Survival and prognostic factors from a multicentre large cohort of unselected Italian systemic sclerosis patients.

OBJECTIVES: Survival and death prognostic factors of SSc patients varied during the past decades. We aimed to update the 5- and 10-year survival rates and identify prognostic factors in a multicentre cohort of Italian SSc patients diagnosed after 2009. MATERIAL AND METHODS: Patients who received a diagnosis of SSc after 1 January 2009 and were longitudinally followed up in four Italian rheumatologic centres were retrospectively assessed up to 31 December 2020. Overall survival of SSc patients was described using the Kaplan-Meier method. Predictors of mortality at 10-year follow-up were assessed by the Cox regression model. A comparison of our cohort with the Italian general population was performed by determining the standardized mortality ratio (SMR). RESULTS: A total of 912 patients (91.6% females, 20% dcSSc) were included. Overall survival rates at 5 and 10 years were 94.4% and 89.4%, respectively. The SMR was 0.96 (95% CI 0.81, 1.13), like that expected in the Italian general population. Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) associated with pulmonary hypertension (PH) significantly reduced survival (P < 0.0001). Main death predictors were male gender (HR = 2.76), diffuse cutaneous involvement (HR = 3.14), older age at diagnosis (HR = 1.08), PAH (HR = 3.21), ILD-associated PH (HR = 4.11), comorbidities (HR = 3.53) and glucocorticoid treatment (HR= 2.02). CONCLUSIONS: In the past decade, SSc patients have reached similar mortality of that expected in the Italian general population. Male gender, diffuse cutaneous involvement, comorbidities and PAH with or without ILD represent the main poor prognostic factors.

Cardiovascular risk estimation with 5 different algorithms before and after 5 years of bDMARD treatment in rheumatoid arthritis.

BACKGROUND: Assessing cardiovascular (CV) risk represents a challenge for clinicians because more variables can impact CV risk. The aim of this study was to evaluate the change of CV risk after 5 years of biological treatment in rheumatoid arthritis (RA) patients and impact of prolonged low disease activity on 5 different CV risk algorithms. MATERIALS AND METHODS: We estimated the CV risk, at baseline and at 5-year follow-up (FU), with the Systematic COronary Risk Evaluation(SCORE) charts, the algorithm 'Progetto Cuore', the QRISK3-2018 score, the Reynold Risk Score(RRS) and the Expanded Risk Score in RA(ERS-RA). Clinical disease activity index(CDAI) was used to define RA activity. Wilcoxon signed-rank test was used to compare CV risk scores. RESULTS: In 110 patients with a 5-year FU on biological disease-modifying anti-rheumatic drug treatment, we observed an increase in the 10-year CV risk estimated by SCORE charts [from mean (SD) 0.9% (1.4) to 1.1% (1.5), P < .001], 'Progetto Cuore' [from mean (SD) 5.5% (7.2) to 6.2% (6.8), P < .001], QRISK3-2018 [from mean (SD) 9.3% (10.1) to 11.9% (10.8), P < .001) and RRS [from mean (SD) 5.6% (6.4) to 6.2% (7.5), P < .05], mainly due to age raise. ERS-RA highlighted a significant decrease of estimated CV risk in patients with persistent CDAI ≤ 10[from mean (SD) 9.6% (11.2) to 7.3% (6.4), P < .05], despite age increase and its impact on the CV risk score. CONCLUSIONS: Algorithms commonly used to estimate 10-year CV risk in RA perform differently. Scores that include specific inflammatory RA-related variables seem to decrease with amelioration of disease activity. Further investigations are warranted to explore the predictive value of their changing over time.

Unmet needs in ANCA-associated vasculitis: Physicians' and patients' perspectives.

In recent years, clinical research has increased significantly and therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis have improved. However, there are still unanswered questions and unmet needs about AAV patients. The purpose of this review is to examine the frontiers of research related to emerging biomarkers eventually predicting relapse, and new therapeutic approaches, not to mention new quality of life assessment tools. Identifying predictors of relapse may help optimize therapeutic strategies, minimize disease recurrence, and reduce treatment-related side effects. In addition, it is important to recognize that patients may suffer long-term consequences of the disease and its treatment, which, although life-saving, is often associated with significant side effects. Our goal, therefore, is to highlight what has been achieved, the pitfalls, and what still needs to be done, comparing the views of physicians and patients.

HLA Allele Prevalence in Disease-Modifying Antirheumatic Drugs-Responsive Enthesitis and/or Arthritis Not Fulfilling ASAS Criteria: Comparison with Psoriatic and Undifferentiated Spondyloarthritis.

