Lung Involvement In Systemic Sclerosis Research Articles

The relationship between peripheral T follicular helper cells and disease severity in systemic sclerosis

We aimed to investigate the association between follicular T helper cells (Tfh) and disease severity in systemic sclerosis (SSc), a chronic connective tissue disease characterized by progressive fibrosis. While Tfh cells have been extensively studied in other autoimmune diseases, their role in SSc remains poorly understood. A cohort of 50 SSc patients, diagnosed based on the ACR/EULAR 2013 classification criteria, was included in the study. Patient data, including demographic information, comorbidities, treatment history and organ involvement, were collected. Disease severity was assessed using the modified Rodnan skin score and Medsger disease severity index. Statistical analyses were performed, considering a p value of < 0.05 as statistically significant. 38% had SSc with limited skin involvement, while 62% had SSc with extensive skin involvement. However, there were no statistically significant differences observed in the levels of CD4+ CXCR5+ , CD4+ ICOS+ , CD4+ CD40L+ and CD4+ PD+ lymphocytes between the two groups. Notably, SSc patients with Raynaud’s phenomenon, digital ulcer and lung involvement exhibited higher levels of CD4+ CXCR5+ lymphocytes compared to those without these manifestations. Furthermore, a significant positive correlation was observed between CD4+ CXCR5+ lymphocyte levels and the severity of lung disease according to the Medsger disease severity index. Based on these findings, we conclude that elevated levels of Tfh cells are associated with lung involvement in SSc and there is a significant correlation between Tfh cell levels and the severity of lung disease. These observations suggest a potential role for Tfh cells in the pathogenesis of lung involvement in SSc and may guide the development of targeted therapies for this aspect of the disease.

P147 Predictors of interstitial lung involvement in systemic sclerosis

Abstract Background/Aims Interstitial lung disease (ILD) and pulmonary hypertension are the leading cause of death in patients with systemic sclerosis (SSc). Identifying SSc-ILD development and initiating treatment is essential to optimize therapeutic benefit. We aimed to identify predictors of SSc-ILD and compared early (&amp;lt;5 years from diagnosis) versus late (&amp;gt;5 years from diagnosis) onset. Methods We conducted a retrospective cohort study by including patients diagnosed with SSc from 1980 to 2020 followed in our unit and compared the clinical profile of patients with SSc-ILD to control SSc-non-ILD patients. Demographic features, clinical and immunological characteristics and baseline pulmonary function were retrieved. Logistic regression modelling was run to identify factors associated with SSc-ILD development. Factors associated with ILD were determined as factors associated with early or late onset. Bonferroni correction was used to limit Type I errors. Results We have included 103 patients from our patient registry from 1980 to 2021 (42% with SSc-ILD). Logistic regression identified risk factors associated with increased or decreased odds ratio for developing ILD is summarized in table 1: smoking history, male sex, the presence of myositis, anti-Scl70 and anti-Ro52 positivity, baseline pulmonary function including FVC and DLCO, mMRC (modified medical research council) dyspnea scale&amp;gt;2, mMSS (modified rodnan skin score)&amp;gt;3 and late pattern in capillaroscopy. Older age at SSc diagnosis, the presence of telangiectasias and smoking status was associated with increased odds of SSc-ILD onset before 5 years, while male gender, the presence of myositis and antiphospholid antibodies were associated with late-onset SSc-ILD. Conclusion We identified 11 factors significantly associated with risk of developing SSc-ILD. We identified early and late-onset predictors for ILD-SSc. Disclosure C. Sieiro Santos: None. R. Rego Salgueiro: None. C. Moriano Morales: None. C. Álvarez Castro: None. E. Díez Álvarez: None.

A Machine Learning Application to Predict Early Lung Involvement in Scleroderma: A Feasibility Evaluation.

