Validation of the Italian version of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire.

Objectives: The primary objective of this study was the translation and validation of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire into Italian, denoted as AAV-PRO_ita. The secondary objective was to evaluate the impact of ANCA-associated vasculitis (AAV) on quality of life (QoL) and work impairment in a large cohort of Italian patients. Methods: The study design took a prospective cohort study approach. First, the AAV-PRO was translated into Italian following the step guidelines for translations. The new AAV-PRO_ita questionnaire covered three disease domains: organ-specific and systemic symptoms and signs; physical function; and social and emotional impact. Second, Italian-speaking AAV patients were recruited from 17 Italian centres belonging to the Italian Vasculitis Study Group. Participants completed the AAV-PRO_ita questionnaire at three time points. Participants were also requested to complete the work productivity and activity impairment: general health questionnaire. Results: A total of 276 AAV patients (56.5% women) completed the questionnaires. The AAV-PRO_ita questionnaire demonstrated a good internal consistency and test-retest reliability. Female AAV patients scored higher (i.e. worse) in all thee domains, especially in the social and emotional impact domain (P < 0.001). Patients on glucocorticoid therapy (n = 199) had higher scores in all domains, especially in the physical function domain (P < 0.001), compared with patients not on glucocorticoid therapy (n = 77). Furthermore, patients who had at least one relapse of disease (n = 114) had higher scores compared with those who had never had one (n = 161) in any domain (P < 0.05). Finally, nearly 30% of the patients reported work impairment. Conclusion: The AAV-PRO_ita questionnaire is a new 29-item, disease-specific patient-reported outcome measuring tool that can be used in AAV research in the Italian language. Sex, glucocorticoids and relapsing disease showed the greatest impact on QoL.

Clinical and histological features of patients with primary Sjögren's syndrome and autoimmune thyroiditis: a national multicentre cross-sectional study.

OBJECTIVES: Primary Sjögren's syndrome (pSS) is frequently associated with autoimmune thyroiditis (AT). The aim of this study was to evaluate the prevalence of AT in a national cohort of pSS and to describe the clinical and histological phenotype of patients with pSS and associated AT. METHODS: In this multicentre cross-sectional study, data from 2546 pSS were collected and the presence of AT was reported. In a subgroup, the histology of minor salivary glands was evaluated. Differences between pSS with and without AT were evaluated. RESULTS: A concomitant pSS and AT was detected in 19.6% of cases. Patients with pSS and AT displayed a lower prevalence of lymphoma, male sex and disease-modifying anti-rheumatic drugs (DMARDs) use and a higher prevalence of fibromyalgia, coeliac disease and hypergammaglobulinaemia. Multivariable analysis confirmed a higher prevalence of fibromyalgia and coeliac disease and lower use of DMARDs. In a subgroup of patients (n=232), a significantly higher focus score and number of foci was detected in pSS without AT (n=169) as compared to pSS with AT (n=54). CONCLUSIONS: This is the largest study evaluating the coexistence of pSS and AT. We confirm a high association between pSS and AT and describe the presence of a different phenotype characterized by a higher rate of celiac disease and fibromyalgia. Although not significant, the lower prevalence of both lymphoma and intake of DMARDs, along with a significantly lower focus score and number of foci, possibly suggest a more favourable outcome in concomitant pSS and AT which further deserve future investigations.

Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study.

