Lung involvement in "stable" undifferentiated connective tissue diseases: a rheumatology perspective

Previous studies of the occurrence of interstitial lung disease (ILD) in undifferentiated connective tissue diseases (UCTD) were conducted in patients admitted to Respiratory Medicine Units. The aim of the present prospective study was to investigate lung involvement in UCTD patients admitted to a Rheumatology Unit. Eighty-one consecutive UCTD patients were enrolled in the study. Each patient underwent history and physical examination, routine laboratory investigations, antinuclear antibody (ANA) profiling, B-mode echocardiography, and lung function study according to previously reported methods. Lung high resolution computed tomography (HRCT) was performed in patients who provided informed consent. Six patients (7.4%) had a history of grade II dyspnea. Three of them had a DLCO ranging from 42 to 55% of the predicted value; and a HRCT-documented ILD with a non-specific interstitial pneumonia (NSIP) pattern. Symptoms in the other three patients were due to cardiac disease. None of the 75 asymptomatic patients, had relevant findings at physical examination, 26/75 had a DLCO <80% (<70% in 10 cases). Of these, 3 of the 30 patients who underwent lung HRCT were affected by NSIP-ILD. Six of the 81 enrolled were affected by ILD, which was symptomatic in three patients. A higher percentage of patients had a reduced DLCO. The latter finding may reflect a preradiographic ILD or a preechocardiographic pulmonary vascular disease

CXCL4 in undifferentiated connective tissue disease at risk for systemic sclerosis (SSc) (previously referred to as very early SSc)

The aim of the study was to evaluate CXCL4 levels in undifferentiated connective tissue disease at risk for SSc (UCTD-SSc-risk) and confirm its increase and investigate its prognostic value. Serum CXCL4 levels were measured in 45 patients and 24 controls. CXCL4 was significantly higher in UCTD-SSc-risk patients than in controls. It resulted higher in patients with a shorter disease duration and in those lacking capillaroscopic alterations. We confirm that CXCL4 levels are increased in UCTD-risk-SSc patients. Further studies are needed to investigate the role of CXCL4 assessment in UCTD-risk-SSc

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P &lt; 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P &lt; 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P &lt; 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P &lt; 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P &lt; 0.001; OR(BP) = 2.4, P &lt; 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P &lt; 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P &lt; 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

Latest Advances in Annuloplasty Protheses for Valvular Reconstructive Surgery

This is the third and final part of our update on the latest advances in cardiac valvular replacement. Part 1 was dedicated to cardiac valvular replacement, and Part 2 focused on transcatheter cardiac valvular treatment. This part concerns annuloplasty prostheses for valvular reconstructive surgery. The number of patients undergoing surgical heart valve repair has been increasing, particularly in high-volume centers. Annuloplasty is now considered the gold standard in mitral valve regurgitation repair secondary to degenerative, ischemic and idiopathic dilated cardiomyopathy disease. The techniques of mitral valve reconstruction have been well established, but controversies remain regarding the type of annuloplasty ring to be used. The available annuloplasty rings include rigid, flexible, complete, partial, and semi-rigid/flexible. The choice of annuloplasty ring has been the focus of extensive investigation and debate, but to date it still largely remains a matter of "surgeon's preference" rather than an evidence-based selection. Functional tricuspid regurgitation was traditionally treated by the classic De Vega annuloplasty, but has since evolved after the development of prosthetic tricuspid annuloplasty. Head-to-head comparisons have demonstrated superior long-term outcomes with device-based annuloplasty compared to suture-based surgery, but the type of ring to be used (flexible versus rigid) has recently been questioned, without reaching definitive conclusions. In contrast to mitral and tricuspid valve repair, aortic repair is more difficult with respect to specific valve features. Annuloplasty is considered to play a key role in controlling aortic regurgitation and preventing recurrence after valve repair. Various modifications of annuloplasty have been advocated (internal/external, with/without ring (suture), rigid/flexible ring). but none of them has become a de facto standard. This paper describes the various rings that are available to help orient surgeons and to serve as a reference for students