Introduction: Systemic sclerosis (SSc) is a systemic immune-mediated disease, featuring fibrosis of the skin and organs, and has the greatest mortality among rheumatic diseases. The nervous system involvement has recently been demonstrated, although actual lung involvement is considered the leading cause of death in SSc and, therefore, should be diagnosed early. Pulmonary function tests are not sensitive enough to be used for screening purposes, thus they should be flanked by other clinical examinations; however, this would lead to a risk of overtesting, with considerable costs for the health system and an unnecessary burden for the patients. To this extent, Machine Learning (ML) algorithms could represent a useful add-on to the current clinical practice for diagnostic purposes and could help retrieve the most useful exams to be carried out for diagnostic purposes. Method: Here, we retrospectively collected high resolution computed tomography, pulmonary function tests, esophageal pH impedance tests, esophageal manometry and reflux disease questionnaires of 38 patients with SSc, applying, with R, different supervised ML algorithms, including lasso, ridge, elastic net, classification and regression trees (CART) and random forest to estimate the most important predictors for pulmonary involvement from such data. Results: In terms of performance, the random forest algorithm outperformed the other classifiers, with an estimated root-mean-square error (RMSE) of 0.810. However, this algorithm was seen to be computationally intensive, leaving room for the usefulness of other classifiers when a shorter response time is needed. Conclusions: Despite the notably small sample size, that could have prevented obtaining fully reliable data, the powerful tools available for ML can be useful for predicting early lung involvement in SSc patients. The use of predictors coming from spirometry and pH impedentiometry together might perform optimally for predicting early lung involvement in SSc.

Lung involvement in systemic sclerosis is associated with adverse hospital outcomes: insights from the National Inpatient Sample

This study aimed to compare outcomes of systemic sclerosis (SSc) hospitalizations with and without lung involvement. The primary outcome was inpatient mortality while secondary outcomes were hospital length of stay...

The Treatment of Lung Involvement in Systemic Sclerosis.

Systemic sclerosis (SSc) patients are often affected by interstitial lung disease (ILD) and, although there have been recent treatment advances, it remains the leading cause of death among SSc, with a 10-year mortality up to 40%. African Americans and subjects with diffuse cutaneous SSc or anti-topoisomerase 1 antibodies are most commonly affected. Currently, early ILD diagnosis can be made, and it is pivotal to improve the prognosis. The diagnostic mainstay test for SSc-ILD is high-resolution computed tomography for the morphology and pulmonary function tests for the functional aspects. Treatment planning and intensity are guided by the disease severity and risk of progression. Traditionally, therapy has depended on combinations of immunosuppressants, particularly cyclophosphamide and mycophenolate mofetil, which can be supplemented by targeted biological and antifibrotic therapies. Benefits have been observed in trials on hematopoietic autologous stem cell transplantation for patients with progressive SSc, whilst lung transplantation is reserved for refractory SSc-ILD cases. Herein, recent advances in SSc-ILD treatment will be explored.

Predictive factors for treatment-related mortality and major adverse events after autologous haematopoietic stem cell transplantation for systemic sclerosis: results of a long-term follow-up multicentre study

BackgroundAutologous haematopoietic stem cell transplantation (HSCT) improves survival in systemic sclerosis (SSc) with poor prognosis, but is hampered by treatment-related mortality (TRM).ObjectiveTo evaluate event-free survival (EFS), TRM, response to treatment,...

Increased frequency of obstructive sleep apnea in the patients with systemic sclerosis

Sleep problems, including obstructive sleep apnea (OSA), profoundly affect quality of life in patients with systemic sclerosis (SSc). This study aimed to determine the prevalence of OSA in patients with SSc and the factors affecting OSA. Consecutive patients with SSc lung involvement who were referred to the "Outpatient Service for Interstitial Lung Disease" in our university hospital between 2015 and 2017 were included in the study. All patients completed the Epworth Sleepiness Scale (ESS) and underwent examination including body mass index (BMI), measurement of waist circumference, upper respiratory tract examination, and polysomnography (PSG). Spirometry, carbon monoxide diffusion test (DLCO), and 6-min walking distance were also performed. Of 38 patients, mean age 51.3 ± 11.6 years, 35 were women (92%). Mean apnea-hypopnea index (AHI) was 11 ± 15 (median 5.5) and prevalence of OSA was 58%. Mild OSA was found in 13 (34%) of patients, moderate OSA in 6 (16%), and severe OSA in 3 (8%). Significant relationships were found between age (p = 0.02), waist circumference (p = 0.01), presence of witnessed apneas (p = 0.005), and presence of OSA. Compared with the general population, the prevalence of OSA is increased in women with SSc. Patients with older age, those with increased waist circumference, and those reporting witnessed apneas should be studied for OSA.

