Acute Respiratory Failure in Autoimmune Rheumatic Diseases: A Review.

This review examines respiratory complications in autoimmune rheumatic diseases within intensive care units (ICUs). The respiratory system, primarily affected in diseases like rheumatoid arthritis, systemic lupus erythematosus, and scleroderma, often leads to respiratory failure. Common manifestations include alveolar hemorrhage, interstitial fibrosis, and acute respiratory distress syndrome. Early recognition and treatment of non-malignant conditions are crucial to prevent rapid disease progression, with ICU mortality rates ranging from 30% to 60%. Delayed immunosuppressive or antimicrobial therapy may result in organ system failure. Collaboration with rheumatic specialists is vital for accurate diagnosis and immediate intervention. Mortality rates for rheumatic diseases in the ICU surpass those of other conditions, underscoring the need for specialized care and proactive management. The review emphasizes comprehensive assessments, distinguishing disease-related complications from underlying issues, and the importance of vigilant monitoring to enhance patient outcomes.

Bathing in Atopic Dermatitis in Pediatric Age: Why, How and When.

Atopic dermatitis is a chronic inflammatory skin disease. The treatment plays an important role in influencing the patients' quality of life. The basic management consists of appropriate skin cleansing, including bathing and eventually using bathing additives. Recommendations regarding frequency and duration of bathing, water temperature and usefulness of bathing additives are widely different, often leading to confusion among patients. This review aims to give insights into the best bathing practices and the use of bathing additives in atopic dermatitis in children. Several bathing additives, including bleach baths, commercial baby cleansers, bath baby oils and bath salt, appear to be promising adjunctive therapies for atopic dermatitis due to their anti-inflammatory, anti-bacterial, anti-pruritus and skin barrier repair properties through different mechanisms of action. However, their efficacy and safety are not fully understood in some cases. The usefulness of other bath additives, such as acidic and more natural substances (green tea extracts, pine tar, sodium bicarbonate), is still under investigation. Further studies are needed to determine their optimal use to achieve clinical benefit safely.

Clinical features, treatment and outcomes of Italian children with enthesitis-related arthritis and juvenile psoriatic arthritis: a cross-sectional cohort study.

OBJECTIVES: Limited information is available on the clinical features, treatment modalities and outcomes of the juvenile idiopathic arthritis (JIA) categories of enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA). This study was aimed to describe the characteristics of Italian children with ERA and JPsA and to compare them with those of patients with the other categories of JIA. METHODS: Patients were part of a multinational sample included in a study aimed to investigate the prevalence of disease categories, treatment approaches, and disease status in patients from across different geographical areas (EPOCA Study). All patients underwent a retrospective assessment, based on the review of clinical chart, and a cross-sectional evaluation, which included assessment of physician- and parent-reported outcomes and laboratory tests, and recording of ongoing therapies. RESULTS: Of the 9081 children with JIA enrolled in the EPOCA Study, 1300 were recruited at 18 paediatric rheumatology centres in Italy. 45 (3.5%) had ERA and 49 (3.8%) had JPsA. Several remarkable differences in demographic features and frequency of articular and extra-articular manifestations, disease damage, impairment in physical function and health-related quality of life, school-related problems, comorbidities, and ongoing treatments were observed between ERA and JPsA and the other JIA categories. CONCLUSIONS: We described the characteristics of Italian children with ERA and JPsA and highlighted their peculiarities and their differences from the other JIA subsets. These data provide useful insights for future revisions of JIA classification and a benchmarking against which the features from other cohorts may be compared.

Feasibility and long-term outcomes of surgery for primary thoracic synovial sarcoma.

