OBJECTIVES:To describe the prevalence of gastrointestinal (GI) symptoms in SSc and Very Early Diagnosis of SSc (VEDOSS), identify clinical and serological features associated with GI involvement and explore a cranio-caudal pattern of symptom distribution, using data from the Italian SPRING-SIR registry. METHODS:This cross-sectional analysis included patients fulfilling 2013 ACR/EULAR SSc or VEDOSS criteria. GI involvement was defined as symptoms in at least one GI tract segment and categorized as upper and lower. Associations between GI involvement and clinical variables were assessed using logistic and ordinal regression models, adjusting for demographics, disease characteristics and autoantibodies. RESULTS:Among 1917 SSc patients, 56% had GI symptoms, associated with longer disease duration, dcSSc, interstitial lung disease (ILD), digital ulcers (DU), telangiectasias and tobacco exposure. Extensive GI involvement correlated with more severe disease. Ordinal regression identified female sex, dcSSc, ILD, DU, telangiectasias, tobacco exposure and anti-centromere antibodies as variables significantly associated with more extensive GI involvement. Disease duration did not show a significant association with GI symptom extent. Among 211 VEDOSS patients, 41.2% reported GI symptoms (mostly oesophageal), significantly associated with puffy fingers and dyspnoea. Among VEDOSS, puffy fingers and anti-centromere antibodies were independent predictors of presence of oesophageal symptoms. CONCLUSION:GI involvement in SSc is linked to more severe disease and longer disease duration. Disease duration resulted linked to the presence of GI symptoms rather than extent of GI involvement. Puffy fingers and anti-centromere antibodies may associate with presence of early oesophageal symptoms in VEDOSS.
OBJECTIVES:To assess the relationship between disease duration and the prevalence/distribution of nailfold videocapillaroscopy (NVC) patterns, named according to the current classification as 'early', 'active' and 'late', in a large cohort of systemic sclerosis (SSc) patients. METHODS:A cross-sectional analysis was conducted on 1689 patients undergoing standardized NVC. Clinical-serological data and treatments were collected. Statistical comparisons and multivariable logistic regression models were applied, including analyses based on disease duration. RESULTS:The prevalence of NVC patterns was as follows: 'early' 21.6%, 'active' 47.4%, 'late' 25.7% and normal/non-specific 5.3%. The distribution by disease duration showed that the three main patterns were always present. While the 'early' and 'active' progressively decreased (from 30.3% and 51.9% in patients with ≤5 yrs, to 14.6% and 43.5% in those >10 yrs, P < 0.01), the 'late' pattern increased from 13.2% (≤5 yrs) to 36.0% (>10 yrs) (P < 0.001) and was associated with internal organ involvement, anti-topoisomerase antibodies and more therapies (P < 0.01). Conversely, the 'early' and 'active' patterns were associated with the limited-cutaneous subset (P < 0.01) and anti-centromere antibodies (P < 0.001). Multivariable analysis confirmed a strong association between the 'late' pattern and skin/peripheral vascular involvement. Notably, the presence of the 'late' pattern in patients with ≤2 yrs (10.9%) was significantly associated with scleroderma renal crisis (P = 0.012). CONCLUSION:SSc-NVC patterns are not strictly time-dependent and can be observed at any stage of the disease, suggesting that microvascular damage progression is heterogeneous across different disease periods. Therefore, a revised classification of NVC changes considering both disease duration and NVC severity could improve its prognostic accuracy.