Background: What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score. Methods: In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjögren Registry. Inclusion criteria consisted of fulfilling 2002/2016 SjS classification criteria, and exclusion criteria included chronic HCV/HIV infections and associated systemic autoimmune diseases. A statistical approach based on a directed acyclic graph was used, with all-cause and Sjögren-related mortality as primary endpoints. The key determinants that defined the disease phenotype at diagnosis (glandular, systemic, and immunological) were analysed as independent variables. Findings: Between January 1st, 2014 and December 31, 2023, data from 11,372 patients with primary SjS (93.5% women, 78.4% classified as White, mean age at diagnosis of 51.1 years) included in the Registry were analysed. 876 (7.7%) deaths were recorded after a mean follow-up of 8.6 years (SD 7.12). Univariate analysis of prognostic factors for all-cause death identified eight Sjögren-related variables (ocular and oral tests, salivary biopsy, ESSDAI, ANA, anti-Ro, anti-La, and cryoglobulins). The multivariate CPH model adjusted for these variables and the epidemiological features showed that DAS-ESSDAI (high vs no high: HR = 1.68; 95% CI, 1.27-2.22) and cryoglobulins (positive vs negative: HR = 1.72; 95% CI, 1.22-2.42) were independent predictors of all-cause death. Of the 640 deaths with available information detailing the specific cause of death, 14% were due to systemic SjS. Univariate analysis of prognostic factors for Sjögren-cause death identified five Sjögren-related variables (oral tests, clinESSDAI, DAS-ESSDAI, ANA, and cryoglobulins). The multivariate competing risks CPH model adjusted for these variables and the epidemiological features showed that oral tests (abnormal vs normal results: HR = 1.38; 95% CI, 1.01-1.87), DAS-ESSDAI (high vs no high: HR = 1.55; 95% CI, 1.22-1.96) and cryoglobulins (positive vs negative: HR = 1.52; 95% CI, 1.16-2) were independent predictors of SjS-related death. Interpretation: The key mortality risk factors at the time of SjS diagnosis were positive cryoglobulins and a high systemic activity scored using the ESSDAI, conferring a 2-times increased risk of all-cause and SjS-related death. ESSDAI measurement and cryoglobulin testing should be considered mandatory when an individual is diagnosed with SjS. Funding: Novartis.

Multicentre case-control study evaluating the safety of anti-SARS-CoV-2 vaccines in a cohort of patients with systemic vasculitis.

OBJECTIVES: Data on the safety of anti-SARS-CoV-2 vaccines in patients with rare rheumatic diseases, such as systemic vasculitis (SV), are limited. The aim of this study was to evaluate the occurrence of a disease flare and the appearance of adverse events (AEs) following administration of anti-SARS-CoV-2 vaccine in a multicentre cohort of patients with SV. METHODS: Patients with SV and healthy controls (HC) from two different Italian rheumatology centres were asked to complete a questionnaire assessing disease flares occurrence, defined as new onset of clinical manifestations related to vasculitis needing an implementation of therapy, and local/systemic AEs appearance following anti SARS-CoV-2 vaccination. RESULTS: 107 patients with SV (57 ANCA-associated) and 107 HC were enrolled. A disease flare occurred in only one patient (0.93%) with microscopic polyangiitis after the first dose of an mRNA vaccine. After both the first and the second vaccine dose administration, no significant differences in AEs between patients with SV and HC were observed; no serious AEs were reported as well. CONCLUSIONS: These data suggest a good risk profile for anti-SARS-CoV-2 vaccine in patients with systemic vasculitis.

Application of Machine Learning Models in Systemic Lupus Erythematosus.

Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disease and is extremely heterogeneous in terms of immunological features and clinical manifestations. This complexity could result in a delay in the diagnosis and treatment introduction, with impacts on long-term outcomes. In this view, the application of innovative tools, such as machine learning models (MLMs), could be useful. Thus, the purpose of the present review is to provide the reader with information about the possible application of artificial intelligence in SLE patients from a medical perspective. To summarize, several studies have applied MLMs in large cohorts in different disease-related fields. In particular, the majority of studies focused on diagnosis and pathogenesis, disease-related manifestations, in particular Lupus Nephritis, outcomes and treatment. Nonetheless, some studies focused on peculiar features, such as pregnancy and quality of life. The review of published data demonstrated the proposal of several models with good performance, suggesting the possible application of MLMs in the SLE scenario.

Maladaptive Autophagy in the Pathogenesis of Autoimmune Epithelitis in Sjögren's Syndrome.