Spondyloarthritis (SpA) is a group of inflammatory rheumatic diseases characterized by common clinical features, such as inflammatory enthesitis, arthritis and/or back pain. SpA is strongly associated with human leukocyte antigen (HLA) class I allotype B27. Ankylosing spondylitis has historically been the SpA subgroup with one of the strongest, best-proven associations with HLA-B27. The remaining SpA subgroups, namely psoriatic arthritis (PsA), inflammatory bowel diseases-associated arthritis/spondylitis, reactive arthritis, and undifferentiated SpA (uSpA), have also been associated with HLA allotypes other than HLA-B27. In this retrospective study, we analyzed the association between the HLA class I and II haplotypes and the susceptibility to enthesitis and/or arthritis (E/A). Special attention was paid to E/A responding to disease-modifying antirheumatic drugs (DMARDs) not fulfilling ASAS classification criteria (ASAS-), as compared to ASAS+ forms including PsA and uSpA. The whole E/A group showed significant independent associations with HLA-A28(68), B27, Cw3, Cw12, and DQ1; taken singly, PsA was associated with HLA-B27 and DQ1, uSpA with HLA-B16(38,39) and B27, and E/A ASAS- with HLA-A28(68), Cw8, and Cw12. This study identified novel risk HLA allotypes for different SpA subgroups in an Italian population. HLA typing could aid the diagnosis and treatment of E/A subgroups, including DMARDS-responsive forms not fulfilling ASAS classification criteria.

Thoracic lymphadenopathy as possible predictor of the onset of interstitial lung disease in systemic sclerosis patients without lung involvement at baseline visit: A retrospective analysis.

Objective: To evaluate clinical, laboratory, or radiographic predictors of the onset of interstitial lung disease in systemic sclerosis. Methods: Sixty-five out of 220 systemic sclerosis outpatients, without interstitial lung disease at baseline and with ⩾3 chest high resolution computed tomography scans during follow-up were recruited. Thoracic lymphadenopathy and interstitial lung disease were assessed by chest high resolution computed tomography. Hazard ratio (95% confidence interval) of interstitial lung disease occurrence was assessed by Cox regression models, adjusting patient's demographics and disease characteristics. Sensitivity, specificity, and accuracy of the interstitial lung disease predictors were evaluated by receiver operating characteristic analysis. Results: The development of interstitial lung disease was observed in 44/65 (68%) patients. Thoracic lymphadenopathies was detected in 40/65 (61%) patients, of whom 36 (82%) developed interstitial lung disease, but only four patients with thoracic lymphadenopathies did not develop ILD at last visit of follow-up (19%) (p = 0.0001). Adjusted hazard ratio of systemic sclerosis-interstitial lung disease onset was 5.8 (95% confidence interval, 2.0-16.5) for thoracic lymphadenopathy, which preceded by 108 ± 98 weeks the systemic sclerosis-interstitial lung disease detection. Thoracic lymphadenopathy had 84% specificity, 81% sensitivity, and 0.82 accuracy to predict interstitial lung disease. In particular, anticentromere antibodies or limited cutaneous subset of systemic sclerosis patients with thoracic lymphadenopathy showed earlier interstitial lung disease onset than those without lymphadenopathy. In addition, patients who developed interstitial lung disease had higher frequency of anti-Scl-70 (57% vs 19%; p = 0.009) and diffuse cutaneous subset (29% vs 3%; p = 0.02) than those who did not. Conclusions: Thoracic lymphadenopathy was the strongest independent predictor of systemic sclerosis-interstitial lung disease, mostly in anticentromere antibodies and limited cutaneous subset of systemic sclerosis patients. Further prospective studies are needed to confirm our preliminary data and to understand whether thoracic lymphadenopathies may have a pathogenetic role in interstitial lung disease development.

Antiphospholipids Syndrome Complicated by a Systemic Capillary Leak-Like Syndrome Treated With Steroids and Intravenous Immunoglobulins: A Case Report.

This report describes the onset of systemic capillary leak (SCL)-like syndrome in a 30-year-old woman with antiphospholipids syndrome (APS) during puerperium.Twelve hours after a cesarean section, she presented a sudden fever and abdominal pains followed by dyspnea, severe edema of the limbs and pelvis.Computer tomography shows congestion of interstitial pulmonary parenchyma, pericardial and pleural effusion, edema of intestinal wall and of perivisceral adipose tissue, and periportal lymphedema. Laboratory tests showed neutrophilic leukocytosis, hypoalbuminemia, and an increase of erythrocyte sedimentation rate and C-reactive protein. Because fever and raised inflammation parameters are not observed in idiopathic capillary leak syndrome (SCLS; Clarkson disease), a diagnosis of SCL-like syndrome was made.Albumin solution, high-dose methylprednisolone and intravenous immunoglobulins (IVIG) infusion were administered with a rapid improvement of her clinical condition.The prompt treatment with steroids and IVIG likely prevented the life-threatening shock syndrome that can occur in SCLS, with acute hypotensive attacks, and severe limbs edema requiring fasciotomy.All clinical and laboratory findings supported autoinflammation as the underlying pathogenic mechanism of the syndrome. The data indicate that SCL-like syndrome can be considered a novel clinical syndrome, which can complicate APS.