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Systemic autoimmune diseases (SADs) are a significant burden on the healthcare system. Understanding the complexity of the peripheral immunophenotype in SADs may facilitate the differential diagnosis and identification of potential therapeutic targets. Single-cell mass cytometric immunophenotyping was performed on peripheral blood mononuclear cells (PBMCs) from healthy controls (HCs) and therapy-naive patients with rheumatoid arthritis (RA), progressive systemic sclerosis (SSc), and systemic lupus erythematosus (SLE). Immunophenotyping was performed on 15,387,165 CD45 live single cells from 52 participants (13 cases/group), using an antibody panel to detect 34 markers. Using the t-SNE (t-distributed stochastic neighbor embedding) algorithm, the following 17 main immune cell types were determined: CD4 /CD57 T cells, CD4 /CD57 T cells, CD8 /CD161 T cells, CD8 /CD161 /CD28 T cells, CD8 T cells, CD3 /CD4 /CD8 T cells, TCRγ/δ T cells, CD4 NKT cells, CD8 NKT cells, classic NK cells, CD56 /CD98 cells, B cells, plasmablasts, monocytes, CD11cdim/CD172dim cells, myeloid dendritic cells (mDCs), and plasmacytoid dendritic cells (pDCs). Seven of the 17 main cell types exhibited statistically significant frequencies in the investigated groups. The expression levels of the 34 markers in the main populations were compared between HCs and SADs. In summary, 59 scatter plots showed significant differences in the expression intensities between at least two groups. Next, each immune cell population was divided into subpopulations (metaclusters) using the FlowSOM (self-organizing map) algorithm. Finally, 121 metaclusters (MCs) of the 10 main immune cell populations were found to have significant differences to classify diseases. The single-cell T-cell heterogeneity represented 64MCs based on the expression of 34 markers, and the frequency of 23 MCs differed significantly between at least twoconditions. The CD3 non-T-cell compartment contained 57 MCs with 17 MCs differentiating at least two investigated groups. In summary, we are the first to demonstrate the complexity of the immunophenotype of 34 markers over 15 million single cells in HCs vs. therapy-naive patients with RA, SSc, and SLE. Disease specific population frequencies or expression patterns of peripheral immune cells provide a single-cell data resource to the scientific community.

An overlap syndrome is a medical condition which shares features of at least two more widely recognized disorders. Autoimmune connective tissue diseases include systemic lupus erythematosus (SLE), scleroderma, polymyositis, dermatomyositis, rheumatoid arthritis and Sjögren's syndrome where overlap syndrome most commonly seen in combination with SLE and systemic sclerosis (SSc). Sjogren's is an autoimmune exocrinopathy, in which systemic diseases such as arthritis, interstitial lung disease and renal disease may develop in addition to the pathognomonic features such as dry eyes and mouth. The other associated disease with Sjogren's includes sialadenitis. Sialadenitis of the parotid gland is one of the major disorders of salivary gland. This article presents a rare case report of a patient diagnosed with sialadenitis of the parotid gland and associated with progressive SSc/lupus overlap syndrome and secondary Sjogren's.An overlap syndrome is a medical condition which shares features of at least two more widely recognized disorders. Autoimmune connective tissue diseases include systemic lupus erythematosus (SLE), scleroderma, polymyositis, dermatomyositis, rheumatoid arthritis and Sjögren's syndrome where overlap syndrome most commonly seen in combination with SLE and systemic sclerosis (SSc). Sjogren's is an autoimmune exocrinopathy, in which systemic diseases such as arthritis, interstitial lung disease and renal disease may develop in addition to the pathognomonic features such as dry eyes and mouth. The other associated disease with Sjogren's includes sialadenitis. Sialadenitis of the parotid gland is one of the major disorders of salivary gland. This article presents a rare case report of a patient diagnosed with sialadenitis of the parotid gland and associated with progressive SSc/lupus overlap syndrome and secondary Sjogren's.

Progressive systemic sclerosis (PSS) is a chronic autoimmune illness. Clinical oral manifestations in Scleroderma are very frequent. To explore the oral manifestations, frequent and rare, to investigate whether there are differences between gender and the observed correlation of changes in relation to Antibodies Anti-Topoisomerase I. in the study were included 75 patients (65 females and 10 males), their mean age was 45.2±10, duration of illness was around 5.1±12 years diagnosed according to the ACR criteria and treated in the period 2010-2013. 98.7% of our patients were ANA positive, whereas 49.3% of them were Anti SCL-70 positive. Patients in 91% of cases had one or more oral manifestations of disease. The most frequent oral manifestations are: small mouth (n = 39), the lingua short frenulum (n = 21), Xerostomia (n = 24) and paradontopathia (n = 16), while more rare are: Telangiectasia (n = 14), decreased interincisal distance (n = 9), missing teeth (n = 9), absorption of dental alveoli (n = 5) and Neuralgia n. trigeminus (n = 3). Oral symptoms have been frequent in patients with Scleroderma, SCL -70 positive but not statistically significant difference. Oral changes have high frequency in patients with Scleroderma and these changes provide high discomfort of the mouth and lower quality of life. Oral health care to patients with Scleroderma is very important and it affects a lot in reducing the level of disease and increase the quality of life.

Systemic sclerosis is unusual in childhood. We describe three children who presented with diffuse hidebound skin associated with gastrointestinal and pulmonary abnormalities. Cardiac and renal dysfunctions, which are often encountered in these patients, were notably absent in our cases.

