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ID PMID Title PublicationDate abstract
27726238 Use of T1ρMR imaging in Sjögren's syndrome with normal appearing parotid glands: Initial 2017 Apr PURPOSE: To explore the feasibility of parotid spin-lattice relaxation time in the rotating frame (T1ρ) MR imaging in the diagnosis of Sjögren's syndrome (SS) without morphological changes of the parotid glands. MATERIALS AND METHODS: The study enrolled 32 consecutive SS patients without morphological changes of parotid glands and 32 age- and gender-matched healthy volunteers who underwent parotid 3.0 Tesla MR imaging, including T1ρ sequences. Follow-up imaging was performed at 3 months. T1 signal intensities and T1ρ values of bilateral parotid glands were compared using paired samples t-test. Parotid T1 signal intensities and T1ρ values were compared using two independent samples t-test. Diagnostic performance of the parotid T1ρ values was evaluated by receiver operating characteristic analysis. The intraclass correlation coefficient (ICC) was calculated to evaluate the reproducibility of parotid T1ρ measurements. RESULTS: There were no significant differences of T1 signal intensities and T1ρ values between bilateral parotid glands in SS patients and healthy volunteers (P = 0.170, 0.886 and 0.942, 0.229). The parotid T1ρ values of SS patients (96.47 ± 15.38 ms) were significantly higher than those of healthy volunteers (84.25 ± 6.11 ms) (P < 0.001), while there were no significant differences of T1 signal intensities between SS patients and healthy volunteers (P = 0.655). With a cutoff value of 88.02 ms, the sensitivity and specificity of the parotid T1ρ value was 75.0% and 100.0% in the diagnosis of SS. The reproducibility of parotid T1ρ measurement was excellent (ICC: 0.934-0.995). CONCLUSION: Parotid T1ρ MR imaging held a potential role in diagnosing SS without morphological changes of parotid glands. LEVEL OF EVIDENCE: 2 J. Magn. Reson. Imaging 2017;45:1005-1012.
26962839 Risk of Chronic Obstructive Pulmonary Disease in Female Adults With Primary Sjögren Syndr 2016 Mar No large-scale population-based cohort study has ever investigated the risk of developing chronic obstructive pulmonary disease (COPD) in patients with Sjögren syndrome (SS). This study evaluated the risk of COPD in women with primary SS (pSS) in a nationwide population.We used the data of the National Health Insurance Research Database of Taiwan to establish a pSS group consisting of 3013 female adults diagnosed between 2000 and 2005, and a non-SS group consisting of 12,052 women without SS matched by a propensity score. Incident COPD cases were identified to the end of 2011. The pSS group to non-SS group adjusted hazard ratios (aHRs) of COPD were estimated using multivariable Cox proportional hazards regression analysis.After a mean follow-up period of 7.99 years, the incidence of COPD was 1.4-fold greater in the pSS group than in the non-SS group (3.87 vs 2.77 per 1000 person-years) with an aHR of 1.39 (95% confidence interval [CI] = 1.10-1.75, P = 0.007). The COPD incidence was 7-fold greater for women aged 50 years and above than women aged 20 to 49, with the aHR of 4.24 (95% CI = 3.06-5.88, P < 0.001). Comorbidity increased the COPD risk further for women with pSS. Women with both pSS and comorbidity had an aHR of 3.11 (95% CI = 2.23-4.33, P < 0.001) for COPD, compared to those free of both pSS and comorbidity.Women with pSS are at a greater risk of developing COPD than those without SS. Patients with SS require close monitoring to prevent COPD development, particularly for those with comorbidity.
26715188 Assessment of the Stiffness of Major Salivary Glands in Primary Sjögren's Syndrome throug 2016 Mar The purpose of the study described here was to evaluate salivary gland stiffness in primary Sjögren's syndrome (pSS) via acoustic radiation force impulse imaging, including Virtual Touch tissue quantification (VTQ) and Virtual Touch tissue imaging quantification (VTIQ). Twenty-one patients with pSS and 11 healthy patients were included, and the paired parotid and submandibular glands of all of the patients were examined using VTQ and VTIQ. Differences between the two groups were compared with independent and paired t-tests. The VTQ value for the parotid in the pSS group was significantly higher than that obtained for the control group (1.33 ± 0.22 and 1.18 ± 0.04 m/s, respectively, p < 0.01). The VTIQ values for the parotid and submandibular gland were both significantly higher in the pSS group than in the control group (p < 0.05). In the pSS group, a positive correlation was observed between the VTQ and VTIQ results for the parotid and submandibular glands. In summary, the stiffness of the major salivary glands in patients with pSS was increased compared with that of patients with normal glands. This finding indicates that VTQ and VTIQ imaging may be valuable adjuncts to gray-scale ultrasonography for the clinical diagnosis of pSS.
