Alfonse T. Masi (born October 1930) passed away peacefully and comfortably in his home in March 2025 at the age of 94 years after a prestigious career...
Alfonse T. Masi (born October 1930) passed away peacefully and comfortably in his home in March 2025 at the age of 94 years after a prestigious career...
Objective: To date, there is no shared national guideline in Italy for the management of reproductive health in rheumatic diseases (RHRD). The Italian Society for Rheumatology (SIR) has committed to developing clinical practice recommendations to provide guidance on both management and treatment regarding RHRD in Italy.
Methods: Using the GRADE-ADOLOPMENT methodology, a systematic literature review was conducted to update the scientific evidence that emerged after the publication of the reference recommendations from the American College of Rheumatology. A multidisciplinary group of 18 clinicians with specialist experience in rheumatology, allergy and clinical immunology, internal medicine, nephrology, gynecology and obstetrics, and neonatology, a professional nurse, a clinical psychologist, and a representative from the National Association of Rheumatic Patients discussed the recommendations in collaboration with the evidence review working group. Subsequently, a group of stakeholders was consulted to examine and externally evaluate the developed recommendations.
Results: Recommendations were formulated for each area of interest: contraception, assisted reproductive technology, preconception counseling, and use of drugs before, during, and after pregnancy and during breastfeeding, considering both paternal and maternal exposure.
Conclusions: The new SIR recommendations provide the rheumatology community with a practical guide based on updated scientific evidence for the management of RHRD.
Objective: Intra-articular injections of hyaluronic acid (HA) have been reported to alleviate pain, reduce disability, and improve joint function in glenohumeral osteoarthritis (GH-OA). This retrospective study aimed to evaluate the effectiveness of a HA-based formulation (Hyalubrix®) in reducing the pain of patients with GH-OA and improving both patient's shoulder functions and quality of life (QoL).
Methods: Data collected during the standard clinical practice of the center was retrospectively analyzed. The Simple Shoulder Test (SST) questionnaire reported data on the patient's ability to perform daily activities; the Euro-Quality of Life Health Assessment (EQ-5D) collected evidence on QoL; and changes in pain were evaluated through the Visual Analog Scale (VAS). SST and EQ-5D scores were analyzed comparing baseline values with those at the last follow-up, while VAS was investigated for all the available visits. Continuous values were summarized as mean ± standard deviation, median, and 25-75th percentiles. The Shapiro-Wilk test assessed normality, with significance set at p<0.05, and no adjustments for multiple comparisons were made.
Results: All scores showed a significant improvement: VAS decreased from 55.4±13.8 to 16.2±16.3 (p<0.001), the SST increased from 38.0 to 65.5 (p<0.001), as did the EQ-5D (from 41.7 to 76.7; p<0.001).
Conclusions: GH-OA treatment with Hyalubrix® proved to be highly beneficial, leading to complete pain reduction in more than 50% of patients and a significant reduction in 27.5% of cases. This resulted in improved joint function and QoL.
Objective: Juvenile dermatomyositis (JDM) is a rare chronic systemic inflammatory disorder with a highly variable clinical course. It is important to identify the patients at risk of developing more severe disease. However, based on the existing literature, there is a lack of data regarding predictors of poor outcomes. Obtaining knowledge about clinical and laboratory risk factors for disease progression and severity at an earlier stage of the disease could potentially lead to a better long-term prognosis for patients with JDM.
Methods: A narrative review to identify risk factors for poor outcomes in patients with JDM, such as death, severe disease, refractory disease, and functional impairment, was conducted. A total of 27 articles were included.
Results: Certain clinical manifestations and immunology features appear to worsen the prognosis in children with JDM. The recognition of these risk factors is essential for all pediatric rheumatologists as it allows the earlier identification of patients with potentially worse outcomes. These patients should receive closer follow-up and aggressive and individualized therapy to reduce their morbimortality.
Conclusions: Additional research is needed not only to identify more predictors of worse outcomes but also more effective treatment approaches targeted toward these patients.
Objective: Patients Acceptable Symptom State (PASS) is a single dichotomized question assessing health satisfaction. We aimed to investigate PASS achievement within 4 weeks of treatment with Janus kinase (JAK) inhibitors (Jakinibs) and its association with treatment response after 4 and 12 weeks in rheumatoid arthritis (RA) patients.
Methods: We recruited consecutive RA patients starting baricitinib or tofacitinib. At baseline, 4 and 12 weeks, we calculated disease activity [Disease Activity Score on 28 joints (DAS28), Clinical Disease Activity Index, Simplified Disease Activity Index], disease status [remission and low-disease activity (LDA)], percentage of patients achieving PASS, and the time to attain PASS. We assessed the impact of clinically relevant variables on PASS achievement by logistic regression analysis.
