Kinetics of response to iloprost evaluated by laser speckle contrast analysis in systemic sclerosis.

OBJECTIVE: Iloprost (ILO) is recommended for the treatment of systemic sclerosis (SSc) microangiopathy, but there is no common consensus on its optimal dosage. The aim of this study is to evaluate the kinetics of response to ILO administered in a daily outpatient scheme in SSc subjects using laser speckle contrast analysis (LASCA). METHOD: Adult SSc patients in stable therapy with ILO administered for 6 h for 2 consecutive days every 4 weeks were enrolled. Peripheral finger perfusion was assessed by LASCA. Each patient underwent five LASCA evaluations: before and after each day of ILO (D1pre, D1post, D2pre, and D2post) and after 4 weeks (D30). RESULTS: Twenty-seven SSc patients (77.8% female, mean age 61.5 years) were enrolled. LASCA showed an increase in perfusion at the end of each ILO course, but on the second day (both D1pre vs D2pre and D2pre vs D2post) the increase was no longer significant in half of the fingers. Moreover, compared to D1post, at the beginning of the second ILO day most of the fingers had already shown a significant reduction in perfusion. After 1 month, there were no statistically significant differences between the perfusion values of D1pre and D30. CONCLUSION: This LASCA study highlights the transience of the vasoactive effect of ILO, with a perfusion benefit that is completely lost after 1 month. The brevity of the perfusion effect of ILO and the use of LASCA are elements to consider in the design of future SSc trials to determine the optimal ILO dosage.

Sleep quality in Behçet's disease: a systematic literature review.

Behçet's Disease (BD) can be correlated with sleep impairment and fatigue, resulting in low quality of life (QoL); however, a comprehensive evaluation of this issue is still missing. We performed a systematic literature review (SLR) of existing evidence in literature regarding sleep quality in BD. Fifteen papers were included in the SLR. Two domains were mainly considered: global sleep characteristics (i) and the identification of specific sleep disorders (ii) in BD patients. From our analysis, it was found that patients affected by BD scored significantly higher Pittsburgh Sleep Quality Index (PSQI) compared to controls. Four papers out of 15 (27%) studied the relationship between sleep disturbance in BD and disease activity and with regards to disease activity measures, BD-Current Activity Form was adopted in all papers, followed by Behçet's Disease Severity (BDS) score, genital ulcer severity score and oral ulcer severity score. Poor sleep quality showed a positive correlation with active disease in 3 out of 4 studies. Six papers reported significant differences between BD patients with and without sleep disturbances regarding specific disease manifestations. Notably, arthritis and genital ulcers were found to be more severe when the PSQI score increased. Our work demonstrated lower quality of sleep in BD patients when compared to the general population, both as altered sleep parameters and higher incidence of specific sleep disorders. A global clinical patient evaluation should thereby include sleep assessment through the creation and adoption of disease-specific and accessible tests.

Hallux valgus associated to osteoarthritis: Clinical-radiological outcomes of modified SERI technique at mid- to long-term follow-up. A retrospective analysis.

INTRODUCTION: Treatment of hallux valgus deformity associated with mild or moderate osteoarthritis (OA) is still a topic of debate. In the literature, there are few studies concerning the management of patients affected by this condition. This study aims to report the experience at mid- to long-term results of an original joint-preserving surgical technique. MATERIALS AND METHODS: Patients affected by mild to moderate hallux valgus deformity and associated to grade 1-2 OA and treated with modified Simple-Effective-Rapid-Inexpensive (SERI) technique from 2008 to 2018 were selected. Inclusion criteria were mild or moderate hallux valgus angle (HVA) <40° and an intermetatarsal angle (IMA) <20° and associated grade 1-2 OA of the first metatarso-phalangeal joint (MTPJ). RESULTS: 128 feet in 120 consecutive patients, undergone modified SERI procedure, have been retrospectively reviewed at a mean follow-up of 5.1 ± 3.8 years (range 2-11). American Orthopaedics Foot Ankle Society (AOFAS) score that was significantly improved from 44.2 ± 13.2 to 88.2 ± 9.6. Pre-operative average HVA and IMA values decreased respectively from 31.6° ± 3.9° to 9.1° ± 4.4° and from 16.2° ± 3.8° to 7.2° ± 3.1°. The average distal metatarsal articular angle (DMAA) value improved from 28.2° ± 6.5° to 7.1° ± 6°. OA of the first MTPJ highlighted a grade 1 in 46 feet and a grade 2 in 82 feet pre-operatively and a grade 0 in 30 feet, grade 1 in 82 feet, and grade 2 in 16 feet at the final follow-up. CONCLUSIONS: The modifications to the SERI technique could extend the indications to patients affected by hallux valgus with mild to moderate OA. The wider case series and the longer follow-up of this study make us believe this technique is very useful for improving the quality of life in these patients. LEVEL OF EVIDENCE: IV.

