ARP Rheumatology最新文献

Immune-related adverse events (irAEs) associated with immune checkpoint inhibitors (ICI) are becoming more frequent with the growing use of these agents in routine clinical oncology. This mechanism enhances the anti-tumor immune response, increasing the risk of activating autoreactive T cells, and leading to immune-mediated manifestations. The spectrum of these manifestations is widely variable. ICI-associated myositis is among the most severe irAEs, being a rare but serious complication with significant morbidity and mortality, particularly when accompanied by myocarditis and/or myasthenic crisis, which can all occur concurrently and worsen clinical outcomes. We report the case of a 78-years-old man with metastatic colon adenocarcinoma who developed ICI-associated myositis with concomitant myocarditis and myasthenic syndrome. In the presented case, the use of tocilizumab, an anti-interleukin 6 (IL-6)-receptor monoclonal antibody, was effective in treating the myositis and myocarditis, but not the myasthenic component of this ICI-associated myositis.

Introduction: Methotrexate-associated pneumonitis (MTX-Pneu) is a rare, idiosyncratic hypersensitivity reaction that can occur in patients receiving methotrexate for rheumatoid arthritis. Although uncommon, MTX-Pneu may present acutely and requires prompt recognition.

Case report: A 75-year-old woman with late-onset, seropositive rheumatoid arthritis started treatment with methotrexate and prednisolone. Two weeks later, she developed fever, cough, and dyspnea. Lung ultrasound and chest computed tomography revealed bilateral pulmonary involvement. Methotrexate was discontinued and the corticosteroid dose was increased. One month later, clinical symptoms and imaging findings had fully resolved, confirming MTX-Pneu.

Conclusion: Methotrexate-associated pneumonitis is an idiosyncratic hypersensitivity reaction with a low incidence and is independent of the development of rheumatoid arthritis-associated interstitial lung disease. Early discontinuation of methotrexate and corticosteroid therapy are key to management, and prognosis is generally favorable. This case highlights the potential role of lung ultrasound as a rapid bedside tool for detection and monitoring of MTX-Pneu.

This Letter to the Editor provides a constructive methodological commentary on the recently published article "Real-world efficacy and retention of guselkumab in psoriatic arthritis." We address issues related to the completers-only analysis, interpretation of axial domain outcomes, and absence of standardized response criteria such as ASAS20/40. Recommendations are proposed to improve the validity and comparability of real-world studies in psoriatic arthritis, with emphasis on axial disease assessment and time-to-event modeling.

Adrenal involvement in antiphospholipid syndrome (APS) is rare but potentially life-threatening. Early recognition reduces morbidity and mortality. We report a 56-year-old male with chronic thrombocytopenia and prolonged activated partial thromboplastin time who developed fatigue, anorexia, and weight loss. On admission, he presented with hypotension, hyperkalemia, thrombocytopenia, anemia, and elevated inflammatory markers. After hospitalization, he underwent an extensive workup study. PET-CT revealed bilateral adrenal uptake, and hormonal tests confirmed primary adrenal insufficiency (PAI). Autoimmune studies showed persistently positive lupus anticoagulant, and high-titer antinuclear and anti-double-stranded DNA antibodies, establishing systemic lupus erythematosus and APS with secondary PAI. Treatment with mineralocorticoids, hydroxychloroquine, anticoagulation, and subsequently high-dose glucocorticoids led to clinical and laboratory improvement. Review of published cases shows that, although APS is more frequent in women, adrenal involvement occurs predominantly in men and usually presents acutely with abdominal pain, hypotension, and electrolyte imbalance. Our patient's atypical presentation delayed diagnosis. Awareness of such variable presentation is crucial, as early recognition and treatment significantly improve outcomes. Clinicians should consider APS in new-onset adrenal failure and systematically evaluate adrenal involvement in APS patients with compatible symptoms.

Objective: This study aimed to evaluate subclinical myocardial involvement in asymptomatic juvenile systemic sclerosis (JSSc) patients using conventional Doppler (CD), tissue Doppler imaging (TDI), and speckle tracking echocardiography (STE).

Methods: In this cross-sectional, retrospective study, nine asymptomatic JSSc patients and ten age- and gender-matched healthy controls were evaluated. All participants underwent echocardiographic assessment, including CD, TDI, and STE, according to standardized protocols. Right and left ventricular systolic and diastolic parameters were compared between groups.

Results: In JSSc patients, tricuspid E/A ratio was lower (p=0.012), deceleration time (DT) and ejection time (ET) were shorter (p=0.025, p=0.006), and myocardial performance index (MPI) was higher (p=0.025) compared to controls. TDI measurements also showed shorter ET (p=0.024) and increased MPI (p=0.002). Right ventricular end-diastolic and end-systolic volumes were significantly smaller (p=0.004, p=0.018), and right ventricular ejection fraction (RVEF) was lower (p=0.019) in JSSc patients. No significant differences were detected in right ventricular strain parameters or left ventricular global longitudinal strain (GLS) and circumferential strain (GCS) values between groups. However, TDI-derived MPI for the left ventricle was elevated in JSSc patients (p=0.018), suggesting early global dysfunction despite preserved left ventricular ejection fraction.