OBJECTIVE:Mycophenolate mofetil (MMF) use in limited cutaneous systemic sclerosis (lcSSc) is relatively uncommon because of the lower fibrotic burden and the predominance of vascular complications. In vitro observations and clinical data from transplanted patients suggest a protective effect of MMF on endothelial function. Our aim was to evaluate the reasons for prescribing MMF treatment in patients with lcSSc and its impact on the need for escalation of vascular complication-related treatments during follow-up. METHODS:Patients with lcSSc enrolled in the Italian Systemic Sclerosis Progression Investigation registry were retrospectively evaluated. All patients treated with MMF were matched to patients not treated with MMF, which was based on a roll-entry time-dependent propensity score built on demographics, clinical features, and baseline treatment. The escalation of vasoactive or vasodilator treatment up to 60 months was defined as the introduction of iloprost, endothelin receptor antagonists, or phosphodiesterase-5 inhibitors on top of the ongoing treatment, because of uncontrolled or newly diagnosed vascular complications. A hazards Cox model was also adopted to quantify the association of MMF treatment with treatment escalation. RESULTS:A total of 1,435 patients with lcSSc were evaluated, of whom 152 were prescribed MMF (17.1% male; mean age at lcSSc onset 48.7 ± 13.9 years, 54.6% anti-Scl70 positive). The prescription of MMF was more common in men and in anti-Scl70 positive, anticentromere negative patients with interstitial lung disease, myositis, and without a history of digital ulcers. After matching 107 patients with MMF-untreated controls, the overall incidence of vasoactive/vasodilator treatment escalation events related to digital ulcers over a median follow-up of 40.5 months (interquartile range 23.3-60.0) was 0.3 per 100 patient-years in the MMF-treated group and 5.4 per 100 patient-years in the matched control group, with a significant difference in treatment escalation-free survival between the two groups (hazard ratio 0.05, 95% confidence interval 0.01-0.38; P value = 0.004). CONCLUSION:In patients with lcSSc, the introduction of MMF has reduced the need for escalation of vasoactive or vasodilator treatment, suggesting that it may also help to prevent vascular complications, which frequently affect patients with lcSSc.
Monoarthritis is a familiar—yet systematically under-theorised—scenario in rheumatoid arthritis and psoriatic arthritis, occurring both at onset and as residual synovitis that persists despite apparently successful treat-to-target care. Contemporary composite indexes, trial eligibility criteria, and guideline algorithms were built for polyarticular disease; as a result, these tools can numerically dilute the functional and prognostic weight of a single inflamed knee, ankle, or dominant wrist. This situation results in an uncomfortable paradox: overall disease activity can meet the predefined therapeutic target (eg, remission or low disease activity), while the patient remains substantially symptomatic or functionally limited because of persistent inflammation in a single joint. When the burden is focal, the usual reflex of escalating systemic immunosuppression deserves scrutiny, because the hazards of escalation do not shrink with joint count, whereas the incremental benefit often does. In this Viewpoint, we argue that monoarthritis is not merely a measurement nuisance but a decision problem. We examine why monoarthritis remains poorly captured by current measurement systems, trial paradigms, and treatment frameworks. We also highlight key research priorities to make monoarthritis visible again—both epidemiologically and methodologically—including index-joint phenotyping, joint-centred outcomes, and comparative evaluation of local versus systemic treatment strategies.
Background:The sequence and temporal relationship between Raynaud's phenomenon (RP) and the first non-Raynaud's sign/symptom (NRP) in systemic sclerosis (SSc) have been partially investigated. Objectives:To evaluate whether the mode and ages of clinical onset are associated with disease endotype and survival in SSc. Design:We included SSc patients from the Systemic sclerosis Progression INvestiGation registry of the Italian Society of Rheumatology (SPRING-SIR) registry in a cohort study, with post hoc cross-sectional and longitudinal analysis. Methods:Patients were grouped based on age-RP and age-NRP quartiles. Additionally, categories were defined based on mode of onset: RP group-RP onset at least 1 year before NRP; Simultaneous group-RP onset within the same year of NRP; NRP group-RP onset after at least 1 year after NRP. Comparisons were made using Chi-square and ANOVA tests. Logistic, linear, and multinomial regression models were applied to assess associations, while Kaplan-Meier curves and Cox regression were used to assess mortality. Results:A total of 1748 patients were eligible: 682 (39.0%) in the RP group, 1026 (58.8%) in the simultaneous group, and 39 (2.2%) in the NRP group. A higher prevalence of anti-centromere antibodies was found In the RP group, while the simultaneous group had more diffuse cutaneous SSc (dcSSc), anti-topoisomerase-I antibodies, and higher Rodnan's skin score (mRSS). The NRP group presented higher prevalence of pulmonary arterial hypertension. On logistic regression, the simultaneous group was associated with a higher prevalence of dcSSc compared to the RP group (odds ratio, 1.491, 95% confidence interval (CI): 1.032-2.154). Younger age at RP onset was associated with lower systolic pulmonary artery pressure and mRSS. In 943 patients with available follow-up (median 24 months), the simultaneous group had higher mortality compared to the RP group (hazard ratio, 1.975, 95% CI: 1.002-3.893). Conclusion:The timing of RP and NRP onset may help define SSc endotype and survival. Patients with simultaneous RP-NRP onset have more severe disease features and higher mortality risk, emphasizing the relevance of onset timing in disease stratification.