Prostanoids in scleroderma microangiopathy: clinical and pharmacoeconomic comparison between two intravenous regimens.

Objectives: Intravenous iloprost (ILO) has widely demonstrated its effectiveness and safety in systemic sclerosis (SSc) patients. Unfortunately, there is no clear consent about dosage, duration, frequency, and infusion modality. The aim of this study was to compare two different therapeutic schemes in the same cohort of consecutive SSc subjects, evaluating differences in terms of effectiveness [digital ulcer (DU) outcome], safety, and direct healthcare costs.Method: This was a retrospective observational study of 47 patients classified with SSc treated with intravenous ILO for severe Raynaud's phenomenon and/or DUs. Two regimens were compared: a continuous inpatient scheme and a daily outpatient scheme. Demographics and clinical data, concomitant therapies, adverse events, and data on resource use and costs were collected.Results: The number of DUs rose slightly with the switch from the continuous to the daily scheme (0.61 ± 1.2 vs 1.1 ± 1.7). Moreover, in the daily scheme there was an increase in the number of therapeutic cycles (2.4 ± 0.7 vs 4.71 ± 1.4, p < 0.001) and an increase in patients treated with other vasoactive drugs. There was a reduction in ILO tolerability and more than half of the patients suspended the treatment. Five patients required hospitalization for severe and refractory DUs in the daily scheme. Moreover, the costs of the two treatments were comparable [median 7174 (range 2748-18 524) EUR vs 6284 (3232-22 706) EUR, p = 0.712].Conclusion: Treatment with a daily scheme of ILO is characterized by worse tolerability and a higher dropout rate compared to a low-flow regimen, with similar costs. We suggest that a low-flow continuous therapeutic scheme is preferable in SSc patients.

A systematic literature review of existing tools used to assess medication adherence in connective tissue diseases: the state of the art for the future development of co-designed measurement tools.

Lack of medication adherence is frequent in chronic connective tissue diseases and is associated with poorer health outcomes, low quality of life and economic loss. This research is based on a systematic literature search and aims to identify the surveys and tools used for the assessment of medication adherence in patients with connective tissue diseases (CTDs) and in particular the tools co-designed with patients. A systematic literature review was performed in PubMed and Embase databases searching for studies concerning the application of surveys or tools designed for medication adherence assessment. A specific analysis was also performed to identify which of these existing tools were developed in co-design with patients affected by CTDs. 1958 references were identified, and 31 studies were finally included. Systemic lupus erythematosus was the most investigated disease, followed by the Behçet's disease. The tools used to assess adherence in CTDs were, in most cases, valid and useful. However, the results showed a certain degree of heterogeneity among the studies and the medication adherence assessment and measurement tools adopted, which were mostly based on selfreported questionnaire. No co-designed tools with patients were found. Low- and non-adherence were explored in some CTDs with valid and useful tools, while other CTDs still need to be assessed. Therefore, more efforts should be made to better understand the specific reasons for the low- and non-adherence in CTDs patients.

[Tuberous breast deformity correction: Long-term satisfaction assessment with BREAST-Q questionnaire]. / Correction des seins tubéreux : évaluation de la satisfaction à long terme à l'aide du BREAST-Q.