25906201 Diffuse Cystic Lung Disease. Part II. 2015 Jul 1 The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management.
26301001 Anatomic Landmarks of the Distal Radioulnar Joint. 2015 HYPOTHESIS: Using Lister's tubercle and the ulnar styloid as landmarks, accurate localization of the distal radioulnar joint can be achieved without the need for an image-guided approach. METHODS: Cadaveric dissection of 16 upper extremities was performed to measure the relationships between the ulnar styloid, Lister's tubercle, and the distal radioulnar joint. In each specimen, the location of the distal radioulnar joint (point A) in relation to Lister's tubercle and the ulnar styloid was determined as follows: (1) the perpendicular distance between the distal radioulnar joint and ulnar styloid-Lister's tubercle was measured; (2) with A' marking the intersection of this distance and the ulnar styloid-Lister's tubercle line, the location of the distal radioulnar joint along the ulnar styloid-Lister's tubercle axis was determined by comparing ulnar styloid-A' and A'-Lister's tubercle with ulnar styloid-Lister's tubercle. RESULTS: The mean distance between ulnar styloid-Lister's tubercle was 4.3 ± 0.4 cm. The mean perpendicular distance between the distal radioulnar joint and the ulnar styloid-Lister's tubercle line was 0.2 ± 0.1 cm proximal to the ulnar styloid-Lister's tubercle line. The ratio of ulnar styloid-A' and A'-Lister's tubercle to ulnar styloid-Lister's tubercle was 0.5 ± 0.03 and 0.5 ± 0.03, respectively. CONCLUSIONS: Simple relationships between the ulnar styloid and Lister's tubercle serve as reliable landmarks for locating the distal radioulnar joint. The distal radioulnar joint is centered about the midpoint of the ulnar styloid-Lister's tubercle axis and slightly proximal to it. This may improve the accuracy and efficacy of corticosteroid injections in the treatment of distal radioulnar joint arthritis without the need for image guidance.
27230945 The expression of tenascin-C and tenascin-W in human ossicles. 2016 Sep The ossicles of the middle ear (the malleus, incus and stapes) transmit forces resulting from vibrations of the tympanic membrane to the cochlea where they are coded as sound. Hearing loss can result from diseases such as rheumatoid arthritis (RA) that affect the joints between the ossicles or degenerative processes like otosclerosis that lead to ankylosis of the footplate of the stapes in the oval window of the cochlea. In this study, immunohistochemistry was used to determine if the extracellular matrix glycoproteins tenascin-C or tenascin-W are expressed in the incudomalleolar and incudostapedial joints of ossicles dissected from human cadavers. Tenascin-C, which is expressed during inflammatory conditions including RA, was seen in the articular cartilage of the incudomalleolar joints and the head of the stapes. Tenascin-W, in contrast, was enriched in the annular ligament that anchors the footplate of the stapes into the oval window of the cochlea.