Imatinib-loaded gold nanoparticles inhibit proliferation of fibroblasts and macrophages from systemic sclerosis patients and ameliorate experimental bleomycin-induced lung fibrosis

Interstitial lung involvement in Systemic Sclerosis (SSc-ILD) is a complication with high morbidity and mortality. Specifically, engineered gold nanoparticles (GNPs) are proposed as targeted delivery system increasing efficacy of drugs with antifibrotic effect, such as tyrosine kinases.We aimed to test in vitro and in vivo the activity of targeted Imatinib (Im)-loaded GNP on SSc-ILD patients derived cells and in experimental model of lung fibrosis.GNPs functionalized with anti-CD44 and loaded with Im (GNP-HCIm) were synthesized. Lung fibroblasts (LFs) and alveolar macrophages from bronchoalveolar lavage fluids of SSc-ILD patients were cultured in presence of nanoparticles. GNP-HCIm significantly inhibited proliferation and viability inducing apoptosis of LFs and effectively reduced IL-8 release, viability and M2 polarization in alveolar macrophages. Anti-fibrotic effect of tracheal instilled GNP-HCIm was evaluated on bleomycin lung fibrosis mouse model comparing effect with common route of Im administration. GNP-HCIm were able to reduce significantly lung fibrotic changes and collagen deposition. Finally, electron microscopy revealed the presence of GNPs inside alveolar macrophages.These data support the use of GNPs locally administered in the development of new therapeutic approaches to SSc-ILD.

The role of nailfold capillaroscopy in monitoring lung involvement in systemic sclerosis

The role of nailfold capillaroscopy in monitoring lung involvement in systemic sclerosis

Association of Serum Homocysteine Level and Interstitial Lung Disease in Systemic Sclerosis: A Case-control Study.

Systemic sclerosis is a disorder of connective tissue with unknown cause, affecting the skin and internal organs, characterized by fibrotic changes. To determine the correlation between serum homocysteine level and interstitial lung involvement in systemic sclerosis. In this case - control study, 59 patients who fulfilled the ACR/EULAR classification criteria for systemic sclerosis and were referred to Hafez Hospital of Shiraz, Iran, were included as the case group. Fifty nine healthy subjects were involved as the control group. Patients were divided into two groups based on interstitial lung involvement and two subtypes, diffuse and limited type. Serum homocysteine, vitamin B12, and folate levels compared between the controls, and cases groups. Of 59 case and control group, 53 (%89.8) were female and the mean age did not differ in both groups (P=0.929). Thirty five (%59.3) patients had interstitial lung involvement and 38(%64.4) had diffuse cutaneous systemic sclerosis. The mean serum homocysteine level was 13.9±6.3 µmol/L in the case and 13.7±9.2 µmol/L in the control group (P=0.86). The mean serum homocysteine level did not differ between the patients with and without interstitial lung involvement (P=0.52). The patients with lung involvement was older than those without lung involvement (P=0.004). Lung disease was more common in diffuse type (P=0.014). In our study, serum homocysteine level did not differ between the patients and healthy subjects. Also, there was no correlation between serum homocysteine level and lung involvement, but lung involvement was more common in older patients and also diffuse subtype.

Quantitative videocapillaroscopy correlates with functional respiratory parameters: a clue for vasculopathy as a pathogenic mechanism for lung injury in systemic sclerosis

BackgroundTo determine whether lung involvement is related to microvascular perturbations, nailfold videocapillaroscopy (NVC) was performed in patients with systemic sclerosis (SSc).MethodsA cross-sectional study was consecutively accomplished in 152 SSc patients. NVC, a pulmonary function test and echocardiography were undergone within a 3-month period. Finally, 134 patients with at least eight NVC (200× magnification) images were selected for quantitative and qualitative examinations.ResultsPatients with interstitial lung disease presented lower median capillary density (4.86/mm vs 5.88/mm, p = 0.005) and higher median of neoangiogenesis (0.56/mm vs 0.31/mm, p = 0.005). A higher quantity of neoangiogenesis capillaries was found in patients with pulmonary arterial hypertension (0.70/mm vs 0.33/mm, p = 0.008). Multivariate linear regression analysis established a correlation between neoangiogenesis and decreased forced vital capacity (FVC) (p < 0.001): for each capillary with neoangiogenesis visualized on average per 1 mm, FVC was 7.3% reduced. In qualitative NVC, a late pattern as defined by Cutolo was also associated with lower FVC (p = 0.018). The number of giant capillaries was associated with reduced diffusion capacity of the lung for carbon monoxide (DLCO) (p = 0.016); for each giant capillary per 1 mm, DLCO was 11.8% diminished.ConclusionsA good correlation was observed between distinctive quantitative and qualitative NVC features with lung functional parameters such as FVC and DLCO. It is suggested that vasculopathy could play a role in SSc lung involvement.