BACKGROUND: Tuberous Breast Deformity (TBD) is a complex breast malformation: shape, size and symmetry of breast can be affected. It causes physical and mental suffering with significant effect on life quality. The purpose of this study is to assess patients satisfaction and patients quality of life after TBD surgery over time. METHODS: All TBD patients operated between January 2007 and December 2018 were retrospectively identified for the study and those treated whith implant and/or mammoplasty were included. Different parameters have been recorded: age, malformation severity, breast symmetry, BMI, pregnancies, breast-feeding, type of primary surgery, complications and number of re-operations. Long-term satisfaction was assessed thanks to a BREAST-Q questionnaire (with a special « augmentation ¼ or « reduction/mastopexy ¼ module according to the primary surgery). RESULTS: Eighty-two patients were included: 35 patients had recieved bilateral breast implants, 14 patients had received unilateral breast implant with or without collateral mammoplasty, and 33 patients had undergone breast reduction surgery. The total average for the medical follow-up was 7.4 years. The number of intervention was significantly higher for patients who had undergone breast augmentation surgery (P=0.001) and for patients with severe TBD (P=0.01). Forty patients replied to the BREAST-Q questionnaire. Patients satisfaction scores were not significantly different between the different groups. Regarding life quality scores, patients undergoing a breast augmentation surgery with bilateral implants seemed to have a better "sexual well-being" score (P=0.03). "Physical well-being" score was lower for patients who had a breast reduction compared to the other groups (P=0.01). Patients with breast implants had significant better quality of life scores, especially for the following parameters: "psychosocial well-being" (P=0.02), "sexual well-being" (P<0.001), "physical well-being" (P<0.001) and "satisfaction with breast" (P=0.03). CONCLUSIONS: TBD surgery basically provides long-term satisfaction for most of the patients. The number of re-operations does not seem to deteriorate satisfaction over time.

[Abdominoplasties: Do we still need to drain in 2021?] / Abdominoplasties : faut-il encore les drainer en 2021 ?

OBJECTIVE: The purpose of this study was to consider the use of drainage when performing an abdominoplasty with regards to postoperative complications for two groups of patients. PATIENTS AND METHOD: From January 1st 2017 to December 31th 2019, 215 patients underwent an abdominoplasty in our institution. In this retrospective, comparative, single institution study, patients were divided into two groups: "drainage" D (n=162) when suction completed abdominoplasty, "no drainage" ND (n=53) when suction didn't completed abdominoplasty. Early and distant complications were retrieved for each group and compared. RESULTS: There was no significant difference between the two groups concerning the occurrence of seroma postoperatively (8% of patients in group D and 11.3% of patients in group AD). The drainage group D experienced more seroma's punctures (2,3± 1,0) and the mean of punctured fluid was higher (386,5ml±350,4ml) compared to the no drainage group ND (1,3+- 0,5 number of punctures with a mean punctured fluid of 165,8mL± 224,2mL). The mean hospital stay was shorter for group ND (2,9± 1,8 days) than for group D (4,4+- 1,7 days), P<0,0001. CONCLUSION: Performing an abdominoplasty with quilting suture but drainless doesn't seem to increase postoperative complications statistically. The authors recommend, under the guise of a quilting suture, not to systematically drain the abdominoplasties and to reserve this technique for patients at risk of complications (high BMI, significant weight loss and co-morbidities).

[Use of coupler for venous anastomoses in cervico-facial reconstruction. Retrospective study on 51 free flaps]. / Utilisation du coupleur pour les anastomoses veineuses en reconstruction cervico-faciale. Étude rétrospective sur 51 lambeaux libres.

OBJECTIVE: The aim of our study was to evaluate the interest of the mechanical anastomosic coupling device for venous anastomoses in a series of cervico-facial reconstructions after carcinologic excision. PATIENTS AND METHODS: Between January 2010 and December 2017, 46 patients underwent free flap cervico-facial reconstruction. We performed 54 venous anastomoses for 51 free flaps. Mechanical venous anastomoses were performed with a coupler (Coupler®). Thrombotic complications and choice of recipient vessels were assessed for the entire series. RESULTS: Venous anastomoses were performed using a coupler in 33.3% of the flaps (n=18). Mean operative time was lower for reconstructions with mechanical coupler anastomoses, and significantly for fibula flaps. Of eight thromboses, six were venous, two of which after the coupler anastomosis. The thrombosed veins were the branches of the internal jugular vein and the anterior jugular veins. The diameter of the couplers used was respectively 2.5mm and 2mm. CONCLUSION: The use of the microvascular coupling system for venous anastomoeis would help reduce the occurrence of venous thrombosis, but larger and prospective studies should be conducted. This coupling system, relatively easy to use, would overcome the lack of congruence of anastomosing veins and reduce the risk of intimal injury. Regarding the recipient vessels, the anterior jugular vein should not be used as first line because the risk of vascular complications seems more important.