27900604 Assessment of interstitial lung disease in Sjögren's syndrome by lung ultrasound: a pilot 2017 Apr The background of this study is to assess the accuracy of lung ultrasound (LUS) to diagnose interstitial lung disease (ILD) in Sjögren's syndrome (Sjs), in patients who have any alterations in pulmonary function tests (PFT) or respiratory symptoms. LUS was correlated with chest tomography (hrCT), considering it as the imaging gold standard technique to diagnose ILD. This is a pilot, multicenter, cross-sectional, and consecutive-case study. The inclusion criteria are ≥18 years old, Signs and symptoms: according to ACEG 2002 criteria, respiratory symptoms (dyspnea, cough), or any alterations in PFR. LUS was done following the International Consensus Conference on Lung Ultrasound protocol for interstitial syndrome (B pattern). Of the 50 patients in follow-up, 13 (26%) met the inclusion criteria. All were women with age 63.62 years (range 39-88). 78.6% of the cases had primary Sjs (SLE, RA, n = 2). The intra-rater reliability k is 1, according to Gwet's Ac1 and GI index (probability to concordance-e(K)-, by Cohen, of 0.52). LUS has a sensitivity of 1 (95% CI 0.398-1.0), specificity of 0.89 (95% CI 0.518-0.997), and a positive probability reason of 9.00 (95% CI 7.1-11.3) to detect ILD. The correlation of Pearson is r = 0.84 (p < 0.001). To check the accuracy of LUS to diagnose ILD, a completely bilateral criterion of yes/no for interstitial pattern was chosen, AUC reaches significance, 0.94 (0.07) (95% CI 0.81-1.0, p = 0.014). LUS reaches an excellent correlation to hrCT in Sjs affected with ILD, and might be a useful technique in daily clinical practice for the assessment of pulmonary disease in the sicca syndrome.
26873295 Insights from the ganglionic acetylcholine receptor autoantibodies in patients with Sjögr 2016 Sep OBJECTIVE: It is not known whether autonomic neuropathy is a feature of Sjögren's syndrome (SS) or whether it is related to circulating antiganglionic acetylcholine receptor (gAChR) antibodies. The goal of the present study was to investigate the autonomic dysfunction in patients with SS and the associations between autonomic dysfunction, anti-gAChR antibodies, and clinical features of SS. METHODS: (1) The first observational study tested for the presence of gAChR antibodies in the serum samples from 39 patients with SS (absent information regarding autonomic symptoms) and healthy volunteers. (2) In the second study, serological and clinical data from 10 Japanese patients diagnosed with SS were reviewed. These patients showed autonomic dysfunction, and luciferase immunoprecipitation systems (LIPS) test was conducted to detect anti-α3 and anti-β4 gAChR antibodies. (3) In the final analysis, we combined the data of seropositive SS patients with autonomic symptom from the first study with all of the patients from the second study, and analyzed the clinical features. RESULTS: (1) The LIPS assay revealed that anti-gAChRα3 and anti-gAChRβ4 antibodies were detected in the sera from patients with SS (23.1%, 9/39). Five of nine SS patients had autonomic symptoms. (2) Anti-α3 and anti-β4 gAChR antibodies were also detected in 80.0% (8/10) of patients with SS with autonomic symptoms. Six of the ten patients were diagnosed as having SS after neurological symptoms developed. These seropositive patients had predominant and severe autonomic symptoms and were diagnosed with autonomic neuropathy. (3) Thirteen of fifteen SS patients with autonomic symptoms (86.7%) were seropositive for anti-gAChR antibodies, and we confirmed sicca complex, orthostatic hypotension, upper and lower gastrointestinal (GI) symptoms, and bladder dysfunction at high rates. CONCLUSION: The present results suggest the possibility of anti-gAChR antibodies aiding the diagnostics of SS with autonomic dysfunction.
26231345 Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force r 2015 Dec OBJECTIVE: To reach a European consensus on the definition and characterization of the main organ-specific extraglandular manifestations in primary SS. METHODS: The EULAR-SS Task Force Group steering committee agreed to approach SS-related systemic involvement according to the EULAR SS Disease Activity Index (ESSDAI) classification and proposed the preparation of four separate manuscripts: articular, cutaneous, pulmonary and renal ESSDAI involvement; muscular, peripheral nervous system, CNS and haematological ESSDAI involvement; organs not included in the ESSDAI classification; and lymphoproliferative disease. Currently available evidence was obtained by a systematic literature review focused on SS-related systemic features. RESULTS: The following information was summarized for articular, cutaneous, pulmonary and renal involvement: a clear, consensual definition of the clinical feature, a brief epidemiological description including an estimate of the prevalence reported in the main clinical series and a brief list of the key clinical and diagnostic features that could help physicians clearly identify these features. Unfortunately we found that the body of evidence relied predominantly on information retrieved from individual cases, and the scientific information provided was heterogeneous. The analysis of types of involvement was biased due to the unbalanced reporting of severe cases over non-severe cases, although the main sources of bias were the heterogeneous definitions of organ involvement (or even the lack of definition in some studies) and the heterogeneous diagnostic approach used in studies to investigate involvment of each organ. CONCLUSION: The proposals included in this article are a first step to developing an optimal diagnostic approach to systemic involvement in primary SS and may pave the way for further development of evidence-based diagnostic and therapeutic guidelines.
