of myocardial fibrosis that precipitates myocardial remodeling, potentially leading to heart failure and arrhythmic complications. To date, clear evidence and guidelines for effectively managing SSc pHI are not established yet, resulting in a lack of a defined therapeutic algorithm. In this review we summarize the most recent scientific literature on the prevailing therapeutic strategies and interventions to manage SSc-pHI, with particular focus on therapeutic strategies to counteract the 3 major pathogenic events of the disease, i.e. microvascular damage, myocardial inflammation and myocardial fibrosis.

Background and objectives: The clinical course, the outcomes of myocarditis, and the imaging progression of cardiac magnetic resonance imaging (MRI) in systemic sclerosis (SSc) are still unknown. We aimed at defining changes in cardiac MRI findings, the clinical course, and the outcomes of SSc patients previously defined as having myocarditis by cardiac MRI. Methods: This prospective cohort study included SSc patients, who had previously been diagnosed with myocarditis through cardiac MRI at the Scleroderma Clinic of Khon Kaen University, between 2018 and 2020 and had had annual follow-ups of cardiac MRI for at least 3 years. Data on demographics, clinical characteristics, cardiac MRI findings, treatment regimens, and outcomes were collected. Serial cardiac MRI on a yearly basis was analyzed to assess changes in myocardial involvement over the 3-year period.

Results: Ten SSc patients diagnosed with myocarditis via cardiac MRI were included. Most belonged to the diffuse cutaneous subset with a mean age of 58.3±8.6 years and were mildly symptomatic. Initial cardiac MRI findings showed myocardial edema and hyperemia in all patients and eight patients had had pre-existing myocardial scars, suggesting disease chronicity. Treatment for concomitant interstitial lung disease involved steroids with either cyclophosphamide or mycophenolate mofetil in 6 patients. Outcomes of myocarditis were stable, improving, and worsening in 4, 4, and 2 patients, respectively. There was no complete resolution of the cardiac MRI indices for myocarditis, and none had had major cardiac events.

Conclusion: Although SSc myocarditis on cardiac MRI may improve or show stability, the changes remained persistent. Among patients with SSc and mildly symptomatic myocarditis, the efficacy of steroids and immunosuppressive therapy is inconclusive. Over a 3-year follow-up, the prognosis had been acceptably good with no cardiac events.

Background and objectives: Psoriasis (PsO) and psoriatic arthritis (PsA) are often undertreated and require a multidisciplinary approach. In recent years, patent expiration has allowed the introduction of tumor necrosis factor inhibitor (anti-TNF) biosimilars, which have stimulated a significant increase in the use of biological therapies. This article reports the findings of a multidisciplinary approach to achieve a consensus on the use of adalimumab in patients with PsO or PsA.

Methods: A voting panel of 36 Italian dermatologists and rheumatologists were chosen by eight Italian clinicians (the Board), to provide a consensus on the real-world management of PsO and PsA with adalimumab using the Delphi Method, comprising three survey rounds. Twelve statements were defined by the Board and submitted to the panel (rating scale 1-7).

Results: Clinicians reached a wide consensus on the effectiveness (score 6-7: 67%) and long-term efficacy (6-7: 100%) of adalimumab in all clinical forms of PsO and PsA, including pediatric patients (6-7: 85%). Considering cost-effectiveness and safety, adalimumab is suggested as a first-line treatment in patients with enthesitis, predominant peripheral arthritis, axial involvement or associated inflammatory bowel disease (IBD) or uveitis. Adalimumab can be also considered after failure of etanercept (6-7: 94%).

Conclusion: Results from this Delphi study clearly show an overall consensus on the use of adalimumab in the management of PsO and PsA, particularly as first-choice for specific subpopulations (uveitis, IBD, hidradenitis suppurativa). Considering the cost-effectiveness of biosimilars within Italy, adalimumab may represent an effective and safe first-line treatment for patients with moderate-to-severe PsO or PsA, and a valid choice for switching after failure.

Systemic autoinflammatory diseases (SAIDs) are distinct from autoimmune diseases. The former primarily results from abnormal innate immune response and genetic testing is crucial for disease diagnosis. Similar cutaneous involvement is a main feature for both SAID and dermatomyositis (DM), so they can be confused with each other. A literature search of PubMed and MEDLINE was conducted for relevant articles. The similarities and differences between these two types of diseases were analyzed. We found phenotypic similarities between these two types of disorders. Accumulating data supports a major role of the innate immune system and a similar cytokine profile. Molecular testing using an autoinflammatory disease gene panel may help identify SAID patients from the DM population and may offer therapeutic benefit using interleukin-1 (IL-1) inhibitors. A subset of DM, notably amyopathic dermatomyositis in the absence of autoantibodies may be on the spectrum of autoinflammatory disease.

Spondyloarthritis (SpA), rheumatoid arthritis (RA), and inflammatory bowel diseases (IBD) are chronic inflammatory autoimmune diseases that are associated with alterations in the composition of the intestinal microbiota (i.e., dysbiosis). For SpA and RA, a gut-joint-enthesis axis is hypothesized and recent data suggests that dysbiosis may contribute directly to initiating and perpetuating joint and spine inflammation. Biologic drugs targeting tumor necrosis factor (TNF) are effective in treating these diseases and have been shown to partially restore the disrupted microbiome. Hence, drugs that affect both the intestinal and joint components of these diseases, such as anti-TNF drugs, may act on the intestinal microbiome. However, despite the remarkable efficacy of anti-TNF-α treatments, non-responders are frequent, and predictors of patient outcomes have not been identified. In this narrative review, we summarize recent research on the downstream effects of anti-TNF drugs on the intestinal microbiota in SpA, RA, and IBD. We also discuss whether these changes could have a role as predictive biomarkers of anti-TNF response.