OBJECTIVE: Salivary gland epithelial cells (SGECs) are key cellular drivers in the pathogenesis of primary Sjögren's syndrome (SS); however, the mechanisms sustaining SGEC activation in primary SS remain unclear. We undertook this study to determine the role of autophagy in the survival and activation of SGECs in primary SS. METHODS: Primary SGECs isolated from the minor SGs of patients with primary SS or sicca syndrome were evaluated by flow cytometry, immunoblotting, and immunofluorescence to assess autophagy (autophagic flux, light chain 3 IIB [LC3-IIB], p62, LC3-IIB+/lysosome-associated membrane protein 1 [LAMP-1] staining), apoptosis (annexin V/propidium iodide [PI], caspase 3), and activation (intercellular adhesion molecule, vascular cell adhesion molecule). Focus score and germinal center presence were assessed in the SGs from the same patients to assess correlation with histologic severity. Human SG (HSG) cells were stimulated in vitro with peripheral blood mononuclear cells (PBMCs) and serum from primary SS patients in the presence or absence of autophagy inhibitors to determine changes in autophagy and epithelial cell activation. RESULTS: SGECs from primary SS patients (n = 24) exhibited increased autophagy (autophagic flux [P = 0.001]; LC3-IIB [P = 0.02]; p62 [P = 0.064]; and as indicated by LC3-IIB/LAMP-1+ staining), increased expression of antiapoptotic molecules (Bcl-2 [P = 0.006]), and reduced apoptosis (annexin V/PI [P = 0.002]; caspase 3 [P = 0.057]), compared to samples from patients with sicca syndrome (n = 16). Autophagy correlated with histologic disease severity. In vitro experiments on HSG cells stimulated with serum and PBMCs from primary SS patients confirmed activation of autophagy and expression of adhesion molecules, which was reverted upon pharmacologic inhibition of autophagy. CONCLUSION: In primary SS SGECs, inflammation induces autophagy and prosurvival mechanisms, which promote SGEC activation and mirror histologic severity. These findings indicate that autophagy is a central contributor to the pathogenesis of primary SS and a new therapeutic target.

Resilience in women with primary Sjögren's syndrome.

To assess the relationship between resilience and several diseases and individual features in primary SjÓ§gren's Syndrome (SS) patients. Resilience was assessed using the Resilience Scale (RS-14). Disease activity, damage, and reported symptoms were assessed by means of ESSDAI (EULAR Sjögren's syndrome disease activity index), SSDDI (SjÓ§gren's Syndrome Disease Damage Index) and ESSPRI (EULAR SjÓ§gren's Syndrome Patient Reported Index). EuroQol, HADS (Hospital Anxiety and Depression Scale), SF-12 (Short-form 12 health survey), FAS (Fatigue Assessment Scale), FACIT-F (Functional Assessment of Chronic Illness Therapy - Fatigue), and IPAQ (International Physical Activity Questionnaire) questionnaires were submitted to evaluate physical and mental well-being of the recruited patients. Data about the autoimmune profile, systemic manifestations, and current therapy were collected. Educational qualifications and work activities were also considered. Descriptive, correlational, and linear regression analysis were performed. 74 consecutive women with primary SS and 74 sex and age-matched healthy subjects as a control group were recruited. SS patients displayed a moderate value of resilience (median 78.5) with no significant difference compared to controls (p = 0.38). An inverse relationship was found between resilience and mood disorders such as anxiety (p = 0.038) and depression (p < 0.001). Greater resilience was associated with a better perception of the quality of life (p = 0.02) and general health (p < 0.001), as well as with less fatigue (p = 0.008) and a more physically active lifestyle (p = 0.001). No significant relationship was found neither between resilience and age, socio-demographic and disease characteristics, nor with ESSDAI (p = 0.26), ESSPRI (p = 0.83) and SSDDI (p = 0.67). This is the first study assessing resilience in a large group of unselected primary SS patients. Most resilient primary SS patients are less depressed and show a better perception of their health. Greater resilience tends to correlate with less anxiety, physical and mental fatigue, and a more active lifestyle, while no association with disease activity and duration, damage, and socio-demographic features was detected. Considering the well-known role of resilience in helping to better cope with chronic illnesses, its assessment in SS patients should not be overlooked and the possible strategies for its improvement should be better explored and further implemented.

Peripheral Nervous System Involvement in Sjögren's Syndrome: Analysis of a Cohort From the Italian Research Group on Sjögren's Syndrome.