OBJECTIVES: Primary thoracic synovial sarcoma (SS) is a rare, high-grade, malignancy. Involvement of vital organs is frequent and may decrease the benefits of surgical resection. We reviewed our practice at a highly experienced thoracic-surgery centre to assess early- and long-term outcomes after surgery. METHODS: We conducted a retrospective, observational, single-centre study of patients undergoing curative-intent surgery for primary thoracic SS between 1 January 2000 and 31 January 2021 as part of a multidisciplinary management. We assessed demographics, medical history, histopathology and follow-up information. RESULTS: We enrolled 20 patients (13 males) with a median age of 40 years old and a median tumour size of 11 cm. Neoadjuvant chemotherapy was administered to 13 patients. Surgery consisted in extrapleural pneumonectomy (n = 7), extrapleural lobectomy (n = 5), chest wall resection (n = 4) or tumour resection (n = 4). R0 resection was achieved in 16 (80%) patients. Adjuvant therapy was given to 13 patients. 6 patients developed postoperative complications. The median hospital stay was 11.5 days. Overall survival at 2 and 5 years was 51% and 22%, respectively; median overall survival was 25 months and median disease-free survival was 8.5 months. Relapses occurred in 15 patients. By univariate analysis, incomplete resection was the only significant predictor of survival (P = 0.01). CONCLUSIONS: Primary thoracic SS is an aggressive disease. Surgery included in a multimodal treatment may contribute to achieving a good outcome, providing that an R0 resection is obtained. Given the considerable technical challenges of surgery, patient selection and referral to an experienced centre are crucial to minimize morbidity and mortality.

Multicenter randomized controlled trial comparing digital and traditional chest drain in a VATS pulmonary lobectomy cohort: interim analysis.

BACKGROUND: The usefulness of digital chest drain is still debated. We are carrying out a study to determine if the use of a digital system compared with a traditional system reduces the duration of chest drainage. To evaluate safety, benefit, or futility of this trial we planned the current interim analysis. METHODS: An interim analysis on preliminary data from ongoing investigator-initiated, multicenter, interventional, prospective randomized trial. Original protocol number: (NCT03536130). The interim main endpoint was overall complications; secondary endpoints were the concordance between the two primary endpoints of the RCT (chest tube duration and length of hospital stay). We planned the interim analysis when half of the patients have been randomised and completed the study. Data were described using mean and standard deviation or absolute frequencies and percentage. T-test for unpaired samples, Chi-square test, Poisson regression and absolute standardized mean difference (ASMD) were used. P-value < 0.05 was considered significant. RESULTS: From April 2017 to November 2018, out of 317 patients enrolled by 3 centers, 231 fulfilled inclusion criteria and were randomized. Twenty-two of them dropped out after randomization. Finally, 209 patients were analyzed: among them 94 used the digital device and 115 the traditional one. The overall postoperative complications were 35 (16.8%) including prolonged air leak (1.9%). Mean chest tube duration was 3.6 days (SD = 1.8), with no differences between two groups (p = 0.203). The overall difference between hospital stay and chest tube duration was 1.4 days (SD = 1.4). Air leak at first postoperative day detected by digital and traditional devices predicted increasing in tube duration of 1.6 day (CI 95% 0.8-2.5, p < 0.001) and 2.0 days (CI 95% 1.0-3.1, p < 0.001), respectively. CONCLUSIONS: This interim analysis supported the authors' will to continue with the enrollment and to analyze data once the estimated sample size will be reached. TRIAL REGISTRATION: Trial registration number NCT03536130 , Registered 24 May 2018 - Retrospectively registered.

Prognostic Value of 18F-FDG PET/CT Metabolic Parameters in Surgically Treated Stage I Lung Adenocarcinoma Patients.

PURPOSE OF THE REPORT: This article aims to explore the prognostic role of 18F-FDG PET/CT metabolic parameters in stage I lung adenocarcinoma patients. PATIENTS AND METHODS: One hundred eighty pathological stage I lung adenocarcinoma patients were retrospectively reviewed. Semiquantitative analysis of FDG tumor uptake was performed with TrueD software on the Siemens Leonardo workstation. SUVmean and MTV were calculated using SUV threshold of 41% of SUVmax; the total lesion glycolysis (TLG) was calculated as the product of SUVmean and MTV. Correlation was evaluated using Spearman correlation coefficient. Maximally selected rank statistics was performed to detect the optimal cutoff used for dichotomizing each PET parameter (6.5 for SUVmean, 9.6 for SUVmax, and 19.1 for TLG). RESULTS: Our main finding was the significant correlation between 18F-FDG PET/CT parameters (SUVmean, SUVmax, and TLG) and disease-free survival in pathologic stage I non-small cell lung cancer. SUVmean has the greatest accuracy in recurrence prediction (integrated area under the curve, 0.803; 95% confidence interval, 0.689-0.918). We run the maximally selected rank statistics to provide the classification of observations in 2 groups by a continuous predictor parameter; the free from recurrence rate was significantly greater in patients with SUVmean ≤6.5, SUVmax ≤9.6, and TLG ≤19.1. CONCLUSIONS: Our research supports the hypothesis that SUVmean, SUVmax, and TLG are well correlated with free from recurrence rate in stage I adenocarcinoma patients, subjected to pulmonary lobectomy. Our findings also indicate these markers as promising prognostic indicators.