Background:Microvascular alterations can be detected with nailfold videocapillaroscopy, useful for systemic sclerosis (SSc) diagnosis upon identification of the "scleroderma pattern" (NVC-SP). However, this pattern is missing in a subset of SSc patients. Methods:This retrospective analysis on the multicenter Italian SPRING cohort of SSc patients assessed the prevalence and characteristics of patients without NVC-SP. Associations with demographic and clinical features were evaluated using logistic regression (cross-sectional) and mixed-effects models (longitudinal). Results:Out of 1689 SSc patients with available NVC information, 90 (5.3%) did not have the NVC-SP. These patients were older at SSc onset (53 vs. 49 years) and more frequently sine scleroderma (31% vs 12%, p < 0.001). The overall burden of vascular complications was milder in this subset, with a lower prevalence of pitting scars (33% vs 48%), telangiectasias (52% vs 74%), and calcinosis (3.4% vs 12%). During the follow-up, mRSS remained lower (60-month mean 4 vs 7, p = 0.002), as well as the estimated probability of vascular complications. Moreover, despite comparable values at baseline, DLCO remained significantly higher in patients without NVC-SP at the following time points (12, 24, and 60 months, p < 0.05). The presence of "late" NVC-SP was independently associated with ILD (OR 1.83, 95% CI 1.05-3.18). Conclusions:The absence of NVC-SP in 5.3% of SPRING SSc patients may identify a subset characterized by milder disease, with a lower burden of vascular and organ complications. Conversely, lung involvement was more frequently observed in patients with more severe NVC microangiopathy.
OBJECTIVES:The aim of this prospective multicentre observational study was to evaluate the clinical course and the incidence of interstitial lung disease (ILD) on a population affected with rheumatoid arthritis (RA). METHODS:Three hundred and twenty-five consecutive RA patients with an ongoing treatment with abatacept were enrolled in the study and followed for 24 months. At the enrolment, ILD was recorded in 20.6% of cases. Patients with ILD were followed according to the current clinical practice, while in patients without ILD, a careful assessment for respiratory symptoms and velcro crackles, also by mean of digital tools, was performed every six months. In this regard, each patient was evaluated by mean of VECTOR, a software able to identify velcro crackles with a diagnostic accuracy of 83.9% and a sensitivity of 93.2% in RA patients. RESULTS:During the study, no patients discontinued abatacept for a worsening of lung function, and no difference was recorded in two-year retention rate of abatacept between patients with and without ILD. After 24 months, ILD improved or remained stable in more than 85% of cases, while a worsening of radiologic picture was recorded in 14.9% of cases, mainly with usual interstitial pneumonia. Finally, the incidence rate of new-onset ILD was 0.39/100 patients/year. CONCLUSIONS:In conclusion, the presence of ILD did not affect the retention rate of the drug, suggesting a role for abatacept in the treatment of RA-ILD.