27853859 Hemophagocytic lymphohistiocytosis in a patient with Sjögren's syndrome: case report and 2017 Apr Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin. In this manuscript, we present the case of a patient with primary Sjögren's syndrome who developed HLH after an acute infection by Cytomegalovirus. We will describe and discuss the pathogenesis, differential diagnosis and a pragmatic approach to the treatment for this critically important and, when diagnosed early, potentially curable syndrome.
26831141 The lived experience of Sjögren's Syndrome. 2016 Feb 1 BACKGROUND: Sjögren's Syndrome is an autoimmune exocrinopathy characterised by lymphocytic infiltration of exocrine glands in multiple sites, with dry mouth as a primary presenting symptom. Although quantitative studies have shown the negative impact of both dry mouth and Sjögren's Syndrome on patients' quality of life, no qualitative diary and interview study has been undertaken to examine the lived experience of dry mouth for Sjögren's Syndrome sufferers. The aim of this qualitative study was to provide clinicians with insight into how dry mouth can impact on the daily lives of Sjögren's Syndrome patients. METHODS: The American-European Consensus Group (AECG) Revised International Classification criteria were used to identify participants from patients seen in an oral medicine clinic. After pilot study work to test the approach, the 10 main study participants were recruited. Diary entries and semi-structured interviews were used to explore how dry mouth affects their lives. Owing to the exploratory nature of the research, thematic content analysis was applied, allowing the themes to arise naturalistically from the data without bias or elicitation. RESULTS: The data showed that it is unrealistic to understand the experience of a single symptom, but that the disease as a whole needs to be taken into perspective. The empirical evidence supported four main themes that depicted the lived experience of Sjögren's Syndrome. These included: (1) the journey to diagnosis; (2) disease impact spectrum (of dry mouth amid other symptoms); (3) interactions with healthcare professionals; and (4) the positive coping process. CONCLUSIONS: The findings revealed patients' perspectives on diagnosis, coping with dry mouth and Sjögren's Syndrome, and interaction with healthcare professionals. Dry mouth is not a trivial symptom for Sjögren's Syndrome sufferers; it has considerable impact on their day-to-day lives. Healthcare professionals need to understand patients as individuals in their environment in order to be part of the Sjögren's journey.
26524897 [Roles of Musculoskeletal Ultrasonography in the Management of Rheumatic Diseases]. 2015 May Inflammation of soft tissues and the subsequent structural damage are the characteristic features of many rheumatic conditions. However, the conventional measures to evaluate these features are not accurate, potentially causing under- and over-diagnosis or treatment. Musculoskeletal ultrasonography, on the other hand, directly visualizes features which are characteristic to rheumatic conditions, such as synovitis, tenosynovitis, bursitis, enthesitis, crystal depositions, bone erosions, and osteophytes/enthesophytes. By visualizing these key features, ultrasound facilitates a more accurate evaluation of rheumatic diseases such as rheumatoid arthritis (RA), spondyloarthropathy, crystal-induced arthritis, and osteoarthritis. For RA, we investigated the impact of ultrasound on the 2010 ACR/EULAR RA classification criteria when joint involvement was determined. We assessed 109 patients with early arthritis using ultrasound. When the presence of joint swelling and number of involved joints were determined by ultrasound, the classification of RA was different from that without ultrasound in approximately 20% of the patients. Moreover, the accuracy of the classification to identify patients who required methotrexate treatment within a year improved by ultrasound. In addition, we and other investigators demonstrated the advantage of ultrasound over conventional measures in the assessment of disease activity of RA. Ultrasound also supports the education of both physicians and patients and communication between them. The utilization of musculoskeletal ultrasonography in more hospitals and clinics is expected to improve the quality of daily practice for rheumatic diseases.
