Reumatismo最新文献

Objective: This study aimed to describe adult Brazilian and Japanese patients with anti-small ubiquitin-like modifier activating enzyme (SEA)-positive dermatomyositis (DM), as there are few studies in the literature. A literature review was also conducted.

Methods: This bicentric international retrospective study, conducted between 2012 and 2023, included patients with anti-SAE-positive DM (2017 European League Against Rheumatism/ American College of Rheumatology classification criteria). All demographic features and clinical, laboratory, therapeutic, and follow-up data were collected from Brazilian and Japanese centers using pre-standardized and parameterized information.

Results: We included 17 adult patients with a median age of 65 years (56-76 years) and a predominance of females (82.4%). Constitutional symptoms at baseline were present in 58.8% of the patients. In addition to classical cutaneous DM lesions, one-third of the patients had myalgia and significant muscle weakness, whereas half presented with dysphagia, interstitial lung disease, and joint manifestations. The first-line treatment consisted of intravenous methylprednisolone and immunoglobulin pulse therapy in 41.2% and 28.6% of the patients, respectively. The median follow-up duration was 20 (13-74) months; at the last medical evaluation, half had active disease and were still using oral glucocorticoids (median dosage, 10.0 mg/day). Approximately one-fifth to one-third of the patients were diagnosed with different types of cancer, had severe infections, or died.

Conclusions: Patients with anti-SAE-positive DM not only resemble the phenotype of antisynthetase syndrome but are also associated with a poor prognosis.

Objective: To establish the diagnostic value of lung ultrasound (LUS) in patients with rheumatoid arthritis (RA) for the detection of interstitial lung disease (ILD).

Methods: A cross-sectional study was performed. Consecutive patients with RA (American College of Rheumatology/European League Against Rheumatism 2010 criteria) who had a chest high-resolution computed tomography (HRCT) performed within 12 months before inclusion, regardless of symptomatology, were included. Demographic, clinical, laboratory, and pharmacological data were recorded. Each patient underwent a LUS with assessment of B-lines (BL) and pleural irregularities (PI). HRCT was considered the gold standard for the confirmatory diagnosis of ILD. Receiver operating characteristic (ROC) curves were calculated to test the ability of LUS findings (BL and PI) in discriminating patients with ILD.

Results: A total of 104 RA patients were included, of which 21.8% had ILD. Patients with ILD had more BL (median 26 versus 1, p<0.001) and PI (median 16 versus 5, p<0.001) than patients without ILD. The diagnostic accuracy in ROC curves was as follows: area under the curve (AUC) 0.88 and 95% confidence interval (CI) 0.78-0.93 for BL and AUC 0.82 and 95% CI 0.74-0.89 for PI. The best cut-off points for (ILD detection) discriminating the presence of significant interstitial lung abnormalities were 8 BL and 7 PI.

Conclusions: The presence of 8 BL and/or 7 PI in the LUS showed an adequate cut-off value for discriminating the presence of significant interstitial lung abnormalities, evocative of ILD.

Objective: Rheumatic musculoskeletal diseases (RMDs) are the causes of frequent absence from work and loss of productivity. As (in)visible diseases, it is up to the individuals to decide if disclosing their diagnosis, with important repercussions also within the workplace. Still little is known about disease disclosure in the workplace (DD-W) in patients with RMDs. This study aimed to investigate socio-demographic, clinical, and psychological predictors of DD-W among working patients with RMDs.

Methods: A cross-sectional Italian national study captured DD-W in people with RMDs. An online survey was developed using ad-hoc questions and scientific questionnaires to explore demographics and work-related, clinical, and psychological factors. Stepwise logistic regressions were run to identify significant predictors of DD-W.

Results: A total of 250 working rheumatic patients completed the survey; 81.2% of the participants enacted DD-W. DD-W behaviors were predicted by perceived visibility of the RMD (p=0.008), work type (p=0.022), general DD behaviors (p<0.001), and perceived family support (p=0.023). Among RMD patients, psoriatic arthritis participants had higher probabilities of DD-W (p=0.02), whereas lower probabilities were detected in fibromyalgia patients (p=0.003). Lower disease duration corresponded in the sample to higher probabilities of DD-W (p=0.036).

