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• Objective clinical, histological and US manifestations of sicca syndrome are highly frequent in SSc patients. • Sjogren-SSc patients (overlap) had more severe glandular involvement than isolated Sjogren and isolated Sicca-SSc patients. • Specific SSc US patterns of salivary gland involvement are still to be defined. This study aimed to characterize the whole phenotype of Systemic sclerosis (SSc) patients with sicca symptoms, using major salivary glands Ultrasound (SGUS) parameters, minor salivary glands biopsies (mSGB) and clinical findings, and to compare these characteristics with those from patients with Sjogren's Disease (SjD), and patients with sicca manifestations from other causes. Sixty SSc patients fulfilling the 2013 ACR/EULAR classification criteria and with subjective self-declared sicca symptoms were consecutively recruited and had SGUS and mSGB. Fifteen SSc patients without subjective sicca symptoms and 65 patients with sicca symptoms from other causes (including 37 SjD with no SSc). SSc patients with subjective sicca symptoms had frequent objective clinical (up to 83 %), histological (44 % of Focus score≥1/ mm2) and US anomalies (63 % of OMERACT ≥2). 53 % patients without subjective clinical complaint also had abnormal objective tests, suggesting the existence of a sub clinical involvement of salivary glands in SSc. SjD-SSc patients had more severe glandular involvement as compared to patients with isolated SjD and isolated Sicca-SSc patients (70%, 48,6 % and 38% of patients with OMERACT ≥2 respectively) suggesting additive impact of both diseases on glandular physiology and structure. SjD-SSc overlap have more severe sicca features as compared to isolated sicca-SSc and isolated SjD, suggesting a specific impact of SSc on salivary gland physiology. Further translational studies are needed to identify the underlying pathways that could serve as therapeutic targets. [ABSTRACT FROM AUTHOR]

Thoracic lymphadenopathy (LA) has been identified as a key prognostic factor in interstitial lung disease (ILD) of all-cause. Crystalline silica is a risk factor of systemic sclerosis (SSc). The association of a history of crystalline silica exposure with chest high-resolution computed tomography (HRCT) features and thoracic LA are still to be determined in SSc patients. We performed an observational study to assess the association of lifetime exposure to silica, with chest HRCT characteristics in a population of SSc patients fulfilling the 2013 ACR/EULAR classification criteria for SSc. A specific questionnaire based on a multidisciplinary approach was used to assess occupational and non-occupational exposure to silica in 100 consecutive SSc patients. Clinical characteristics and chest HRCT at diagnosis and at the latest visit were evaluated to assess the association of silica exposure with disease characteristics. 16% of the overall population and 58% of men had an occupation with specific high silica exposure. A higher silica exposure score was associated with the combination of mediastinal and hilar LA on HRCT (OR=8.09, 95%CI=2.01–32.52, P = 0.002). More than 12% of the patients had a combination of mediastinal and hilar LA on HRCT. This marker of silica exposure was predictive of worsening of pulmonary involvement in univariate analysis (OR=5.86, 95%CI=1.64–20.89, P = 0.007) and multivariate analysis (OR=4.57, 95%CI =1.12–18.60, P = 0.034). In patients with SSc, the combination of mediastinal and hilar LA on HRCT was associated with exposure to silica and was also significantly associated with a more severe evolution of ILD. [ABSTRACT FROM AUTHOR]

Background As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature. Methods 46 patients from 19 French centers were retrospectively recruited. They were included if they had a diagnosis of SSc and received at least 1 IVIG infusion at a dosage > 1 g/kg/cycle. Relevant data collected at IVIG discontinuation were compared to those collected at IVIG initiation. A comprehensive literature review was performed. Results We observed a significant improvement of muscle pain (74% vs. 20%, p < 0.0001), muscle weakness (45% vs. 21%, p = 0.01), joint pain (44% vs. 19%, p = 0.02), CK levels (1069 ± 1552 UI vs. 288 ± 449 UI, p < 0.0001) and CRP levels (13.1 ± 17.6 mg/L vs. 9.2 ± 16.6 mg/L, p = 0.001). We also noted a trend for an improvement of gastro-esophageal reflux disease (68% vs. 53%, p = 0.06) and bowel symptoms (42% vs. 27%, p = 0.06). Skin and cardiorespiratory involvements remained stable. Finally, corticosteroid daily dose was significantly lower by the end of treatment (13.0 ± 11.6 mg/day vs. 8.9 ± 10.4 mg/day, p = 0.01). Only two severe adverse events were reported (one case of deep vein thrombosis and one case of diffuse edematous syndrome). Conclusion Our work suggests that IVIG are a safe therapeutic option that may be effective in improving musculoskeletal involvement, systemic inflammation, digestive tract symptoms and could be corticosteroid sparing. [ABSTRACT FROM AUTHOR]

Abstract: Systemic manifestations of monoclonal gammopathies (MG) are rare but extremely varied. This general review focuses on the hyperviscosity syndrome, neurological disorders, skin changes, the POEMS syndrome, and biological manifestations, with the exception of amyloidosis AL and cryoglobulinemia. The hyperviscosity syndrome usually involves a combination of general, hemorrhagic, ocular and central neurological disorders. The principal neurological manifestations are peripheral neuropathies, mainly due to IgM with anti-MAG activity. Skin disorders include overload dermatoses (xanthomatosis, mucinosis), neutrophilic dermatosis, urticaria, edema and the AESOP syndrome. The POEMS syndrome classically consists of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasmocyte proliferation, and cutaneous manifestations. MG interference with assay methods can lead to false hyponatremia, hypoglycemia, hyperbilirubinemia, hypercalcemia and hypertransferrinemia. These systemic manifestations can reveal classical MG-related disorders such as monoclonal gammopathy of undetermined significance (MGUS), solitary plasmocytoma, multiple myeloma, and Waldenstrom''s disease. They are due either to the chemicophysical properties of the monoclonal immunoglobulin, or to its antibody activity (especially against autoantigens), with potential therapeutic implications. [Copyright &y& Elsevier]