Muscle endurance, strength, and active range of motion in patients with different subphenotypes in systemic sclerosis: a cross-sectional cohort study

Objective: Proximal muscle weakness is common in patients with systemic sclerosis (SSc). Dynamic muscle endurance, muscle strength in the lower extremities, and active range of motion (AROM) in the upper extremities are less studied. We investigated functional muscle endurance, strength, and AROM, and explored differences depending on skin and/or lung involvement in SSc patients.Method: The study divided 205 patients with limited/diffuse cutaneous systemic sclerosis (lcSSc/dcSSc) into no–mild and moderate–end-stage lung involvement, the latter based on the Medsger disease severity score. Dynamic muscle endurance in shoulder and hip flexion was assessed by the Functional Index-2, lower extremity muscle strength by the Timed-Stands Test (TST), and shoulder–arm AROM by the Functional Shoulder Assessment (FSA).Results: Shoulder and hip flexion muscle endurance were reduced in relation to reference values median (IQR) [53% (27–100%) and 40% (23–90%), respectively, p < 0.001]. Patients with moderate–end-stage lung involvement had less endurance in shoulder [39% (21–71%) and hip flexion 35% (20–70%)] than patients with no-mild lung involvement [57% (33–99%) and 48% (28–100%), p < 0.05]. All patients, regardless of subtype/grouping, needed longer to complete the TST [21 s (17–27 s)] compared to reference values [17 s (15–18 s), p < 0.001], and patients with moderate-end-stage lung involvement had worse TST score than patients with no-mild lung involvement, [25 s (18–30 s) vs 19 s (16–25 s), p < 0.001]. The FSA sum scores were lower compared with reference values (p < 0.01). DcSSc patients had a lower FSA-sum score [53 (48–57)] than lcSSc patients [57 (52–60), p < 0.01].Conclusion: SSc patients have markedly reduced muscle endurance in the upper and lower extremities, reduced muscle strength in the lower extremities, and impaired AROM in the shoulders and arms. Patients with moderate–end-stage lung involvement had more impaired muscle endurance and strength but no differences were found between lcSSc and dcSSc patients. Not only muscle strength, but also dynamic muscle endurance should be measured in SSc patients.

Serum alarm antiproteases in systemic sclerosis patients

Alarm antiproteases, i.e. secretory leukocyte protease inhibitor ad elafin, are key mediators in innate immune response and integrate innate and adaptive immunity systems. The aim of the study was to assess clinical significance of serum levels of alarm antiproteases, elafin and secretory leukocyte protease inhibitor (SLPI) in patients with systemic sclerosis (SSc). Twenty-eight patients with SSc, 25 patients with rheumatoid arthritis (RA) and 22 healthy controls were recruited. Serum elafin and SLPI levels were examined using enzyme-linked immunosorbent assay (ELISA). The patients with SSc had significantly increased serum levels of SLPI in comparison with the RA patients and the healthy controls (p<0.01), and the RA patients presented significantly higher serum levels of elafin in comparison with the controls (p=0.003). In the SSc subgroup serum SLPI level negatively correlated with diffusing capacity of the lung for carbon monoxide (DLCO) (r=−0.41, p=0.03) and total lung capacity (r=−0.42, p=0.03). Both alarm antiproteases, elafin and SLPI could be potentially implicated in the pathogenesis of SSc and SLPI may be considered a candidate for serum biomarker of lung involvement in SSc.

The Role of EDD (Exercise Capacity, Dyspnea, and Diffusing Capacity of Lungs for Carbon Monoxide) Index in Pulmonary Fibrosis Secondary to Scleroderma

