Cristiana Sieiro Santos, Marcelo Neto, Manuel Silvério-António, Maria Seabra Rato, Catarina Resende, Raquel Miriam Ferreira, Tânia Santiago
{"title":"Clinical spectrum and outcomes of pericardial and myocardial disease in systemic sclerosis: a multicentre case series and literature review.","authors":"Cristiana Sieiro Santos, Marcelo Neto, Manuel Silvério-António, Maria Seabra Rato, Catarina Resende, Raquel Miriam Ferreira, Tânia Santiago","doi":"10.63032/CSQZ3453","DOIUrl":"10.63032/CSQZ3453","url":null,"abstract":"<p><strong>Objectives: </strong>Cardiac involvement is a leading cause of morbidity and mortality in systemic sclerosis (SSc) yet is often underdiagnosed. Early recognition is crucial but published data remains limited. This study aimed to characterize the clinical presentation, diagnostic features, and outcomes of SSc patients with acute primary heart involvement.</p><p><strong>Methods: </strong>This multicentre retrospective case series included patients meeting the 2013 ACR/EULAR SSc classification criteria with clinically significant pericardial effusion or myocardial involvement, identified across tertiary rheumatology centres in Portugal (Coimbra, Lisbon, and Porto) and Spain (León). A complementary literature review was performed to identify additional published cases.</p><p><strong>Results: </strong>Of 23 screened cases, 6 met the inclusion criteria. The literature review identified 13 additional reports, totalling 19 patients. In 8 (42.1%) of patients, heart involvement was either the initial manifestation or occurred concurrently with the diagnosis of SSc. Pericardial disease (n=6) often presented as tamponade requiring emergent pericardiocentesis. Myocardial involvement (n=13) was classified as acute myocarditis (AM) in 9 (69.2%) and chronic inflammatory cardiomyopathy (CIC) in 4 (30.8%). Cardiac magnetic resonance showed late gadolinium enhancement in 9 (72.7%) and myocardial oedema in 4 (36.4%); however, both were absent in two biopsy-confirmed AM cases. Cyclophosphamide was the second most common therapy after corticosteroids. Two patients died during hospitalization, with three more deaths during long-term follow-up.</p><p><strong>Conclusion: </strong>Severe cardiac manifestations in SSc frequently occur early in the disease, presenting as tamponade or myocardial involvement. Reliance solely on imaging may lead to myocarditis underdiagnosis. Distinct clinical profiles between AM and CIC point toward separate phenotypes with unique prognostic and therapeutic considerations. Large-scale, prospective studies are essential to refine early diagnostic approaches and optimize treatment.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"5 1","pages":"15-26"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147663257","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Nailfold capillaroscopy in idiopathic inflammatory myopathies.","authors":"Cláudia Pinto Oliveira, Raquel Campanilho Marques, Eduardo Dourado","doi":"10.63032/JRLV5483","DOIUrl":"10.63032/JRLV5483","url":null,"abstract":"<p><p>Nailfold videocapillaroscopy (NVC) is a non-invasive technique that enables the evaluation of peripheral microcirculation by visualising nailfold capillaries. Although traditionally used in systemic sclerosis, NVC may also be useful in other systemic autoimmune rheumatic diseases, and particularly idiopathic inflammatory myopathies (IIMs). This review aims to summarise the current evidence on the prevalence of NVC findings in IIMs and their correlation with clinical and serological characteristics, as well as diagnostic, follow-up, and prognostic implications.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"5 1","pages":"2-14"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147663223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carla Campinho Ferreira, Inês Borges, Mariana Pinheiro, Hugo Gonçalves, Paulo Pereira, Emanuel Costa
{"title":"Factors associated with methotrexate-related gastrointestinal intolerance and toxicity in rheumatoid arthritis and psoriatic arthritis.","authors":"Carla Campinho Ferreira, Inês Borges, Mariana Pinheiro, Hugo Gonçalves, Paulo Pereira, Emanuel Costa","doi":"10.63032/LNUD9165","DOIUrl":"10.63032/LNUD9165","url":null,"abstract":"<p><strong>Background: </strong>Methotrexate (MTX) is the cornerstone therapy for rheumatoid arthritis (RA) and psoriatic arthritis (PsA), yet gastrointestinal adverse events (GIAE), including intolerance and hepatotoxicity, remain major causes of treatment modification and discontinuation. Identifying baseline predictors of these reactions is essential to optimizing treatment safety and persistence.</p><p><strong>Objectives: </strong>To identify clinical and laboratory predictors of MTX-related GIAE and to compare risk profiles between RA and PsA.