Bubbles-in-the-chamber vs digital screen in chest drainage: A blind analysis of compared postoperative air leaks evaluation.

BACKGROUND: Chest drainage systems are affected by intra and inter-observer variability and poor sensibility in detecting minimal or apparent air leaks. OBJECTIVES: Overcome intra and inter-observer variability in detecting air leaks. METHODS: After surgery, a single apical chest tube was connected to the Drentech™ PalmEVO device and air leaks were checked twice a day by observation of both bubbles-in-the-chamber and digital data. RESULTS: On a total of 624 observations, disagreement between digital and traditional systems was recorded in 60(9.6%) cases. In 25(21.4%) patients, a disagreement was recorded. Overall, the digital evaluation influenced clinical management in 13(52%). In 10(40%) patients with temporary discordant features, the presence of high pleural fluid output led to a progressive final concordance. CONCLUSIONS: Disagreement between traditional and digital systems in checking air leaks is not negligible. Digital systems could give advantages in making an objective assessment of air leaks, standardizing the timing of chest tube removal.

Transbronchial Cryobiopsies in Lung Allograft Recipients for Surveillance Purposes: Initial Results.

Transbronchial biopsy (TBB) using standard forceps is the main procedure to establish the presence of lung allograft rejection (AR) after lung transplantation. Few studies report the use of the transbronchial cryobiopsy (TCB) as a scheduled procedure for surveillance purposes in lung allograft, despite this the technique yields larger biopsies. We aimed to analyze the diagnostic yield and potential complications of TCB compared with conventional forceps biopsy for acute rejection surveillance in lung transplantation. In our center, TCBs are performed to monitor lung allografts at 3, 6, and 12 months after transplantation. From March 2018 to September 2019 TCBs were performed in 54 lung transplanted patients for surveillance purposes. Clinical and functional data, complications, and histologic results were collected. We analyzed through a retrospective study our first 75 cases of cryobiopsies for surveillance purposes in lung allograft recipients. The diagnostic rate of AR using TCB was 100% compared with 83% using conventional TBB. Also, diagnostic rate of airway inflammation and chronic rejection was 17% and 21% higher, respectively, for TCB compared with TBB. The overall major complication rate was 9%: 1 pneumothorax case required chest tube drainage and 6 moderate bleedings. Bleeding rate in the scheduled TCB group (8%) seems to be higher if compared with scheduled TBB group (1%). TCB seems to be safe and effective for diagnosis of lung AR compared with transbronchial conventional forceps biopsy.

Bone Marrow Foot Oedema in Adolescents: The Role of Vitamin D.

BACKGROUND: Bone marrow oedema (BMO) in children/adolescents is a rare clinical condition without an etiologic cause. It is associated with typical increased signal intensity on T2-weighted magnetic resonance images (MRI) and an increase in bone turnover in which vitamin D plays a pivotal role. No treatment guidelines for these young patients are to date available. METHODS: We performed a retrospective study in a pediatric setting of 13 patients with diagnosis of primary BMO of the foot on the basis of clinical and radiological findings. Data collection included sex, age, patient history, symptoms at presentation, clinical examination, laboratory bone turnover markers, vitamin D levels, MRI, treatment, and outcome. RESULTS: Vitamin D deficiency or insufficiency was found in 76.9% of cases. All patients were treated with adequate vitamin D daily intake, a short course of analgesic therapy, physical therapy, avoiding detrimental feet and ankle immobilization. All fully recovered in 3-month lag period. CONCLUSIONS: Our data highlight that environmental factors, such as underestimated articular or bone microtraumatisms, as well as joint hyper mobility, in a bone turnover milieu of vitamin D deficiency could be the cause of this clinical conditions. Adequate vitamin D supplementation, associated with physical and analgesic therapy, is crucial in the management of BMO.