Latest Advances in Annuloplasty Protheses for Valvular Reconstructive Surgery

This is the third and final part of our update on the latest advances in cardiac valvular replacement. Part 1 was dedicated to cardiac valvular replacement, and Part 2 focused on transcatheter cardiac valvular treatment. This part concerns annuloplasty prostheses for valvular reconstructive surgery. The number of patients undergoing surgical heart valve repair has been increasing, particularly in high-volume centers. Annuloplasty is now considered the gold standard in mitral valve regurgitation repair secondary to degenerative, ischemic and idiopathic dilated cardiomyopathy disease. The techniques of mitral valve reconstruction have been well established, but controversies remain regarding the type of annuloplasty ring to be used. The available annuloplasty rings include rigid, flexible, complete, partial, and semi-rigid/flexible. The choice of annuloplasty ring has been the focus of extensive investigation and debate, but to date it still largely remains a matter of "surgeon's preference" rather than an evidence-based selection. Functional tricuspid regurgitation was traditionally treated by the classic De Vega annuloplasty, but has since evolved after the development of prosthetic tricuspid annuloplasty. Head-to-head comparisons have demonstrated superior long-term outcomes with device-based annuloplasty compared to suture-based surgery, but the type of ring to be used (flexible versus rigid) has recently been questioned, without reaching definitive conclusions. In contrast to mitral and tricuspid valve repair, aortic repair is more difficult with respect to specific valve features. Annuloplasty is considered to play a key role in controlling aortic regurgitation and preventing recurrence after valve repair. Various modifications of annuloplasty have been advocated (internal/external, with/without ring (suture), rigid/flexible ring). but none of them has become a de facto standard. This paper describes the various rings that are available to help orient surgeons and to serve as a reference for students

Longitudinal analysis of quality of life in patients with undifferentiated connective tissue diseases

To prospectively assess the quality of life (QoL) of patients affected by undifferentiated connective tissue diseases (UCTDs) and to identify factors associated with changes over time

Serum CXCL4 increase in primary Sjögren’s syndrome characterizes patients with microvascular involvement and reduced salivary gland infiltration and lymph node involvement

CXCL4 is an antiangiogenic and immunomodulatory chemokine. We aimed to investigate serum levels of CXCL4 in primary Sjögren’s syndrome (pSS), looking for associations with disease features. Thirty-nine consecutive pSS patients underwent clinical-serological assessment and nailfold videocapillaroscopy (NVC). Thirty-six patients and 30 controls affected by osteoarthritis were also investigated for serum levels of CXCL4 and soluble E-selectin (sE-selectin). CXCL4 was higher in pSS patients than in controls (1.79 [0.2–11.18] vs 1.023 ng/ml [0.02–14.45], p < 0.05), particularly in those without anti-La/SSB antibodies (2.89 [1.01–11.18] vs 1.69 ng/ml [0.2–2.72], p < 0.05), while it was lower in pSS patients with a focus score ≥1 at lip biopsy (1.44 [0.86–2.1] vs 2.24 ng/ml [1.64–3.25], p < 0.05) and clinically evident lymphadenopathy (1.53 [0.38–1.7] vs 2.08 ng/ml [1.45–3.03], p < 0.05). CXCL4 correlated with disease duration (r = 0.35, p < 0.05) and sE-selectin (r = 0.45, p < 0.01). Patients with Raynaud’s phenomenon (RP) had more frequently abnormal CXCL4 levels than patients without RP (11/15 vs 3/21, p < 0.001), enlarged capillaries (14/16 vs 7/23, p < 0.001) and capillary loss at NVC (14/16 vs 6/23, p < 0.001). The hitherto unknown association of increased serum CXCL4 with features of microvascular impairment in pSS, along with the negative association with features of lymphocytic response (i.e., the absence of subset disease-specific autoantibodies, a low focus score, and the absence of lymphadenopathy) suggest clarifying the possible implication of this chemokine in pSS pathogenesis in larger studies