Published reports have strong limitations and are highly heterogeneous, hindering the evidence to support the use of skin ultrasound in clinical practice. Further studies, with modern devices and appropriate methodology, are needed to establish the real value of skin ultrasound assessment in the early diagnosis and monitoring of SSc patients. This article is protected by copyright. All rights reserved.

Central nervous system (CNS) vasculitis (CNS) in systemic erythematosus lupus (SLE) is a rare and challenging diagnosis. We report four cases of CNS vasculitis that occurred 5 to 16 years after the diagnosis of SLE. Magnetic resonance imaging (MRI) detected different features suggestive of CNS vasculitis: enhancement and thickening of the vascular wall, vascular stenosis, ischemic brain lesions and intracerebral haemorrhage unlikely to correspond to other mimic aetiologies. Three patients received combination therapy with glucocorticoids (GC) and cyclophosphamide (CYC). Intravenous human immunoglobulin (IVIG) was administered when the patient had a past history of serious adverse event to CYC or high infectious risk. All patients showed imagiological improvement, at least partially, 5 to 23 days after starting treatment. We discuss the management of CNS in SLE including the role of magnetic resonance imaging (MRI).

Invalidation occurs in all rheumatic diseases and patients with FM experience the most invalidation. Psychological factors (happiness, agreeableness, and conscientiousness), loneliness, and pain intensity are associated with invalidation, irrespective of the rheumatic disease and may deserve dedicated interventions.

ObjectiveTo investigate the ex vivo pro-inflammatory properties of classical and non-classical monocytes as well as myeloid dendritic cells (mDCs) in systemic sclerosis (SSc) patients.MethodsSpontaneous production of CXCL10, CCL4, CXCL8 and IL-6 was intracellularly evaluated in classical, non-classical monocytes and Siglec-3-expressing mDCs from peripheral blood of SSc patients and healthy controls (HC) through flow cytometry. In addition, production of these cytokines was determined upon toll-like receptor (TLR) 4 plus Interferon-γ (IFN-γ) stimulation.ResultsThe frequency of non-classical monocytes spontaneously producing CXCL10 was increased in both limited (lcSSc) and diffuse cutaneous (dcSSC) subsets of SSc patients and CCL4 was augmented in dcSSc patients. The proportion of CCL4-producing mDCs was also elevated in dcSSc patients and the percentage of mDCS producing CXCL10 only in lcSSc patients. Upon stimulation, the frequency of non-classical monocytes expressing CXCL8 was increased in both patient groups and mDCs expressing CXCL8 only in lcSSc. Moreover, these parameters in unsupervised clustering analysis identify a subset of patients which are characterized by lung fibrosis and reduced pulmonary function.ConclusionsThese data point towards a role of activated non-classical monocytes and mDCs producing enhanced levels of proinflammatory cytokines in SSc, potentially contributing to lung fibrosis.Electronic supplementary materialThe online version of this article (10.1007/s00011-017-1106-7) contains supplementary material, which is available to authorized users.