Conclusions: The majority of RMD patients in this study enacted DD-W. DD-W was associated with medical, occupational, and psychological factors, supporting the multidimensionality of the process. Further research on the subject might help foster better DD-W decision-making processes for RMD patients while promoting intervention strategies in education, policy, and culture.

Objective: Diffuse idiopathic skeletal hyperostosis (DISH) is a common disorder characterized by ossification of tendons and ligaments. DISH has been largely associated with an increased risk of metabolic syndrome and type 2 diabetes. The objective of the present study is to investigate the role of DISH in the risk of coronary artery disease (CAD).

Methods: We conducted an observational cross-sectional study of patients without a history of rheumatic musculoskeletal diseases who underwent coronary angiography between March 2016 and April 2021. The SYNergy between percutaneous coronary intervention with TAXus and cardiac surgery (SYNTAX) score was calculated based on coronary angiography images. DISH diagnosis was based on standard X-ray images and computed tomography scans (Resnick criteria). Demographic and clinical characteristics were retrieved from electronic medical records. Multinomial and binary logistic regression models were employed to determine the association between SYNTAX score (dependent variable) and DISH (independent variable).

Results: The study included 187 patients, 82.9% of whom were men, with valid radiological imaging. 83 (44.4%) patients had a confirmed radiological diagnosis of DISH. Diagnosis of DISH was associated with a higher SYNTAX score [adjusted odds ratio (aOR) 34.1, 95% confidence interval (CI) 1.41-79.2 p=0.049], independently from traditional cardiovascular risk factors. In patients aged <70 years, DISH was associated with a 7-fold higher risk of belonging to the highest category of SYNTAX (≥34), compared to non-DISH (aOR 7.23, 95% CI 1.08-48.4; p=0.041). The extension of vertebral calcification was significantly associated with SYNTAX score (r2 0.378, p<0.0001).

Conclusions: DISH diagnosis is common in patients at high risk of cardiovascular disease or with definitive CAD. DISH was independently associated with higher CAD complexity.

Cerebrovascular events (CE) are one of the most common and severe events in antiphospholipid syndrome (APS), a condition characterized by thrombosis and circulating anti-phospholipid antibodies (aPL). Seronegative APS (SN-APS) refers to a group of patients with clinical features of APS but persistently negative tests for "criteria aPL": anti-cardiolipin antibodies (aCL) and anti-β2glycoprotein I antibodies detected by enzyme-linked immunosorbent assay (ELISA), and the lupus anticoagulant detected by clotting assays. We report a series of five cases of SN-APS in young or middle-aged patients who tested positive for "non-criteria" aPL. We retrospectively collected cases of SN-APS patients who experienced CE without an identified cause despite an extensive diagnostic work-up and tested negative for criteria aPL. All the patient sera were tested for aCL by immunostaining on thin-layer chromatography (TLC) and anti-vimentin/cardiolipin (aCL/Vim) by ELISA. We identified five cases of female patients aged 21 to 58 years, evaluated at the Rheumatology Unit and/ or Stroke Unit/Emergency Department of the Sapienza University Hospital of Rome, "Policlinico Umberto I". All patients presented a clinical history suggestive of APS. All the patients tested positive for aCL by TLC-immunostaining, and one patient was positive for aCL/Vim. In young or middle-aged patients with cryptogenic CE and a clinical history suggestive of APS, the use of new diagnostic tools for identifying aPL, if validated in future studies, could represent an important step in the prompt diagnosis of APS.

Takayasu's arteritis (TA) is a granulomatous vasculitis that involves the aortic artery and its branches, resulting in stenosis, occlusion, and aneurysmal dilatation. Cardiovascular involvement is one of the main complications and a major cause of mortality in these patients. Herein, we describe the case of a woman with TA who presented with severe acute heart failure secondary to myocarditis and responded well to immunosuppressive therapy.

We describe the case of a 76-year-old man affected by pneumoconiosis, secondary to silica dust exposure, who was diagnosed with antineutrophil cytoplasmic antibody (ANCA)-positive microscopic polyangiitis (MPA)-related cervical myelitis. Pneumoconiosis is reported to trigger autoantibody production and the onset of different autoimmune diseases, including ANCA-associated vasculitis (AAV). MPA is an AAV of the small vessels that can often affect the nervous system, although involvement of the spinal cord in the form of myelitis is described as an anecdotal occurrence. Our experience suggests that an autoimmunity workup should be considered for patients with pneumoconiosis who present with neurological symptoms consistent with AAV.