Systemic sclerosis is an autoimmune connective tissue disease affecting both skin and internal organs. Progressive disease with multiple organ involvement is considered to have a poor prognosis. Treatment possibilities are limited, but certain patients may benefit from autologous hematopoietic stem cell transplantation (auto-HSCT). We report a case of a 30-year-old woman with progressive diffuse systemic sclerosis treated with parenteral cyclophosphamide with unsatisfactory results. Due to progression of the disease and lack of alternative therapies auto-HSCT was performed. After instituting treatment with autologous hematopoietic stem cell transplantation no immunosuppressive therapy has been required during 5-year follow-up. Improvement in exertion tolerance, partial regression of skin lesions and stabilization of pulmonary and cardiovascular changes were observed. Currently therapeutic options in patients with progressive systemic sclerosis are limited. Hematopoietic stem cell transplantation might become an alternative therapeutic solution not only in the early phase of the disease but also among selected patients with progressive systemic sclerosis resistant to standard therapy.

Intra-thoracic manifestations of progressive systemic sclerosis (PSS) are not well known particularly the imaging features, which forms the basis of accurate and timely diagnosis. The aim of this study is to familiarize the physicians and radiologists with these features. The diagnosis can remain elusive because of the non-specific nature of symptoms which mimic many common conditions. Thus, the diagnosis of PSS can be missed leading to continuous morbidity if the correct imaging is not pursued. The authors examined the records of rheumatology patient referrals of over a 5 year period. A hundred and seventy patients with systemic sclerosis and mixed connective tissue disorders were chosen for detailed study of the imaging available, which form the basis of this review. The images included conventional chest radiographs, digital radiographs computed radiography (CT) and high resolution computed tomography (HRCT). Where applicable computed pulmonary angiography (CTPA) and radionuclide scans were also interrogated.

Vasculopathy, immunological abnormalities, and excessive tissue fibrosis are key elements in the pathogenesis of progressive systemic sclerosis (SSc). Extracorporeal shock waves (ESW) have anti-inflammatory and regenerative effects on different tissues. We hypothesized that ESW can reduce endothelial cell damage and skin fibrosis in patients with SSc. We enrolled 30 patients affected by SSc, 29 females and 1 male. Rodnan Skin Score (RSS) and Visuo-Analogical Scale (VAS) for skin wellness were performed before and immediately after ESW therapy (ESWT) and at 7, 30, 60, and 90 days after the treatment. Sonographic examination of the patients’ arms was performed before and 7, 30, 60, 90 days after treatment. Blood samples were obtained before and 30 and 60 days after treatment to measure serological levels of von Willebrand factor, vascular endothelial growth factor, intracellular adhesion molecule-1, monocyte chemotactic protein-1. The number of endothelial progenitor cells (EPCs) and circulating endothelial cells (CECs) were determined at the same time points. After ESWT we observed a rapid and persistent reduction of RSS and decrease of VAS. There was no difference in skin thickness before and after ESWT; however, we observed a more regular skin structure and an improvement in skin vascularization 90 days after treatment. EPCs and CECs increased 60 and 90 days after treatment, while serological biomarkers showed no variation before and after therapy. In conclusion, ESWT resulted in an improvement of VAS, RSS, and of skin vascular score, and in an increase of CECs and EPCs.

The purpose of this study was to assess the impact of enteric-coated mycophenolate sodium (EC-MPS) on skin and pulmonary manifestations of patients with progressive systemic sclerosis (Ssc). A prospective, open-label single-centre trial with EC-MPS 2 × 720mg/day over 12 months and a long-term follow-up of 50 months were conducted. Modified Rodnan skin score (mRSS) was used to assess the skin and pulmonary function tests to assess the pulmonary involvement. In order to quantify the extent of alveolitis/fibrosis via densitometry, the high attenuation value, median lung density and percentiles of lung tissue densities were obtained by high-resolution computed tomography. Eleven patients were included. Three patients had to stop medication before month 6 (2× side effects, 1× progression). For the remaining eight patients, the median mRSS was non-significantly reduced from 13.5 at baseline to 11 at month 12. According to the CT histography, median lung density and high attenuation values remained stable. However, the course of percentiles −200 to −300 and particularly −300 to −400 Hounsfield units slightly increased in seven of eight patients after 12 months, suggesting worsening of pulmonary involvement. Accordingly, median diffusing capacity for carbon monoxide showed a tendency to decline (75.1 % vs. 70.2) while forced vital capacity non-significantly improved (78.0 vs. 85.5 %) during the study. Four patients are still on EC-MPS without clinical signs of progression after 50 months follow-up. EC-MPS showed non-significant improvement of the skin. Pulmonary fibrosis remained stable with only a slight tendency towards progression which might be ascribed to the medication as well as the natural course of the disease. CT histography appears to be a sensitive method for the detection of progression of pulmonary fibrosis and therefore should be considered for further studies in Ssc.

Pneumothorax in patients with progressive systemic sclerosis (PSS) often presents as a difficult‑to‑treat disease. Autologous blood‑patch pleurodesis has previously been used for the treatment of pneumothorax. Blood outside its own environment is an irritant; therefore, chest physicians must watch closely for an allergic reaction. The injection is simple, painless, causes no side effects, is an inexpensive treatment for pneumothorax and is available not only in patients with persistent air leak but also in those with residual air space. A case is reported here of blood‑patch pleurodesis for pneumothorax in lung fibrosis due to PSS. As an alternative therapy for difficult‑to‑treat pneumothorax in patients with PSS with persistent air leak and residual air space, autologous blood‑patch pleurodesis would be one of the treatment options.

Keywords Progressive systemic sclerosis * Contemporary update * Scleroderma