Introduction: Pulmonary involvement secondary to Systemic Sclerosis (SSc) is the major cause of morbidity and mortality in SSc patients. We designed this study to determine the correlation of important lung function parameters with lung High Resolution CT (HRCT) scan findings. Materials and Methods: Thirty–two consecutive diffuse SSc patients with pulmonary fibrosis were enrolled in this cross-sectional study. Patients with pulmonary fibrosis secondary to other causes, previous restrictive lung disease, and history of smoking were excluded. Complete lung function evaluation was performed. The EDD (Exercise capacity, Dyspnea, and Diffusing capacity of lungs for carbon monoxide) index was determined. The Warrick score was calculated based on lung HRCT findings. Results: The mean age of the patients was 39.18 years ±9.39 (SD).Seventeen (53%) patients were in EDD stage 1 (score: 0≤score≤3), 9 patients (28%) in stage 2 (3<score ≤6), and 6 patients (19%) in stage 3(6<score≤9). The mean Warrick score was 10.84±6.94 (SD).There was statistically significant correlation between EDD index and Warrick score (r=0.72, P=0.001).Also there was statistically significant strong correlation between EDD stages and Warrick scores (r=0.8, P=0.002). Conclusion: The results of this study revealed that EDD may be a valuable representative marker of lung involvement in SSc and in the future, it can be a suitable and safe alternate modality comparing lung HRCT in our clinical practice and close follow-up.

Th-17 cytokines and interstitial lung involvement in systemic sclerosis

The two phenotypes of both limited and diffuse systemic sclerosis (SSc) have different forms of pulmonary involvement: pulmonary arterial hypertension (limited phenotype) or interstitial lung disease (ILD) (diffuse phenotype). We aimed to investigate whether Th17-related cytokines, as measured in exhaled breath condensate (EBC) and in serum were connected to ILD in diffuse SSc patients. We found that for both limited and diffuse SSc, the EBC levels of all cytokines and most of the cytokine serum levels were significantly higher in patients than in controls, while, the EBC levels of Th-17 cytokines and the serum levels of IL-10 and TNF-α were significantly higher in diffuse than in limited SSc. Moreover, the thoracic CT-scan score of ILD was significantly associated with the EBC levels of IL-1 beta and with the serum IL-23, TNF-α and IL-10 levels, whereas lung carbon monoxide diffusing capacity was negatively related to the EBC levels of IL-1 beta, IL-17 and serum IL-10. Serum IL-23 was also inversely correlated with vital capacity. In conclusion, in diffuse SSc patients our results show a clear link between Th-17 cytokines measured both in EBC and in serum with interstitial lung involvement. This highlights how important it is to target Th-17 cytokines when developing new treatments for lung fibrosis.

Cross-sectional study of soluble selectins, fractions of circulating microparticles and their relationship to lung and skin involvement in systemic sclerosis.

BackgroundEndothelial damage and activation may play central roles in the pathogenesis of systemic sclerosis (SSc) and are reflected by microparticles (MPs) and soluble selectins. The objective of this study was to determine if these potential biomarkers are associated with specific organ involvements or cutaneous subgroups of SSc patients.MethodMPs in platelet-poor plasma from 121 patients with SSc, 79 and 42 with limited and diffuse cutaneous disease, respectively, were characterized by flow cytometry for their capacity to bind annexin V in combination with surface markers of either platelets (PMPs), leukocytes (LMPs) or endothelial cells (EMPs). Soluble E- and P-selectin levels were determined in plasma. By correlation analyses, this was held against involvement of skin, lung function, lung fibrosis, pulmonary artery hypertension, and serology.ResultsNone of the markers were associated with cutaneous subgroups of SSc. Concentrations of annexin V non-binding EMPs and annexin V non-binding LMPs were negatively correlated to pulmonary diffusing capacity (DLCO) (r = -0.28; p = 0.003; r = -0.26; p = 0.005) and forced vital capacity (FVC) (r = -0.24; p = 0.009; r = -0.29; p = 0.002), driven by patients with limited and diffuse cutaneous disease, respectively. Soluble E-selectin levels correlated negatively to DLCO (r = -0.21, p = 0.03) and FVC (r = -0.25; p = 0.007); and soluble P-selectin correlated negatively to DLCO (r = -0.23, p = 0.01).ConclusionNegative correlations between annexin V non-binding EMP and LMP concentrations with lung function parameters (DLCO and FVC) differed between limited and diffuse cutaneous subsets of SSc, indicative of various pathogeneses of lung involvement in SSc, possibly with a differential role of MPs.

Physical capacity in performing daily activities is reduced in scleroderma patients with early lung involvement.