This case series aims to characterize the development of systemic lupus erythematosus (SLE) induced by anti-tumor necrosis factor α (anti-TNFα) therapy in patients with inflammatory rheumatic diseases, namely rheumatoid arthritis (RA), spondylarthritis (SpA), and psoriatic arthritis (PsA). Patients with a diagnosis of SLE induced by anti-TNFα therapy and registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) who started their first anti-TNFα between 2001 and 2020 were included. Demographic, clinical, and laboratory data were obtained by consulting Reuma.pt. The diagnosis of SLE induced by anti-TNFα was considered if there was a temporal relationship between the onset of anti-TNFα therapy and manifestations (clinical and immunological) in accordance with the American College of Rheumatology/European League Against Rheumatism criteria (2019). A total of 607 patients with inflammatory rheumatic diseases and six cases of SLE induced by anti-TNF-α therapy were reviewed: two patients were affected by RA, three patients by SpA, and one by PsA. All these patients had articular and constitutional symptoms that improved after discontinuation of the anti-TNFα agent. After switching to a second anti-TNFα agent, there was no recurrence of SLE over time. The development of SLE secondary to anti-TNFα agents in inflammatory rheumatic patients is rare. In this case series, all patients had a mild disease that improved after therapy discontinuation without recurrence of the disease. SLE induced by anti-TNFα should be considered in the follow-up of RA, SpA, and PsA patients.

Visceral muscle dysmotility syndrome (VMDS) is a rare syndrome described in the systemic lupus erythematosus (SLE) clinical course. It is characterized by diffuse thickened intestinal wall and gastrointestinal-genitourinary-hepatobiliary hollow viscera dilatation and dysmotility. Due to the rarity and the heterogeneity of the clinical characteristics of this syndrome, it is not entirely clear which is the best diagnostic imaging technique for the diagnosis and/or follow-up, even if, in all the described cases, computed tomography (CT) was generally used to study visceral involvement. However, there are no cases describing the visceral metabolic activity by 18 fluorodeoxyglucose-positron emission tomography-CT (18FDG-PET-CT). Here, we reported the first clinical case of VMDS studied by 18FDG-PET-CT, characterizing the metabolic activity of this rare syndrome during SLE flare. We found a high intestinal metabolic burden, hyper-fixation in duodenum, and high hepatic metabolic activity. Moreover, we reviewed the literature on VMDS in SLE, focusing on imaging techniques in different anatomical sites (bowel, urinary tract, bile ducts), patients' symptoms, and treatment.

Objective: This study aimed to analyze the status of liver [aspartate aminotransferase (AST) and alanine aminotransferase (ALT)] and kidney (serum creatinine) function in ankylosing spondylitis (AS) patients assuming continuously non-steroidal anti-inflammatory drugs (NSAIDs) alone over a long period.

Methods: Between 2013 and 2022, there were records of 385 AS patients. Of them, 56 were receiving only NSAIDs, and the files of these patients were retrospectively analyzed. Demographic and clinical characteristics were collected. Blood tests, including serum creatinine, AST, and ALT, were assessed at each visit.

Results: Of the 56 patients, 39 were male. The mean age was 45.30 years, and the follow-up period was 9.80 years. Of them, 44.6% used indomethacin, 26.8% naproxen, 17.9% diclofenac, 5.4% acemetacin, 3.6% meloxicam, and 1.8% celecoxib. The mean baseline serum creatinine was 0.71 mg/dL. The mean baseline serum AST and ALT were 19.6 u/L and 22.9 u/L, respectively. Baseline creatinine, AST, and ALT were not statistically significantly different between sexes. There was a statistically significant difference between mean creatinine concentrations at baseline and at year 3. One patient on naproxen discontinued treatment due to elevated creatinine. The creatinine level decreased during the patient's follow-up. Liver enzymes above 3 times the normal value were not seen in any patient.

Conclusions: Based on real-world data, long-term use of NSAIDs has generally not led to acute liver and kidney injury or progressive impairment of hepatorenal function requiring discontinuation of treatment.