Purpose: The prevalence of peripheral nervous system (PNS) involvement in primary Sjögren's syndrome (pSS) has been reported to range from 2% to over 50%. Bias in study designs, including low number of patients and unclearly defined rheumatological and neurological diagnosis could explain such variability. Consequently, the exact depiction of PNS involvement in pSS is still lacking. This study aimed at analyzing the prevalence and the clinical and laboratory factors associated with PNS involvement in a very large cohort of well-characterized pSS patients with a clearly defined neurological diagnosis. Methods: Clinical and serological data of 1,695 pSS patients with specific and accurate information on PNS involvement were analyzed. Comparisons between patients with and without PNS involvement and between patients with distinct subsets of PNS manifestations were performed. Results: Prevalence of PNS involvement was 3.7%. The most frequent types observed were pure sensory neuropathies and axonal sensorimotor polyneuropathies (SMP). Patients with PNS involvement exhibited a more active disease profile and were more frequently treated with immunosuppressants. Intriguingly, clinical and serological negative prognostic factors, including purpura, extra-glandular manifestations, leukopenia, low complement and cryoglobulinemia, principally characterized patients with SMP, while subjects with pure sensory neuropathy displayed a milder phenotype. Conclusion: Our results highlight that PNS involvement is rather rare, but prognostically relevant in pSS. Main adverse prognostic features characterize patients with SMP, while pure sensory neuropathies are usually associated with a mild clinical picture. These findings, useful for patient stratification, may suggest protean pathogenic pathways underlying different types of PNS manifestations in pSS.

Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis of 158 children.

OBJECTIVES: To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. RESULTS: Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. CONCLUSIONS: Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.

Salivary Gland Ultrasonography in Sjögren's Syndrome: A European Multicenter Reliability Exercise for the HarmonicSS Project.

Objectives: Salivary gland ultrasonography (SGUS) is increasingly applied for the management of primary Sjögren's syndrome (pSS). This study aims to: (i) compare the reliability between two SGUS scores; (ii) test the reliability among sonographers with different levels of experience. Methods: In the reliability exercise, two four-grade semi-quantitative SGUS scoring systems, namely De Vita et al. and OMERACT, were tested. The sonographers involved in work-package 7 of the HarmonicSS project from nine countries in Europe were invited to participate. Different levels of sonographers were identified on the basis of their SGUS experience and of the knowledge of the tested scores. A dedicated atlas was used as support for SGUS scoring. Results: Twenty sonographers participated in the two rounds of the reliability exercise. The intra-rater reliability for both scores was almost perfect, with a Light's kappa of 0.86 for the De Vita et al. score and 0.87 for the OMERACT score. The inter-rater reliability for the De Vita et al. and the OMERACT score was substantial with Light's Kappa of 0.75 and 0.77, respectively. Furthermore, no significant difference was noticed among sonographers with different levels of experience. Conclusion: The two tested SGUS scores are reliable for the evaluation of major salivary glands in pSS, and even less-expert sonographers could be reliable if adequately instructed.

Multicolor flow cytometric analysis of TLR2 and TLR9 expression and function in NK cells from patients with ANCA-associated vasculitis.

BACKGROUND: The primary objective of this study was to provide an assessment of NK cells in patients with ANCA-associated vasculitis (AAV). METHODS: Patients were classified based on the presence or absence of ANCAs and compared with healthy controls (HCs). By multiparameter flow cytometry, we evaluated the number and proportion of NK cells (CD3-CD56+) and the CD56dim , CD56bright , CD56dim CD57bright subsets; TLR2 and TLR9 expression; intracellular IFN-γ production upon stimulation with TLR2 and TLR9 ligands; degranulation activity; serum cytokines; immunohistochemical staining of available biopsies. RESULTS: No differences in the number and proportion of NK cells between patients and HC were observed, except for a lower proportion of CD56dim subset in ANCA-negative patients than in HC (P = 0.02). With respect to HC, TLR2 expression levels were reduced in NK cells from ANCA-negative patients (P = 0.03), in the CD56dim subset of ANCA-positive (P = 0.02) and ANCA-negative patients (P = 0.01), in the CD56bright subset of ANCA-positive patients (P = 0.007), and in the CD56dim CD57bright subset of ANCA-positive (P = 0.04) and ANCA-negative patients (P = 0.03). No differences between patients and HC were found concerning IFN-γ production and degranulation activity. IL-22 levels were lower in ANCA-positive patients than in HC (P = 0.01). The immunohistochemical analysis showed sporadic CD56+ cells in one renal biopsy, and a diffuse and moderate infiltrate of IL-22+ cells in all renal biopsies and in skin tissue. CONCLUSIONS: Our data suggest a role of infectious stimuli triggering NK cells in AAV pathogenesis. Poor detection of NK cells in affected tissues suggests a marginal involvement in local inflammatory responses. © 2017 International Clinical Cytometry Society.