Background Antitopoisomerase I (ATA), anticentromere (ACA) and anti-RNA polymerase III (RNAP3) antibodies are included in the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for systemic sclerosis (SSc). A subset of patients with SSc satisfy criteria but may lack these specific autoantibodies, being classified as ‘triple-negative’.Methods We conducted a retrospective evaluation of triple-negative patients with SSc prevalence and clinical features among the multicentric Systemic sclerosis Progression INvestiGation registry.Results Out of 1480 patients with SSc, 295 (19.9%) were triple-negative, while 1185 (81.1%) had SSc-specific antibodies: ACA (54.3%), ATA (43.6%) and RNAP3 (2.1%). The triple-negative group showed a higher prevalence of myopathy (16.7% vs 10.1%, p=0.003), suggested by higher creatine phosphokinase (CPK) levels (126.2 vs 92.5 U/mL, p=0.002), more frequent CPK increase over 2–3 times (2.4% vs 0.2%, p=0.028). Triple-negative patients also exhibited fewer vascular complications, including digital ulcers (17.3% vs 22.8%, p=0.04) and calcinosis (8.2% vs 12.8%, p=0.027), and a higher prevalence of interstitial lung disease (p<0.001). Consistently, lower diffusing capacity for carbon monoxide (66.4% vs 70.98%, p=0.004) and forced vital capacity (97.01% vs 102.92%, p<0.001) were found in the triple-negative group. Triple-negative patients more frequently received corticosteroids (79.3% vs 67.9%, p=0.003), cyclophosphamide (43.4% vs 26%, p<0.001) and azathioprine (38.5% vs 22.3%, p=0.002), while less frequently received prostanoids (71.6% vs 85.9%, p<0.001), calcium channel blockers (80.1% vs 87.7%, p=0.005) and phosphodiesterase-5 inhibitors (4% vs 20%, p<0.001).Conclusions A higher prevalence of myopathy and interstitial lung disease and a reduced vascular burden were found in the triple-negative patients, suggesting that the non-specific and non-routinely tested autoantibodies may identify an SSc endotype resembling sclero-myositis.
INTRODUCTION:Primary heart involvement (pHI) is an overlooked and poorly characterised complication of systemic sclerosis (SSc), associated with the risk of heart failure, arrhythmia and death. Despite consensus definition by the World Scleroderma Foundation/Heart Failure Association (WSF/HFA), diagnostic criteria and risk factors remain poorly elucidated. METHODS:Out of 1922 patients in the Italian national SPRING registry, we excluded those with potentially confounding conditions according to WSF/HFA, and those with incomplete ECG or echocardiographic assessment, resulting in 600 subjects with clearly defined parameters to intercept SSc-pHI. Cross-sectional and longitudinal analyses were performed to identify factors associated with pHI. RESULTS:ECG and/or echocardiographic signs of SSc-pHI were identified in 25% of patients at enrollment and were associated with older age (OR 1.04; 95% CI 1.02-1.06), diffuse cutaneous SSc (OR 1.85; 95% CI 1.05-3.26) and intestinal symptoms (OR 1.79; 95% CI 1.03-3.08). Diastolic dysfunction (62%) and conduction disturbances (34%) were the most frequent phenotypes, while diffuse hypokinesia with reduced ejection fraction was the least common (3%). During follow-up, new-onset signs of pHI were observed in an additional 25% of patients, particularly in those with skeletal muscle involvement (HR 2.83; 95% CI 1.01-7.73). CONCLUSIONS:pHI is a severe complication potentially affecting one-quarter of patients with SSc. Early detection is crucial, particularly in those with diffuse skin fibrosis, muscular involvement and intestinal manifestations.
INTRODUCTION:Behçet syndrome (BS) is a multifactorial disorder with several clinical manifestations that occurs during the childbearing age. The relationship between pregnancy and BS has been previously analysed as the pregnancy immunometabolic reprogramming in case of autoinflammatory and/or autoimmune diseases can influence both the course of the disease and the pregnancy outcomes. We aim to analyse the relationship between BS and pregnancy, investigating: (1) the prevalence and clinical characteristics of disease flares during pregnancy and (2) the pregnancy outcomes in a large cohort of Italian patients with BS. METHODS:This retrospective study was conducted recruiting a cohort of BS patients according to the criteria of the International Study Group for Behçet (ISG), following at the Rheumatology Department of Lucania (San Carlo Hospital, Potenza) from January 2000 to December 2021. We reviewed medical records and collected demographic and clinical data besides pregnancy-related data, in particular: maternal age, disease flares during and after pregnancy, post-partum maternal and neonatal complications (up to 6 months after pregnancy). RESULTS:We studied 153 pregnancies in 96/117 subjects (21/117 patients did not get pregnant). Three patients showed active disease (oral ulcers) at the time of conception. Disease flares were observed during gestation for 25/153 (16.3%) pregnancies, mainly mucocutaneous (oral and genital ulcers, erythema nodosum) and joint (arthralgia and arthritis) manifestations. A total of 27/153 (17.6%) cases of disease flares were observed after pregnancy, with mucocutaneous (oral and genital ulcers, erythema nodosum) and joint (arthralgia and arthritis) involvement, as well as anterior uveitis. Miscarriages, preterm delivery, pre-eclampsia and eclampsia were observed in 27/153 (17.6%), 19/153 (12.4%), 6/153 (3.9%) and 2/153 (1.3%) pregnancies, respectively. No cases of neonatal complications or death were observed. CONCLUSIONS:The results of the present study underlined that: (a) BS disease clinical symptoms do not appear to worsen during pregnancy and (b) the pregnancy does not appear to be associated with increased gestational complications and adverse maternal-fetal outcomes in case of BS. However, due to the potential adverse events, especially of the vascular involvement, a strong multidisciplinary pregnancy follow-up is recommended.
ObjectiveBosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the Systemic Sclerosis Progression Investigation (SPRING) registry.MethodsPatients with SSc from the SPRING registry, meeting 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria with data on PAH onset, DU status, BOS exposure, and at least 1 year of follow-up between 2015 and 2020, and having no known PAH at baseline, were included. PAH was diagnosed with right heart catheterization during the follow-up, and its incidence rate (IR) was calculated. Kaplan-Meier curves were determined, and multivariate regression identified PAH risk factors.ResultsAmong 727 eligible patients with SSc, followed for a median of 2.0 years, 54 (7.4%) developed PAH (IR 3.71 per 100 patient-years [PYs]). Patients with DU who were never exposed to BOS had a higher incidence of PAH (IR 4.90 per 100 PYs) compared to those exposed to BOS, whose rates matched those without DU and who were never exposed to BOS. Risk factors independently associated with PAH development included DU (hazard ratio [HR] 1.86), age (HR 1.05), modified Rodnan skin score > 4 (HR 2.07), interstitial lung disease (HR 2.29), and acetylsalicylic acid treatment (HR 1.78).ConclusionIn our cohort, the presence of DU was confirmed as a leading risk factor for PAH development, and BOS use for DU prevention may reduce this risk. Only patients with DU who were not using BOS had an increased PAH incidence.
Digital ulcers (DU) are one of the most frequent manifestations in systemic sclerosis (SSc). The presence of DU seems to be a sentinel sign of internal organ involvement and is related to a poor prognosis of the disease. The aim of this study was to evaluate the prevalence and the relationship of DU with clinical manifestations/variants in a large SSc cohort from the SPRING registry. SSc patients fulfilling the ACR/EULAR 2013 classification criteria without missing data on digital ulcers were enrolled in a cross-sectional study. Logistic regression models were built to test the association between the presence of DU and SSc-related features. Among 1873 eligible SSc patients, the presence of DU was significantly associated with gastrointestinal involvement (OR 1.88, 2.04 and 1.74; p < 0.001) and serum ATA positivity (OR 2.15; p < 0.001), as well as with telangiectasias, sclerodactyly, digital pitting scar, and calcinosis (OR 1.40, p = 0.005; OR 3.43, p < 0.001, OR 9.12, p < 0.001 and OR 2.77, p < 0.001; respectively). In the multivariable regression models, even after adjustment for covariates, ATA positivity (OR 1.76, p = 0.039), puffy fingers (OR 2.82, p < 0.001), and a higher revEUSTAR-AI (OR 6.63, p < 0.001) emerged as risk factors for the presence of DU. Moreover, a low presence of DU was recorded in SSc patients with a history of previous immunosuppressive treatments (OR 0.53, p = 0.032). In our Italian SSc cohort, DUs were significantly associated with the presence of puffy fingers, high revEUSTR-AI, and ATA seropositivity. Noteworthy, immunosuppressive treatments were associated with a low rate of DU, suggesting that they might contribute to the prevention of these harmful manifestations. Key Points • Digital ulcers were significantly associated with the presence of puffy fingers, high disease activity, and anti-Scl70 seropositivity. • Immunosuppressive treatments were associated with a low rate of digital ulcers, suggesting that they might contribute to the prevention of these harmful manifestations.