Impact of Cold Ischemic Time on Airway Complications After Lung Transplantation: A Single-center Cohort Study.

BACKGROUND: Despite significant improvements in lung transplantation procedures, the incidence of airway complications (ACs) remains high (2%-18%); these complications are associated with high costs, great morbidities, and a decreased quality of life. There is general disagreement over potential risk factors determining ACs, including graft cold ischemic time (CIT). The aim of this study was to evaluate the association between CIT and ACs. METHODS: All patients undergoing lung transplantation between January 2011 and December 2017 were evaluated. We excluded retransplantations and patients with 90-day mortality. Demographic and clinical data regarding donors, recipients, and surgical procedures were analyzed using propensity score weighted marginal Cox regression model. RESULTS: Out of the 161 lung transplantations performed in the study timeframe, 147 fulfilled the inclusion criteria and supplied complete data to be analyzed. Median follow-up was 25.5 months (interquartile range = 35.2). Ten patients (6.8%) had late ACs; out of the 260 anastomoses considered, 14 proved to be complicated (5.4%). Median time to event was 5.5 months (range, 3-15). ACs were classified as bronchial stenosis (12) and malacia (2). Mean CIT was 446.6 minutes (range, 117-1200). Without considering time-to-event data, CIT was significantly higher in complicated anastomoses (P = .002). The unweighted marginal univariate Cox model showed a significant association between ACs and CIT (P < .001). The propensity score weighted marginal univariable Cox model confirmed this significant association (P < .001). CONCLUSIONS: The prolonged CIT time seems to be a risk factor for the development of late ACs; we endorse any measure that could limit CIT within 600 minutes.

Correction to: The Italian version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The family name of author Francesco La Torre was incorrect in the published article. The correct family name should read as La Torre F.

The Italian version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Italian language.The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents.The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity).A total of 1296 JIA patients (7.2% systemic, 59.5% oligoarticular, 21.4% RF negative polyarthritis, 11.9% other categories) and 100 healthy children, were enrolled in 18 centres. The JAMAR components discriminated well healthy subjects from JIA patients except for the Health Related Quality of Life (HRQoL) Psychosocial Health (PsH) subscales. All JAMAR components revealed good psychometric performances.In conclusion, the Italian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Baseline ultrasound examination as possible predictor of relapse in patients affected by juvenile idiopathic arthritis (JIA).

OBJECTIVES: To define the correlation between joint ultrasonography and clinical examination in patients with juvenile idiopathic arthritis (JIA) and to assess whether synovitis detected by ultrasonography in clinically inactive patients predicts arthritis flares. METHODS: 88 consecutive patients with JIA-46 (52%) with persistent oligoarthritis, 15 (17%) with extended oligoarthritis, 15 (17%) with rheumatoid factor-negative polyarthritis and 12 (14%) with other forms of JIA, all clinically inactive for a minimum of 3 months-underwent ultrasound (US) assessment of 44 joints. Joints were scanned at study entry for synovial hyperplasia, joint effusion and power Doppler (PD) signal. Patients were followed clinically for 4 years. RESULTS: US was abnormal in 20/88 (22.7%) patients and in 38/3872 (0.98%) joints. Extended oligoarthritis and rheumatoid factor-negative polyarthritis were more frequent in US-positive than in US-negative patients (35.0% vs 11.8% and 30.0% vs 13.2%, respectively; P=0.005). During 4 years of follow-up, 41/88 (46.6%) patients displayed a flare; 26/68 (38.2%) were US-negative and 15/20 (75%) were US-positive at baseline. Abnormality on US examination, after correction for therapy modification, significantly increased the risk of flare (OR=3.8, 95% CI 1.2 to 11.5). The combination of grey scale and PD abnormalities displayed a much higher predictive value of relapse (65%, 13/20) than grey scale alone (33%, 6/18). CONCLUSIONS: US abnormalities are a strong predictor of relapse at individual patient level. Irrespective of treatment, the risk of flare in US-positive versus US-negative patients was almost four times higher. In case of US abnormalities, patients should be carefully followed regardless of both the International League of Associations for Rheumatology and Wallace categories.