Results: We enrolled 113 patients [98 (86.7%) females; median age 59.6 (interquartile range 16.9), median disease duration 144 (132) months]. 90 (79.6%) patients achieved PASS after 10 (8) days. A similar percentage of PASS achievers and non-achievers was in remission/LDA at weeks 4 and 12, but the reduction of disease activity was significantly greater in PASS achievers. All patients achieving Boolean remission at weeks 4 and 12 had achieved PASS within 4 weeks. The impact of Patients Global Assessment (PGA) on DAS28 was significantly greater in PASS non-achievers compared to PASS achievers; inversely, the impact of C-reactive protein was more relevant in PASS achievers. At multivariate analysis, pain and PGA were significantly associated with PASS.
Conclusions: In our cohort, Jakinibs allowed an early achievement of PASS in a great percentage of RA patients. PASS is strictly dependent on PGA and pain and could suggest, early in the management of RA patients, therapeutic success.
Objective: This study aimed to investigate the correlated risk factors and presence of radiological enthesopathies of the Achilles tendon and plantar fascia in patients with axial spondyloarthropathy (axSpA).
Methods: 242 patients (121 female and 121 male) with axSpA were included in this study. The Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), the Bath Ankylosing Spondylitis Functional Index (BASFI), the Ankylosing Spondylitis Disease Activity Score with C-reactive protein (ASDAS-CRP), the Bath Ankylosing Spondylitis Radiology Index (BASRI), the Maastricht Ankylosing Spondylitis Enthesitis Score (MASES), and CRP were evaluated in all patients. The lateral foot X-rays of the patients were assessed for enthesopathies of the Achilles tendon and plantar fascia attachments.
Results: Calcaneal spur and Achilles enthesopathies were present in 57.4% of the patients. 39.3% of patients had enthesopathies in both regions. The male and female groups differed statistically in terms of weight, height, body mass index (BMI), positive family history, and duration since diagnosis (p<0.05). The presence of calcaneal spur and Achilles enthesopathies was found to be significantly correlated with age, weight, BMI, symptom duration, and the scores of BASDAI, BASFI, ASDAS-CRP, BASRI, and MASES (p<0.05).
Conclusions: The presence of enthesopathies appears to be associated with age, weight, BMI, symptom duration, and disease activity. Conventional radiography can be used as an auxiliary tool in the evaluation of entheseal abnormalities in patients with SpA, especially in patients with advanced age, long symptom duration, and high BMI.
Alopecia universalis (AU), an advanced form of alopecia areata (AA), is a condition characterized by the complete loss of hair over the entire skin surface. Recent progress has significantly enhanced our understanding of the pathogenesis of AU. In particular, interferon-γ (IFN-γ) and interleukin (IL)-15 seem to play a pivotal role in the pathogenesis of the disease. Nonetheless, a variety of medications has been used to treat the disease with frequently inconsistent results. Given the broad modulation of the immune system and inhibition of key molecules, including IFN-γ and IL-15, oral janus kinase (JAK) inhibitors represent a treatment option for moderate to severe cases of AA, as demonstrated in case reports supporting their efficacy and tolerability. We present the case of a patient suffering from psoriatic arthritis and AU who experienced a sudden improvement in peripheral arthritis and AU while receiving JAK1 selective treatment with upadacitinib. So far, there are very limited case reports of successful upadacitinib treatment for patients with AA, mostly in patients also suffering from atopic dermatitis. Thus, we provide evidence for the efficacy of upadacitinib in managing AU in adults, as well as in the context of inflammatory arthritis such as psoriatic arthritis.
Objective: To investigate the association between the volume of exercise and the quality of sleep in patients with fibromyalgia.
Methods: This is a cross-sectional study carried out from 2010 to 2019 in patients over 18 years old from the research project at a university in Brazil. Instruments related to sociodemographic and clinical characteristics, physical exercise, and the Pittsburgh Sleep Quality Index (PSQI) were applied. Participants were classified as inactive, insufficiently active, or active. In the statistical analysis, the Kruskal-Wallis and Mann-Whitney U tests were used. Binary logistic and multinomial regression were also performed.
Results: The majority of participants were physically inactive and had poor sleep quality; 68.3% with poor sleep quality were inactive. In the analysis of the difference between the three groups, sleep latency (time it takes to fall asleep) (p=0.00) and total PSQI (p=0.04) were significantly different. When the analysis was performed between active and inactive individuals, significant differences were found in sleep latency (p=0.02), daytime dysfunction (difficulties in performing daytime tasks due to poor sleep quality) (p=0.02), and the total PSQI (p=0.02). Binary logistic regression with crude analysis showed that inactive participants are 4.3 times more likely to have poor sleep quality when compared to active participants (odds ratio = 4.311; 95% confidence interval 1.338-13.888; p=0.014). Multinomial regression analysis showed that being physically active can be a protective factor.
Conclusions: There is a high prevalence of sleep disorders and insufficient practice of physical exercise among patients with fibromyalgia. It is suggested that regular physical exercise may be related to sleep quality, and more active participants have fewer sleep disorders, with exercise being a protective factor.
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.