OBJECTIVES:The aim of this study was to provide evidence- and expert-based indications for the use of non-steroidal anti-inflammatory drugs (NSAIDs) in psoriatic arthritis (PsA). METHODS:A working group, composed of six rheumatologists with known expertise in the management of PsA and seven methodologists, identified key research questions related to NSAID use in PsA, which guided the systematic literature review (SLR) in Medline and Embase databases. RCTs and observational studies published until 26/1/2022 were included for efficacy and safety questions, respectively. Based on the results of the systematic search, the working group developed statements, which were evaluated by a multidisciplinary group of external reviewers through a Delphi exercise. RESULTS:The SLR retrieved only 7 manuscripts of interest, 5 RCTs and 2 observational studies. The drugs evaluated in the RCTs were indomethacin, diclofenac, ibuprofen, nimesulide, and celecoxib. These studies addressed peripheral joint involvement but not the other domains of PsA. Nimesulide and celecobix were reported to be significantly more effective than placebo in controlling joint inflammatory-related symptoms in the short-term. Based on this evidence and on expert opinion, the working group developed 12 statements on the use of NSAIDs in PsA. CONCLUSIONS:This study provides a set of indications that may be helpful to the practicing rheumatologist in the prescription of NSAIDs for the relief of the symptoms due to the various clinical manifestations of PsA.
OBJECTIVES:To develop a practical consensus for standardizing communication between rheumatologists and radiologists regarding magnetic resonance imaging (MRI) of the sacroiliac joints and spine in the diagnosis and management of axial spondyloarthritis (axSpA). METHODS:A task force comprising six rheumatologists and five musculoskeletal radiologists with expertise in axSpA imaging reviewed the Assessment of SpondyloArthritis International Society (ASAS) framework to draft initial recommendations and define project goals. A broader expert panel (21 rheumatologists, 19 radiologists) then participated in a voting process to refine and validate these recommendations. Final endorsement was sought from the steering committees of the Italian Society of Medical and Interventional Radiology (SIRM) and the Italian Society of Rheumatology (SIR) using a modified Delphi method. RESULTS:Thirty-one recommendations were validated, organized into eight domains. Domain 1 outlines five overarching principles. Domain 2 comprises recommendations on clinical features, symptoms, and risk factors. Additional domains address MRI technical parameters, including image quality and sequencing (Domain 3), and standardized reporting criteria. For the sacroiliac joints (Domains 4 and 5), five signs of inflammation and six of structural damage are defined. For the spine (Domains 6 and 7), five inflammatory and four structural features are specified. Domain 8 provides guidance on report conclusions. The recommendations were endorsed by SIRM/SIR with 88.5% approval. CONCLUSION:This consensus offers structured guidance for MRI requesting and reporting in axSpA, fostering clear communication between radiologists and rheumatologists. The standardized approach aims to improve diagnostic accuracy and patient outcomes.
Background: Hormonal changes in menopause might interact with the presentation of underlying autoimmune diseases, such as systemic sclerosis (SSc). Objectives: Our study aimed to evaluate the association of (1) current menopausal status, (2) early menopause, and (3) disease onset during fertile or post-menopausal age on SSc clinical phenotype in a large SSc cohort from the Italian Systemic sclerosis Progression INvestiGation (SPRING-SIR) registry. Design: Female SSc patients from the SPRING-SIR registry, fulfilling the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 classification criteria, with data on SSc disease onset, menopausal status, and menopausal age, were eligible. SSc onset was categorized as pre-menopausal if SSc onset happened >1 year before menopause or as post-menopausal onset if it occurred >1 year after menopause. An early menopause was defined by a menopausal age <45 years. Methods: Descriptive statistics and regression models were built to test the association between current menopausal status, pre-menopausal disease onset, and early menopause with SSc-related features. Results: At baseline, 1157/1538 (75%) patients were in menopause, 632 (50.4%) had a pre-menopausal SSc onset, and 130 (14.4%) reported an early menopause. Post-menopausal patients had more frequent limited cutaneous SSc, anti-centromere antibody positivity, interstitial lung disease, and gastrointestinal manifestations. Pre-menopausal onset cases showed more frequent diffuse cutaneous involvement and peripheral vasculopathy. Patients with early menopause had more frequent peripheral vasculopathy and interstitial lung disease, being early menopause an independent risk factor for digital ulcers and lower diffusing capacity of the lung for carbon monoxide. Conclusion: Current post-menopausal status and early menopause may impact SSc presentation, being associated with vascular and gastrointestinal manifestations. Menopausal status and age should therefore be thoroughly addressed, aiming at better disease management.