Relationship between body composition and both cardiovascular risk factors and lung function in systemic sclerosis.

The aims of this study were to evaluate body composition in systemic sclerosis (SSc) and to assess its association with the traditional risk factors for atherosclerosis and parameters of lung function. Eighty-six patients affected by SSc (13 men and 73 women, mean age 58.5 years, mean disease duration 10.7 years, 31 with diffuse form and 55 with limited pattern) underwent evaluation of body composition using a dual-energy X-ray (DXA) fan beam densitometer (GE Lunar iDXA) in order to assess total and regional body fat mass and fat-free mass. Clinical features, pulmonary function parameters, and the concomitant presence of the traditional cardiovascular risk factors were recorded. Android fat resulted to be higher in SSc patients with coexistence of hypercholesterolemia (P = 0.021), hypertension (P = 0.028), and overweight/obesity (P < 0.001) and positively correlated with body mass index (P < 0.001). Forced vital capacity (FVC) was inversely correlated with android fat (P = 0.034) and with the android fat/gynoid fat ratio (P = 0.013) and positively correlated with android lean (P = 0.041); the correlations were improved when FVC data were adjusted for sex, age, disease duration, and smoking habits (P = 0.010 for android fat, P = 0.010 for android fat/gynoid fat ratio, P = 0.011 for android lean). In this study, we showed that visceral abdominal fat, measured by DXA, is correlated with the main cardiovascular risk factors and lung volumes in SSc patients. Longitudinal studies are needed to evaluate if decrease of abdominal fat would improve lung function.

The co-occurrence of Hashimoto thyroiditis in primary Sjogren's syndrome defines a subset of patients with milder clinical phenotype.

To evaluate in a cohort of 100 consecutive patients affected by primary Sjogren's syndrome (pSS) the incidence of Hashimoto thyroiditis (HT) and to compare the clinical features and the laboratory parameters of patients affected by pSS with and without concomitant HT. In 100 consecutive patients affected by pSS, the occurrence of other autoimmune diseases was recorded and a full examination of thyroid function obtained. HT was associated with pSS in 27 cases. The comparison between pSS cases with and without HT showed that only patients with isolated pSS had low C4 level [p = 0.032, OR (IC 95 %) 230 (13.13-4,046)]. In addition, only patients affected by pSS without HT had evidence of cryoglobulins, cutaneous vasculitis with palpable purpura, peripheral neuropathy, and development of lymphoma, although all these manifestations were observed in a 4.1-8.2 % of the cases, without reaching statistical significance. The association of HT in patients suffering from pSS defines a subset of patients with milder disease and normal C4 levels.

A study on thrombophilic factors in Italian Behcet's patients.

BACKGROUND: Behcet's disease (BD) may complicate with arterial and venous thrombosis. The purpose of this work is to evaluate in an Italian group of BD patients with thrombotic events a large panel of inherited and acquired thrombophilic factors. METHODS: Thirty BD patients, of which nine with previously arterial or venous thrombosis and 21 without, underwent the following investigations: plasma antithrombin activity, protein C activity, free protein S level, sensitivity to APC, total plasma homocysteine concentration, serum folate level, determination of anti-phospholipid antibodies, serum Lp(a) levels, tests for gene polymorphisms of factor V Leiden, prothrombin and methylenetetrahydrofolate reductase genes. Tests for the gene polymorphisms were also performed in a group of healthy control subjects. RESULTS: All the six patients with arterial or deep venous thrombosis showed thrombophilic conditions such as protein C or protein S deficiency (one case each), hyperhomocysteinemia (two cases), positivity of anti-phospholipid antibodies associated with APC resistance or hyperhomocysteinemia (one case each). Among three subjects with superficial thrombophlebitis only one showed a mild hyperhomocysteinemia. No differences were found between BD patients and control subjects concerning polymorphisms of the genes considered. Among BD patients the Factor V H1299R mutation showed a weak association with venous thrombosis (P=0.048). CONCLUSION: In BD patients different concomitant significant thrombophilic risk factors may contribute to the development of thrombotic events. Patients affected by vasculo-Behcet should be evaluated for the presence of coexisting major thrombophilic conditions.

Anti-TNFalpha blockers, autoantibodies and autoimmune diseases.