27659405 Cystic lung disease in Sjögren's syndrome: An observational study. 2017 May OBJECTIVES: To analyze the prevalence, characteristics and outcome of cystic lung disease associated with Sjögren's syndrome (SS). METHODS: From June 2010 to February 2015, 90 consecutive SS patients [60.1±14.8years; 88 (97.8%) female, 75 (83.3%) primary SS] had a systematic chest CT-scan. The presence of thin-walled cysts was analyzed by one experienced radiologist. Demographic data, clinical history, laboratory findings, and pulmonary function tests were extracted retrospectively from medical records. RESULTS: Twenty-one (23.3%) patients had cysts on CT scan performed 40.5±54.5months after SS diagnosis. Cysts number ranged from 1 to 25 were often bilateral (52.4%) and mostly located in the middle lung zone (76.2%). Cysts were isolated (n=6, 28.6%) or associated with other lesions, including bronchiectasis (n=5, 23.8%), micronodules (n=5, 23.8%), ground-glass opacity (n=4, 19%) and/or air trapping (n=3, 14.3%). Most patients with cysts (57.1%) had no respiratory symptoms. When comparing SS patients with and without cysts, patients with cysts tended to be older (65.3±15.3 versus 58.5±14.4years, P=0.06). Smoking habits were similar in both groups. Anti-SSB antibodies were more frequently detected in patients with cysts (57.1% vs. 26.1%, P=0.02). Pulmonary function tests were normal or displayed only mild small airways obstruction and reduced diffusion capacity to carbon monoxide. Four (19%) patients with cysts had a past history of associated pulmonary disease, including interstitial lung disease. During follow-up (25.1±17.7months), no patient developed specific lung disease or lymphoproliferative disorders. CONCLUSIONS: Cystic lung disease is frequent, benign, associated with anti-SSB/La antibodies and has no impact on outcome in SS.
26628608 Test-retest Reliability and Correlations of 5 Global Measures Addressing At-work Productiv 2016 Feb OBJECTIVE: Several global measures to assess at-work productivity loss or presenteeism in patients with rheumatic diseases have been proposed, but the comparative validity is hampered by the lack of data on test-retest reliability and comparative concurrent and construct validity. Our objective was to test-retest 5 global measures of presenteeism and to compare the association between these scales and health-related well-being. METHODS: Sixty-five participants with inflammatory arthritis or osteoarthritis in paid employment were recruited from 7 countries (UK, Canada, Netherlands, France, Sweden, Romania, and Italy). At baseline and 2 weeks later, 5 global measures of presenteeism were evaluated: the Work Productivity Scale-Rheumatoid Arthritis (WPS-RA), Work Productivity and Activity Impairment Questionnaire (WPAI), Work Ability Index (WAI), Quality and Quantity questionnaire (QQ), and the WHO Health and Performance Questionnaire (HPQ). Agreement between the 2 timepoints was assessed using single-measure intraclass correlations (ICC) and correlated between each other and with visual analog scale general well-being scores at followup by Spearman correlation. RESULTS: ICC between measures ranged from fair (HPQ 0.59) to excellent (WPS-RA 0.78). Spearman correlations between measures were moderate (Qquality vs WAI, r = 0.51) to strong (WPS-RA vs WPAI, r = 0.88). Correlations between measures and general well-being were low to moderate, ranging from -0.44 ≤ r ≤ 0.66. CONCLUSION: Test-retest results of 4 out of 5 global measures were good, and the correlations between these were moderate. The latter probably reflect differences in the concepts, recall periods, and references used in the measures, which implies that some measures are probably not interchangeable.
27756976 Rituximab in autoimmune diseases. 2016 Aug Rituximab is a monoclonal antibody that depletes B cells from the circulation. It was originally used to treat lymphoma but is increasingly used for the treatment of autoimmune diseases. Rituximab was found to be effective in randomised controlled trials for rheumatoid arthritis, granulomatosis with polyangiitis and other antineutrophil cytoplasmic antibody-associated vasculitides. However, evidence of efficacy is very limited for many other autoimmune conditions. Before starting rituximab, it is important to check the patient's baseline immunoglobulins and immunisation status. Patients should also be screened for latent infections and other contraindications.