Objectives The AsseSSing Impact in pSoriatic Treatment (ASSIST) study investigated prescribing in routine PsA care and whether the patient-reported outcome—PsA Impact of Disease questionnaire (PsAID-12)—impacted treatment. This study also assessed a range of patient and clinician factors and their relationship to PsAID-12 scoring and treatment modification. Methods Patients with PsA were selected across the UK and Europe between July 2021 and March 2022. Patients completed the PsAID questionnaire and the results were shared with their physician. Patient characteristics, disease activity, current treatment methods, treatment strategies, medication changes and patient satisfaction scores were recorded. Results A total of 503 patients were recruited. Some 36.2% had changes made to treatment, and 88.8% of these had treatment escalation. Overall, the mean PsAID-12 score was higher for patients with treatment escalation; increase in PSAID-12 score is associated with increased odds of treatment escalation (odds ratio 1.58; P < 0.0001). However, most clinicians reported that PsAID-12 did not impact their decision to escalate treatment, instead supporting treatment reduction decisions. Physician’s assessment of disease activity had the most statistically significant effect on likelihood of treatment escalation (odds ratio 2.68, per 1-point score increase). Escalation was more likely in patients not treated with biologic therapies. Additional factors associated with treatment escalation included: patient characteristics, physician characteristics, disease activity and disease impact. Conclusion This study highlights multiple factors impacting treatment decision-making for individuals with PsA. PsAID-12 scoring correlates with multiple measures of disease severity and odds of treatment escalation. However, most clinicians reported that the PsAID-12 did not influence treatment escalation decisions. Psoriatic Arthritis Impact of Disease (PsAID) scoring could be used to increase confidence in treatment de-escalation.
OBJECTIVE:Elderly-onset systemic sclerosis (SSc) is relatively uncommon, and its clinical phenotype and prognostic implications remain poorly characterized, with conflicting evidence regarding disease course and outcomes. METHODS:Within the Italian SPRING (Systemic Sclerosis PRogression INvestiGation) registry, we compared demographic and clinical characteristics of patients with elderly-onset SSc (≥70 years at the time of the first non-Raynaud's manifestation) to those with younger onset. Cross-sectional analyses, multivariable logistic regression, and unsupervised cluster analysis were conducted to identify features associated with elderly-onset SSc. Longitudinal analysis was performed to assess mortality risk within SSc patients and compared to the general Italian population. RESULTS:Elderly-onset accounted for 8.5 % (160/1893) SSc cases in SPRING. These patients exhibited fewer peripheral vascular complications (digital ulcers: 13 % vs. 23 %; p = 0.016), higher prevalence of anticentromere antibodies (60 % vs. 39 %; p = 0.007), a lower prevalence and likelihood of diffuse skin subset (OR 0.40; 95 % CI 0.19-0.83) but an increased risk of pulmonary arterial hypertension confirmed on right-heart catheterization (OR 14.1; 95 % CI 3.68-54.5) at multivariate analysis. As expected, patients with elderly onset SSc had an increased risk of death compared to younger-onset individuals. Compared with the age-, sex-, and calendar year-matched general Italian population, patients with SSc showed a fivefold increased mortality, with a trend toward a higher risk in young-onset (SMR 6.3; 95 %CI 4.1-9.1) compared with elderly-onset (SMR 4.5; 95 %CI 2.4-7.7) cases. CONCLUSIONS:Elderly-onset identifies a distinct clinical subset of SSc, mainly characterized by mild cutaneous and peripheral vascular involvement, but showing a greater burden of pulmonary vascular disease and increased mortality compared to the age-matched general population.
Limited data are available on sex differences in the disease characteristics, burden and treatment outcomes of early psoriatic arthritis (PsA). The “Spondyloarthritis Italian Registry: Evidence from a National Pathway” (SIRENA) study is a prospective, observational, Italian study conducted in 23 Rheumatology sites on patients with recent diagnosis of spondyloarthritis and naïve to any disease-modifying antirheumatic drugs (DMARDs). The study included 203 patients with early PsA, observed per clinical practice, who were studied regarding the influence of sex on the disease presentation and the likelihood of achieving minimal disease activity (MDA). Clinical and patient-reported outcome (PRO) measures were collected at both study entry and each follow-up visit until 24 months. Treatment was assigned by the treating rheumatologist based on standard clinical practice. At baseline, 87