Conclusion: Subclinical right ventricular dysfunction occurs early in JSSc, even in asymptomatic patients without pulmonary hypertension. Routine use of advanced echocardiographic techniques such as STE and TDI-derived MPI assessment may facilitate earlier detection of cardiac involvement, enabling timely intervention to improve long-term outcomes.

Objectives: To assess adverse events (AE) associated with first-line therapies for rheumatoid arthritis (RA) in a real-world setting.

Material and methods: Retrospective multicenter cohort study of patients fulfilling classification criteria for RA and followed up in 66 rheumatology centers from the Rheumatic Diseases Portuguese Registry (Reuma.pt). All AE reports associated with first-line disease-modifying antirheumatic drugs (DMARDs) up to November 2024 were included. Demographic and clinical data were analyzed, and AE characteristics were investigated. Categorical and continuous variables were compared using chi-square tests and Mann-Whitney U tests, respectively. Statistical significance was defined as p < .05.

Results: Among 1 880 AE entries, 377 (20.1%) were attributed to first-line DMARDs, most commonly methotrexate (62.9%) although no information on drug dosage was available. The median age at AE occurrence was 58.6 years (IQR: 19.32), and 82% were female. A causality assessment was available in 317 reports, with 40.3% deemed "probable," 28.1% "possible," and 10.6% "definitive." Severe AE were reported in 13.2% of cases, with pulmonary involvement being the most common (20.8%).Overall, 46.7% of patients discontinued treatment for any reason. Male sex was significantly associated with severe AE (OR = 2.31; 95% CI: 1.17-4.55; p = .014), and older patients were more likely to experience severe AE (median age 65.7 vs. 57.9 years; p < .001). The most affected body organ systems were gastrointestinal (9.3%), skin (8.2%), and hematological (8.2%). The median time to AE onset from treatment initiation was 1.27 years (IQR: 2.63), and from disease onset was 8.56 years (IQR: 11.76).

Conclusions: AE related to first-line RA therapies can lead to significant clinical consequences, including treatment discontinuation. Male sex and advanced age were associated with increased AE severity. The most affected systems appear consistent with known drug safety profiles, particularly that of methotrexate; however, the absence of information regarding drug dosage precludes more detailed conclusions. These findings emphasize the need for individualized monitoring strategies and improved pharmacovigilance to optimize long-term treatment safety and adherence in RA management.

Introduction: Apart from exocrine glands involvement with sicca symptoms, several extra-glandular manifestations can occur in Sjögren's disease (SjD) such as pulmonary manifestations. Interstitial lung disease (ILD) is the most common lung manifestation in SjD. We aim to evaluate the presence of ILD in a national cohort of patients with SjD, identify variables associated with its development and progression, as well as describe the treatment used for SjD-ILD and its effectiveness and tolerability.

Methods: We conducted an observational multicenter study of SjD-ILD patients prospectively followed in Reuma.pt. Demographic and clinical data were collected. We compared patient characteristics between groups using Chi-square or Fisher's exact test, Mann-Whitney or independent samples t-test, as appropriate. Logistic regression analysis was used to identify predictors of SjD- ILD. A linear mixed model with random intercept was used to compare results from pulmonary function tests (PFTs) before and after immunosuppression initiation.

Results: Of the 1532 patients enrolled in the Reuma.pt-SjD protocol, 1333 (87%) had information on the presence of pulmonary manifestations. Among these, 127 (9.5%) had documented lung involvement, with ILD being the most common manifestation (74%). Ever smoking (OR=2.175; [95%CI:1.214-3.899]; p=0.009) and older age at SjD diagnosis (OR=1.047 per year; [95%CI:1.025-1.069]; p<0.001) were predictors of ILD. Nonspecific interstitial pneumonia was the most frequent ILD pattern (45.7%). Immunosuppression was used in 62 (66%) SjD-ILD patients and antifibrotics in eight patients (in seven of them in association with immunosuppression). Among the 26 patients with serial PFTs available, 10 (38.5%) showed ILD progression. Progressors had a higher forced vital capacity (FVC 96.8 ± 22.1 vs. 71.1 ± 21.4%; p=0.007) and diffusion capacity for carbon monoxide (DLCO 75.5 ± 12.1 vs. 50.2 ± 16.6%; p=0.002) at baseline and tend to have hypergammaglobulinemia more often (80% vs. 43.8%; p=0.069). Nevertheless, immunosuppression interrupted the decline of FVC (absolute value) and DLCO (percent predicted).

Conclusion: This work demonstrated that a substantial proportion of SjD-ILD patients present with progressive fibrosis. Immunosuppression seems to delay the progression of lung disease. Therefore, identifying predictors for ILD development and progression is essential for recognizing which patients will require closer monitoring and intervention.