the initial laboratory evaluation revealed an erythrocyte sedimentation rate (ESR) of 61 mm/h (normal value <10) and a serum C-reactive protein (CRP) level of 12 mg/L (normal value <5). Rheumatoid factor was 184 uI/mL (normal range <20), and serum angiotensin-converting enzyme (SaCE) was 65 u/I (normal value 8-52 u/L). Full blood count and blood chemistry were all within the normal range. Immunoglobulin G levels were 21.3 g/L (normal values 7.0-16.0 g/L); kappa light chain 18.58 g/L (6.66-14.65 g/L); and lambda light chain 7.43 g/L (2.99-6.99 g/L), with no detection of monoclonal component. Purified protein derivative test result was negative (an induration <5 mm).her chest radiograph (Fig. 1a) and high-resolution computed tomography (hRCt) (Fig. 2a) showed hilar and mediastinal lymphadenopathy and multiple small pulmonary nodules. Lung function tests and carbon monoxide diffusing capacity were normal. Bronchoalveolar lavage demonstrated an increase in CD4+/CD8+ t cell ratio (12.4). mycobacterial and fungal cultures were negative. to investigate the pulmonary nodules, a mediastinoscopydirected biopsy was performed. the histological exam revealed multiple noncaseating granulomas.the diagnosis of sarcoidosis (stage II) was established based on the clinical, imaging, and pathological findings. the patient was diagnosed with coexisting pSS and sarcoidosis.Because the respiratory symptoms and fatigue worsened over time, the patient was empirically treated with prednisolone 0.3 mg/kg/day for 6 weeks, with a slow tapering over 6 months, associated with calcium supplementation and alendronic acid/colecalciferol (70 mg/5,600 uI, weekly) [1].Gradually, all the symptoms improved, and 1 year later, the patient denied any respiratory or systemic symptoms.Abstract herein, we describe a 44-year-old female diagnosed with histologically proven coexistence of primary Sjögren's syndrome and sarcoidosis with pulmonary and muscular involvement. the differential diagnosis may be difficult, but this is not an exceptional case, which highlights the need to critically revise the consideration of sarcoidosis as an exclusion for primary Sjögren's syndrome, as established in current classification criteria.Keywords Primary Sjögren's syndrome · Sarcoidosis · Lung · muscle Case reporta 44-year-old Caucasian female was referred to our Department due to a 2-week history of dyspnea, persistent dry cough, arthralgia, and fatigue. Four years previously, primary Sjögren syndrome (pSS) had been diagnosed on the presence of sicca symptoms associated with SSa and SSB auto-antibodies, and a salivary gland biopsy demonstrating lymphocytic infiltrates (Chisholm-mason classification grade IV). She had been successfully treated with hydroxychloroquine 400 mg/day and an ophthalmic emulsion. her family and occupational and environmental histories were not contributory.Physical examination showed normal vital signs, and cardiovascular, pulmonary, and musculoskeletal clinical examinations were unremarkable. no peripheral adenopathies were noted.

Background: Measurement of skin involvement is essential for the diagnosis and assessment of prognosis and disease progression in systemic sclerosis (SSc). The modified Rodnan skin score (mRSS) is the gold standard measure of skin thickness, but it has been criticised for the lack of objectivity, poor inter-observer reproducibility and lack of sensitivity to change. Recently, shear-wave elastography (SWE) emerged as a promising tool for the objective and quantitative assessment of the skin in SSc patients. However, no studies have evaluated its sensitivity to change over time. Objective: To assess changes in skin stiffness in SSc patients using SWE during a 5-year follow-up. Methods: Skin stiffness [i.e. shear-wave velocity values (SWV) in metres per second] was assessed by SWE ultrasound (using virtual touch image quantification) at the 17 sites of the mRSS, in each participant, at baseline and follow-up. mRSS was performed at both time points. Differences between groups were analysed using the related-samples Wilcoxon signed-rank test and the Mann-Whitney U test. Results: We included 21 patients [85.7% females; mean age 56.3 (10.4) years at baseline, 57.1% with limited SSc] and 15 healthy controls [73.3% females; mean age 53.6 (14.1) years)]. The median follow-up was 4.9 (0.4) years. Skin stiffness decreased significantly at all Rodnan sites (p ≤ 0.001) (except in the fingers), in SSc patients, over time. The same phenomenon occurred in controls, but to a lesser degree, in terms of percentage change. The percentage reduction in skin stiffness varied in the different Rodnan sites and in different phases of the disease. In addition, SWV values also decreased significantly in 15/16 skin sites with local normal Rodnan at baseline, whereas local Rodnan skin score only changed significantly in the upper arm (p = 0.046) and forearm (p = 0.026). Conclusion: This study provides first-time evidence suggesting that skin SWV values are more sensitive to change over time than mRSS and reduce significantly over time in SSc and normal controls.

Significant alterations on circulating γδ T-cell subsets suggest a deregulated (increased) cytotoxic activity and thus enhanced pathogenic potential of CD27 γδ T cells in SSc.

While the present findings demonstrate the anti-inflammatory role of IL-4 in preventing the expression of IL-8 by Ac, the regulation of chemokines by anti-inflammatory cytokines is complex and depends on the cellular lineage.