Late-onset biopsy-proven lupus nephritis without other associated autoimmune diseases: severity and long-term outcome.

BACKGROUND/PURPOSE: Lupus nephritis (LN) usually develops within the first years of systemic lupus erythematosus (SLE) onset and rarely after that. There are scarce studies comparing early- versus late-onset nephritis (before versus after five years of SLE diagnosis). The aim of this study was to compare the severity and long-term outcome (after 7 years) in these two, late-onset and early-onset, nephritis groups. METHODS: This study included 93 patients from rheumatology tertiary centers from Brazil and Italy, all of them with biopsy-proven LN with > 7 years follow-up. Patients were divided in two groups: early-onset nephritis ( n = 75) and late-onset nephritis ( n = 18). Clinical and laboratorial data were obtained using a standardized electronic chart database protocol carried out at 1-6 months interval and established in 2000. Patients >50 years or with concomitant autoimmune diseases were excluded. Variables evaluated at the LN presentation were Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), creatinine, albumin, anti-DNA positivity and nephritis class. Variables evaluated at the long-term outcome (after 7 years) were Systemic Lupus International Collaborating Clinics Damage Index (SDI), creatinine, dialysis and mortality. RESULTS: The average time of LN presentation was 10.94 ± 3.73 years for the late-onset and 1.20 ± 1.60 years for the early-onset group. Their similar nephritis duration (12.44 ± 3.2 versus 13.28 ± 4.03 years, p = 0.41) and comparable mean ages (49.17 ± 9.9 versus 44.11 ± 10.8 years old, p = 0.06) allow a more accurate comparison. Regarding severity, late-onset was similar to early-onset group: SLEDAI (8 (range: 6-22) versus 12 (range: 2-24), p = 0.47), creatinine (1.36 ± 0.94 versus 1.36 ± 1.13 mg/dl, p = 0.99); albumin (2.84 ± 0.65 versus 2.59 ± 0.84 mg/dl, p = 0.30); proteinuria (3.77 ± 2.18 versus 5.01 ± 4.51 g/vol, p = 0.26); proliferative nephritis (44% ( n = 8) versus 60% ( n = 45), p = 0.23). There was also no difference in the long-term outcomes between groups: SDI (1 (range: 0-5) versus 0.5 (range: 0-5), p = 0.27); creatinine (2.04 ± 2.38 versus 1.69 ± 2.26 mg/dl, p = 0.56); dialysis (22% ( n = 4) versus 13% ( n = 10), p = 0.46) and mortality (0% ( n = 0) versus 12% ( n = 9), p = 0.19). CONCLUSION: This study provides novel evidence of comparable long-term outcomes between late-onset and early-onset nephritis, which is most likely explained by the observation that at presentation, the clinical, laboratorial and histological features of late-onset and early-onset nephritis are similar. This suggests that there should be no distinct treatment targets and therapeutic interventions for the late- and early-onset groups.

Translation, cultural adaptation and validation of the Systemic Lupus Erythematosus Activity Questionnaire (SLAQ) in a cohort of Italian systemic lupus erythematosus patients.