</p><p><strong>Methods: </strong>Retrospective observation study including MTX-treated patients with RA or PsA. Baseline demographics, comorbidities, laboratory results, MTX characteristics, and concomitant medications were extracted from medical records. GIAE comprised either gastrointestinal (GI) intolerance or toxicity. Associations were assessed through univariate tests followed by multivariable logistic regression. Kaplan-Meier curves evaluated treatment survival according to administration route and disease type.</p><p><strong>Results: </strong>Among 369 patients (62.6% female; mean age 57.5 +/- 12.6 years), 50.9% developed GIAE. GI intolerance occurred in 127 patients, mainly presenting as nausea (68.5%). GI toxicity occurred in 75 patients, with baseline alanine transaminase (ALT) significantly higher in affected patients. Independent predictors of GIAE were diabetes mellitus (aOR 2.22), female sex (aOR 1.82) and PsA (aOR 1.67). Predictors of GI intolerance included higher baseline ALT (aOR 1.02), concomitant leflunomide (aOR 1.91), and female sex (aOR 2.08). Predictors of GI toxicity included diabetes (aOR 2.98), alcohol consumption (aOR 2.79), and baseline ALT (aOR 1.03). Survival analysis showed earlier MTX-related GIAE in patients receiving the subcutaneous formulation across diseases (p<.001).</p><p><strong>Conclusions: </strong>MTX-related GIAE are frequently and largely driven by metabolic comorbidities, lifestyle exposures, sex and baseline ALT. These routinely available parameters allow early identification of high-risk patients and may guide personalized MTX initiation and monitoring strategies.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"5 1","pages":"47-56"},"PeriodicalIF":1.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147663287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hugo Gonçalves, Duarte Augusto, Paulo Pereira, Carla Ferreira-Campinho, Ana Margarida Correia, Catarina Ferreira, Emanuel Costa, Joana Leite Silva, Ana Roxo Ribeiro, Diogo Esperança Almeida
{"title":"Tocilizumab in immune checkpoint inhibitor-induced myositis, myocarditis, and myasthenic syndrome: a rare case report and review of the literature.","authors":"Hugo Gonçalves, Duarte Augusto, Paulo Pereira, Carla Ferreira-Campinho, Ana Margarida Correia, Catarina Ferreira, Emanuel Costa, Joana Leite Silva, Ana Roxo Ribeiro, Diogo Esperança Almeida","doi":"10.63032/RFPY6607","DOIUrl":"10.63032/RFPY6607","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"300-304"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890341","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
José S Cortés, Javier Correa, Wilder Carvajal, Wilmer Aponte, Luis Javier Cajas
{"title":"Methotrexate-associated pneumonitis: usefulness of lung ultrasound.","authors":"José S Cortés, Javier Correa, Wilder Carvajal, Wilmer Aponte, Luis Javier Cajas","doi":"10.63032/PQTT9927","DOIUrl":"10.63032/PQTT9927","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Case report: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"320-321"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890364","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reuma.pt - a dynamic registry supporting clinical practice and research in rheumatology.","authors":"Elsa Vieira-Sousa","doi":"10.63032/JIVF6788","DOIUrl":"10.63032/JIVF6788","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"264-266"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890348","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Subclinical right ventricular dysfunction in juvenile systemic sclerosis: a cross-sectional echocardiographic study.","authors":"Ozlem Sarisoy, Seyma Turkmen, Betul Sozeri","doi":"10.63032/NLOB9461","DOIUrl":"10.63032/NLOB9461","url":null,"abstract":"<p><strong>Objective: </strong>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).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"294-299"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Comment on \"Real-world efficacy and retention of guselkumab in psoriatic arthritis: a 12-month multicenter study.","authors":"Umut Bakay","doi":"10.63032/JKUR6010","DOIUrl":"10.63032/JKUR6010","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"315-316"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890303","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mariana Emília Santos, Catarina Gama, Bernardo Marques, Tiago Saldanha, Joana Tremoceiro, Maria Helena Lourenço, Maria João Gonçalves, Manuela Costa, Alexandre Sepriano
{"title":"Adrenal failure as an antiphospholipid syndrome manifestation: case-based review.","authors":"Mariana Emília Santos, Catarina Gama, Bernardo Marques, Tiago Saldanha, Joana Tremoceiro, Maria Helena Lourenço, Maria João Gonçalves, Manuela Costa, Alexandre Sepriano","doi":"10.63032/SLBJ1623","DOIUrl":"10.63032/SLBJ1623","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"305-310"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}