26653129 Diagnostic and Prognostic Metabolites Identified for Joint Symptoms in the KORA Population 2016 Feb 5 This study aims at identifying metabolites that significantly associate with self-reported joint symptoms (diagnostic) and metabolites that can predict the change from a symptom-free status to the development of self-reported joint symptoms after a 7 years period (prognostic). More than 300 metabolites were analyzed for 2246 subjects from the longitudinal study of the KORA (Cooperative Health Research in the Region of Augsburg, Germany), specifically the fourth survey S4 and its 7-year follow-up study F4. Two types of self-reported symptoms, chronic joint inflammation and worn out joints, were used for the analyses. Diagnostic analysis identified dysregulated metabolites in cases with symptoms compared with controls. Prognostic analysis identified metabolites that differentiate subjects in S4 who remained symptom-free after 7 years (F4) from those who developed any combination of symptoms. 48 metabolites were identified as nominally significantly (p < 0.05) associated with the self-reported symptoms in the diagnostic analysis, among which steroids show Bonferroni significance. 45 metabolites were identified as nominally significantly associated with developing symptoms after 7 years, among which hippurate showed Bonferroni significance. We show that metabolic profiles of self-reported joint symptoms are in line with metabolites known to associate with various forms of arthritis and suggest that future studies may benefit from that by investigating the possible use of self-reporting/questionnaire along with metabolic markers for the early referral of patients for further diagnostic workup and treatment of arthritis.
25936155 [Rheumatic diseases and sex differences]. 2015 Apr Recently a concept of "sex differences" in medicine has been spreading. It is well known that autoimmune diseases, especially rheumatoid arthritis or systemic lupus erythematosus mostly affect women, and it is clear that there is a strong sex differences in many rheumatic diseases. Sex difference influences to progression or prognosis of the disease. Although the mechanisms of sex difference are not clear, the influence of sex hormones or sex chromosomes which gives immune system is partially elucidated. In addition, sex hormones may become the future new treatment target, and progress in the study regarding this event is expected in the future.
26742238 [Interstitial lung disease in connective tissue diseases]. 2015 Nov 18 Interstitial lung disease represents a major clinical aspect of the four major connective tissue diseases: rheumatoid arthritis, scleroderma, polymyositis/dermatomyositis and systemic lupus erythematosus (SLE). Early recognition in the course of the disease is essential, as interstitial lung disease will often determine the vital prognosis of these patients. Treatment is most frequently based on experts' opinion, because there are only few randomized controlled trials in this field.
26028960 Biologic monotherapy in the treatment of rheumatoid arthritis. 2015 Biologics, possibly in combination with a conventional disease-modifying antirheumatic drug (DMARD) - preferably methotrexate (MTX), are used in accordance with the recommendations of the international rheumatological societies. However, in clinical practice, this recommendation is often problematic, as many rheumatologists know from personal experience. The quality of life of the patient is affected mainly by drug-induced intolerances (eg, MTX). Thus, the acceptance of the patient to treatment is often so inadequate that a discontinuation of the drug is necessary. In daily practice, approximately 30% of patients with biological therapy receive no concomitant DMARD according to the register data.
27538753 Deciphering cardiac involvement in systemic inflammatory diseases: noninvasive tissue char 2016 Nov Cardiac involvement in systemic inflammatory diseases (SID) has a major impact on patients' morbidity and mortality, yet the pathways to its recognition and management remain poorly established. Areas covered: Overall clinical management in SID patients is primarily guided by systemic symptoms. Cardiovascular disease goes largely undetected, as it evolves through years of a protracted and subclinical course. Despite the increased awareness and insights into the mechanistic role of the inflammatory pathways, clinical management of cardiac involvement continues to rely on diagnostic means, which are frequently insensitive, invasive and rely on radiation exposure. Advanced tissue characterisation with cardiovascular magnetic resonance (CMR) offers an accurate, non-invasive and radiation-free diagnostic method with obvious advantages: it is able to inform on a range of cardiovascular pathophysiology, as well as support safe serial examinations, informing on the disease presence, progress and response to treatment. Expert commentary: We summarise the recent advances in non-invasive imaging, and bridge the novel insights into pathophysiology with future posibilities in diagnosis and manangement of SID patients. We propose an interdisciplinary framework to screening of cardiac involvement in SID using an indepth phenotyping of evolution of cardiovascular disease, to decipher the opportunities to improve patients' cardiac care.