Myositis in primary Sjögren's syndrome: data from a multicentre cohort.

OBJECTIVES: In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. METHODS: Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. RESULTS: In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was performed in 13/17 (76.4%) cases with histologically confirmed myositis in 6/13 (46.1%) (1"IBM-like"-5"PM-like"). In two "PM-like" cases, several fibres showed a decreased histochemical cytochrome C oxidase (COX) stain. Two biopsies tested "negative", four showed "non-specific" findings. All patients were treated with corticosteroids followed by different DMARDs. CONCLUSIONS: Our retrospective analysis shows a prevalence of myositis in pSS lower than previously reported, mainly appearing as an overlapping syndrome. Histological findings confirm the possible presence of an IBM or of a myopathy more similar to PM with a decreased COX activity. Classical immunosuppressants are effective although in most difficult cases IVIg or RTX may be used with benefit.

The use of musculoskeletal ultrasound in a rheumatology outpatient clinic.

INTRODUCTION: Musculoskeletal ultrasound (US) represents a valid, reliable and sensitive-to-change tool for the evaluation of patients suffering from rheumatic conditions. This method demonstrates a wide applicability and availability, finding place in the clinical practice in rheumatology outpatient clinic. AIM: To perform an epidemiological evaluation related to the use of US in a university rheumatology outpatient clinic. MATERIAL AND METHODS: During a 3-month period, data concerning consecutive patients attending to the US Unit of Department of Rheumatology, Sapienza University of Rome were registered. We collected the demographic data, the diagnosis, the reason for the US examination, the examined joints, as well as the requesting physicians' specialty. RESULTS: In the period October-December 2013, 572 patients (M/F 137/435; mean age+/-SD 55.2+/-15.8 years) were registered. The US examination was more frequently requested for the following diseases: rheumatoid arthritis (29.5%), osteoarthritis (10.6%), spondyloarthritis (9.1%), and connective tissue diseases (8.9%). In 239 of cases (41.8%), the US evaluation was requested for other indications. The US evaluation was requested slightly more frequently for monitoring (55.7%) compared to diagnosis (44.3%). The requesting physician was a rheumatologist in the majority of the cases (80.6%). The most frequent requested were the hand joints (28.9%) and wrists (23.3%). CONCLUSIONS: US examinations are most frequently used in the evaluation of patients with rheumatoid arthritis and mainly to monitor the disease. The exam is requested mostly by rheumatologists. The hand joints and wrists were the most frequently evaluated.

Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients.

OBJECTIVE: The aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients' serological profile on disease severity and on immunosuppressive drug utilization. METHODS: Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression. RESULTS: The cohort included 1115 (1067 female, 48 male) pSS patients. Severe extraglandular manifestations were detectable in 15% of the patients and were represented by active synovitis (11%), axonal sensory-motor neuropathy (2%), severe leucocytopenia (14%), cutaneous vasculitis (6%) and non-Hodgkin's lymphoma (4.5%). We found that low C3/C4, hypergammaglobulinaemia, RF and cryoglobulinaemia were markers of severity for pSS. According to the number of serological variables, the patients were subdivided into three distinct groups: favourable (no serological markers), intermediate (one serological marker) and poor (two or more serological markers). In comparison with the other two patient groups, pSS patients presenting with two or more adverse determinants had a higher frequency of severe visceral disease complications and required more aggressive therapeutic interventions. CONCLUSION: This study confirmed that the prevalence of the pSS high-risk subset for severe systemic manifestations is ∼15%. Serological markers might help in the early identification of patients who are candidates to receive more aggressive treatments.

The Italian MSUS Study Group recommendations for the format and content of the report and documentation in musculoskeletal ultrasonography in rheumatology.

OBJECTIVE: The objective of this study was to draw up a set of recommendations for the format and content of the musculoskeletal ultrasonography (MSUS) report in rheumatology. METHODS: A panel of rheumatologists, members of the MSUS Study Group of the Italian Society of Rheumatology, met in order to identify the main discrepancies in the MSUS report. A set of 15 recommendations was then defined, aimed at resolving the main discrepancies. They consisted of information about the motivations for the MSUS examination, the equipment, the US modalities and scanning technique, a list of the examined structures and findings, the scoring/grading systems, the number of images and main findings to include and conclusions. Subsequently a Delphi-based procedure was started in order to obtain agreement on a core set of recommendations. Consensus for each recommendation was considered achieved when the percentage of agreement was >75%. RESULTS: Three complete rounds were performed. The response rate was 85.2% for the first round, 78.3% for the second and 88.9% for the third. Finally, consensus was obtained for 14 of 15 statements. These 14 statements represent the recommendations of the group for the format and content of the report and documentation in MSUS in rheumatology. CONCLUSION: To the best of our knowledge, our group has produced the first recommendations for the format and content of the report and documentation in MSUS in rheumatology. The report is an integral part of the MSUS examination and its use in a homogeneous form can help in the correct interpretation of the findings.

Ultrasound in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by synovial inflammation that can lead to structural damage of cartilage, bone and tendons. Assessing the inflammatory activity and the severity is essential in RA to help rheumatologists in adopting proper therapeutic strategies and in evaluating disease outcome and response to treatment. In the last years musculoskeletal (MS) ultrasonography (US) underwent tremendous technological development of equipment with increased sensitivity in detecting a wide set of joint and soft tissues abnormalities. In RA MSUS with the use of Doppler modalities is a useful imaging tool to depict inflammatory abnormalities (i.e. synovitis, tenosynovitis and bursitis) and structural changes (i.e. bone erosions, cartilage damage and tendon lesions). In addition, MSUS has been demonstrated to be able to monitor the response to different therapies in RA to guide local diagnostic and therapeutic procedures such as biopsy, fluid aspirations and injections. Future applications based on the development of new tools may improve the role of MSUS in RA.

Outcome of pregnancy in Italian patients with primary Sjögren syndrome.

OBJECTIVE: To investigate pregnancy and fetal outcomes in patients with primary Sjögren syndrome (pSS). METHODS: An obstetric history of 36 women with established diagnosis of pSS at pregnancy was obtained from a multicenter cohort of 1075 patients. In a subgroup case-control analysis, 12 deliveries in patients with pSS were compared with 96 control deliveries. RESULTS: Thirty-six women (31 with anti-SSA/Ro and/or anti-SSB/La antibodies) with an established diagnosis of pSS had 45 pregnancies with the delivery of 40 newborns. Two miscarriages, 2 fetal deaths, and 1 induced abortion were recorded. Mean age at the first pregnancy was 33.9 years; mean number of pregnancies was 1.25; 18/40 (45%) cesarean births were delivered; mean pregnancy length was 38.5 weeks (range 32-43), with 6 preterm deliveries. The mean Apgar score at 5 min was 8.9, mean birthweight was 2920 g (range 826-4060 g). Congenital heart block (CHB) occurred in 2/40 (5%) newborns. The reported rate of breastfeeding for at least 1 month was 60.5%. In 4/40 pregnancies (10%) a flare of disease activity was observed within a year from delivery. In the case-control subgroup analysis, 12 deliveries were compared with 96 controls and no significant differences were found. CONCLUSION: Patients with pSS can have successful pregnancies, which might be followed by a mild relapse. CHB was the only cause of death for offspring of mothers with pSS.

Osteoarthritis of the foot: a review of the current state of knowledge.

Foot osteoarthritis(OA)is very common disease that mainly affects older people deeply influencing their quality of life.The join most frequently involved in the articular degenerative process is the first metatarsophalangeal joint. Its severe impairment may lead to a specific clinical pattern known as hallux rigidus that often requires surgical treatment.Currently conventional radiograms of feet associated with an accurate clinical examination should be performed in order to diagnose foot OA.However, new imaging modalities as ultrasonography and magnetic resonance imaging are emerging as valuable tools to assess foot OA.Therapeutic options for foot OA consist of conservative strategies, including life-style modification and pharmacological treatment, options that are usually adopted in early-stage disease and in invasive surgical procedures reserved to late-stage conditions. At the present time there is a lack of evidence in international literature specifically dealing with foot OA, so further investigation on this topic is required to clarify its pathogenesis, the diagnostic pathway and the best clinical management.