Anti-TNFalpha blockers are extensively used in the management of chronic inflammatory disorders. Their administration may be associated with the generation of autoantibodies; this review focuses on the autoimmune phenomena linked to anti-TNFalpha inhibition, on the hypothesized pathogenetic mechanisms and on the clinical implications. While the development of antinuclear and, less frequently, of anti-DNA antibodies is a common finding, the onset of autoimmune diseases during anti-TNFalpha blocker treatment is a rare event, which needs to be promptly recognized in order to plan the appropriate management. Moreover the specific autoantibodies associated with rheumatoid arthritis are considered before and after biotherapy. Similarities and differences among infliximab, etanercept and adalimumab concerning induced autoimmune phenomena are underlined.

A score of risk factors associated with ischemic digital ulcers in patients affected by systemic sclerosis treated with iloprost.

A single series of patients affected by systemic sclerosis (SSc) and cyclically treated with iloprost was reviewed in order to evaluate the incidence of digital ulcers (DUs) and to compare the characteristics between the patients with and without this painful and disabling vascular complication. The record charts of 85 SSc patients were revised. Ischemic DUs and scleroderma contracture ulcers were separately considered. Twenty-nine subjects developed ischemic DUs during the course of the disease; whereas, scleroderma contracture ulcers occurred in six subjects. Ischemic DUs were associated with younger age at scleroderma onset, a longer disease duration, a longer time delay from scleroderma diagnosis to iloprost therapy, a bigger skin involvement, the presence of joint contractures, a videocapillaroscopic late pattern, a history of smoking, and of corticosteroids therapy. After the exclusion of four subjects with concomitant peripheral arterial disease, a forward-stepwise logistic regression analysis showed that only four variables, i.e., age at scleroderma onset, delay in beginning iloprost therapy, history of smoking, and presence of joint contractures remained significantly associated with ischemic DUs. In a score reflecting the sum of these four risk factors, the prevalence of ischemic DUs increased progressively from the lowest to the highest value of the score. The predictivity of this model was evaluated by the receiver-operating characteristics curve, with an estimated area under the curve of 0.836 with 95% confidence interval from 0.736 to 0.937. All the patients with scleroderma contracture ulcers were characterized by both diffuse pattern of disease and positivity for anti-Scl70 antibody. In this retrospective study, scleroderma patients with ischemic DUs are characterized by early disease onset, delay in beginning iloprost therapy, smoking habit, and presence of joint contraction. A score reflecting the sum of these factors may be useful to predict the risk of developing ischemic DUs.

Clinical associations of anti-CENP-B and anti-Scl70 antibody levels measured by multiplexed fluorescent microsphere immunoassay in systemic sclerosis.

The aim of this study was to assess the association between anti-CENP-B and anti-Scl70 antibody levels, measured by multiplexed fluorescent microsphere immunoassay, and the clinical features in patients affected by systemic sclerosis. Clinical evaluation of 80 scleroderma patients was performed in order to evaluate disease activity and organ involvement. Scleroderma-specific autoantibodies were detected using multiplexed fluorescent microsphere immunoassay. Unexpectedly, 11 patients resulted positive for both anti-Scl70 and anti-CENP-B antibodies; six cases showed a weak positivity for one of the two autoantibodies and a stronger positivity for the other one; five cases showed an intense positivity for both autoantibodies. This latter subgroup was excluded from the analysis of the associations between autoantibody levels and the clinical features. In the anti-CENP-B positive patients higher antibody levels were associated with a less extensive skin involvement in comparison with the cases affected by a more extensive skin involvement (521 +/- 208 vs 395 +/- 166 U/ml, respectively, P 0.038). In the anti-Scl70 positive patients autoantibody levels were directly correlated with skin involvement (P 0.018), showing higher levels in patients with a more extensive skin involvement in comparison with cases characterized by less extensive skin involvement (734 +/- 135 vs 490 +/- 183 U/ml, respectively, P 0.001). The findings of our study supports the association between autoantibody profile and disease severity in systemic sclerosis. In particular high levels of anti-Scl70 antibodies are associated with a worse cutaneous involvement, while high levels of anti-CENP-B antibodies seem to have a protective effect on skin manifestations.