Patients with systemic sclerosis (SSc) often complain reduced capacity at submaximal exercise; conversely, physical capacity in performing daily duties has never been measured in SSc. The aim of this study is to evaluate this performance and its correlates, in patients with SSc compared with healthy controls, in a free-living setting. Twenty-seven outpatients with stable SSc and 11 controls were recruited. Physical activity was assessed by portable multiple sensor device (SenseWear Armband) worn for at least 6 days. Physical activity duration (PAD; in minutes) for non-sedentary activities and physical activity level (PAL = total daily energy/resting energy expenditure) per day were calculated. Nutritional status was estimated by bioelectrical impedance analysis and pulmonary arterial hypertension excluded by echocardiography. Daily physical activities (243 ± 145 min per day vs 397 ± 142 min, respectively; P = 0.005) and PAL were significantly reduced in SSc compared with controls (1.5 ± 0.4 vs 2 ± 0.7, respectively; P = 0.019). Seventy-four per cent of SSc patients showed PAL < 1.70, whereas only 27% of controls were below this threshold for sedentary life style. Both PAD and PAL positively correlated with DLco. Patients and controls did not differ for spirometric parameters, body mass index, phase angle at bioelectrical impedance analysis, fat mass or fat-free mass indexes. In SSc, exercise capacity during daily activity was reduced compared with controls, and was associated with early evidence of functional decay (decreasing DLco) but not with malnutrition (undernutrition). A reduction of daily physical activity is already present even in early stages of lung involvement in SSc, characterized by unaltered spirometry and well-preserved nutritional status.

Early stage lung cancer detection in systemic sclerosis does not portend survival benefit: a cross sectional study.

BackgroundSystemic Sclerosis (SSc) is a rare connective tissue disorder associated with an increased risk of malignancy including lung cancer.MethodsA single center review of all cases of lung cancer in patients with SSc was conducted. Clinical, radiographic, and detailed pathologic data was collected. Risk factors were compared with our center’s SSc Registry. Cancer characteristics were compared with the National Cancer Institute SEER Cancer Statistics (NCI SEER) data.Results17 cases were identified; the majority were females (82%) with the lung cancers diagnosed after the onset of SSc (88%). Tobacco use was identified in 65% of cases. Serologic testing showed 50% of cases were Scl-70 positive. Twelve cases had radiographic evidence of SSc lung involvement, however only 6 had restrictive physiology on pulmonary function testing. Thirteen cases had pulmonary nodules preceding lung cancer. Thirteen of the cancers were adenocarcinoma. Ten underwent molecular mutational profiling: 2/8 had KRAS mutation and 1/10 had EGFR mutation. More of the non-small cell lung cancers were diagnosed at localized disease (56%) than in the NCI SEER database. However, 5 years survival among the stage I cases was 25% versus an expected survival of 54%.ConclusionsThe high proportion of adenocarcinomas seen in our study is different from that reported in the literature. Lung cancers were diagnosed at an early stage, likely due to our center’s practice of radiographic screening for SSc associated lung involvement, however this did not confer a survival advantage. A high proportion of patients who developed lung cancer had interstitial lung disease.

Management of Scleroderma-Associated Pulmonary Involvement

In the absence of a disease-modifying treatment for systemic sclerosis (SSc), the management of the main causes of morbidity and mortality is essential to offer the best possible patient care. Accordingly, early diagnosis and treatment of lung involvement plays a central role. Several new agents against pulmonary artery hypertension (PAH) have driven a notable progress in managing this complication in the recent years. Prostacyclin derivates, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, or guanylate cyclase stimulators are used today in single or combined therapy to improve the prognosis of PAH patients. Regarding interstitial lung disease (ILD) cyclophosphamide remains the most evidence-based therapy for SSc ILD. Small-scale interventional or observational studies support mycophenolate mofetil for either first-line or maintenance therapy after an initial course of cyclophosphamide. Observational studies also show promising results for biologic agents like Rituximab, and autologous stem cell transplant appears to be an option for severe and carefully selected cases. Moreover, nintedanib and pirfenidone have been shown to be effective in recent studies for idiopathic lung fibrosis. Randomized controlled trials are needed to test the effects of these potential therapies in SSc-ILD. The development of targeted therapies for lung involvement in SSc, together with the increasing knowledge regarding patient selection and management of the available drugs, will help to improve care of SSc patients with lung involvement.

AB0608 Elevated Circulating Tumor-Associated Antigens in Systemic Sclerosis: Association with Lung Fibrosis

BackgroundSome tumor-associated antigens (TAAs), apart from cancer cells, are expressed on the surface of inflammatory cells. The production of some TAAs may also be increased in some autoimmune diseases including...