Introduction The Systemic Lupus Erythematosus Activity Questionnaire (SLAQ) is a patient-reported instrument for the assessment of disease activity in systemic lupus erythematosus (SLE). The aims of the present study are translation, cultural adaptation and validation of an Italian version: the SLAQit. Methods The process of translation and cultural adaptation followed published guidelines. SLAQit was pretested in a group of 35 SLE patients to evaluate acceptability, comprehension and feasibility. Internal consistency, test-retest validity and external validity were tested on consecutive SLE patients attending the clinic. Results In total, 135 SLE patients were enrolled in this study. The pilot test provided a 99.9% response rate and demonstrated feasibility and comprehensibility of the questionnaire. A good internal consistency was found among the three components of the score (SLAQ score, numerical rating scale (NRS), patient global assessment question (PGA); α = 0.79). SLAQit showed very high reliability (test-retest α > 0.8). NRS and PGA showed a strong positive correlation with both Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) ( p = 0.002 and p < 0.001, respectively) and European Consensus Lupus Measurement (ECLAM) scores ( p = 0.01 and p < 0.001, respectively), while the SLAQ score did not. A significant agreement was observed between the physician's intention to treat and both the NRS and PGA scores, while no significant association was reported with the SLAQ score. Conclusions SLAQit was demonstrated to be a reliable and valid instrument for self-assessment of disease activity in SLE patients.

Early Lupus Project: one-year follow-up of an Italian cohort of patients with systemic lupus erythematosus of recent onset.

Objective To describe the clinical and serological features of a prospectively followed cohort of early diagnosed systemic lupus erythematosus (SLE) patients during a one-year follow-up period. Methods SLE patients with disease duration less than 12 months were consecutively enrolled in a multicentre, prospective study. At study entry and then every 6 months, a large panel of data was recorded. Results Of 260 patients enrolled, 185 had at least 12 months of follow-up; of these, 84.3% were female, 92.4% were Caucasians. Mean diagnostic delay was about 20 months; higher values of European Consensus Lupus Activity Measurement (ECLAM) and of organs/systems involved were both associated with shorter diagnostic delay. Clinical and serological parameters improved after study entry. However, patients' quality of life deteriorated and cardiovascular risk factors significantly increased. About one-third of patients with active disease at study entry went into remission (ECLAM = 0). Negative predictors for remission were: oral ulcers, arthritis, low C4, anti-SSB (Ro) antibodies and therapy with mycophenolate. There was a widespread use of glucocorticoids both at baseline and during follow-up. Conclusion Clinical symptoms and serological parameters improve during the first period after diagnosis. However, patients' quality of life deteriorates. The widespread use of glucocorticoids is probably the reason for the early significant increase of some cardiovascular risk factors.

Histopathology of the muscle in rheumatic diseases.

The presence of muscular symptoms is common in rheumatological clinical practice, but often the differential diagnosis between muscular involvement in connective tissue diseases, vasculitis and drug-induced myopathy may be difficult. In addition to clinical assessment, laboratory analysis and instrumental examinations, muscle biopsy may help to clarify the diagnosis in patients with muscular involvement. The purpose of this review is to provide a critical analysis of the current medical literature on muscular histopathology, to help clinicians to identify when to perform muscular biopsy and to provide a practical guide to a better understanding of the pathology report. Moreover, we provide an overview of the muscular involvement and the most common histopathological findings in rheumatic diseases.

The use of rituximab in idiopathic inflammatory myopathies: description of a monocentric cohort and review of the literature.

Rituximab (RTX), a chimeric monoclonal antibody targeted against CD20, has been used to treat refractory inflammatory myopathies (IIM). The primary objective of this study was to retrospectively assess the efficacy of RTX in reducing disease activity in patients with IIM refractory to conventional therapy. Secondary aim was the evaluation of adverse events (AE) during the treatment period. We examined 26 patients with a diagnosis of IIM, referred to our Rheumatology Unit and treated with RTX for active refractory disease. Patients were treated with RTX 1000 mg i.v., twice, with a 2-week interval. RTX treatment was associated with a significant reduction of creatine kinase (p=0.001) after six months compared to the baseline, an improved muscular strength measured with MMT8 (p<0.001) and a reduction of the extramuscular activity of the disease measured with MYOACT (p<0.001). In particular, RTX improved DM skin rash, arthritis and pulmonary manifestations. Autoantibody positivity (in particular antisynthetase, anti- SRP and antiRo/SSA), and a disease duration <36 months at the moment of the treatment are associated with a better response rate. Treatment with RTX was also associated with a reduction of the mean daily dose of steroids needed to control disease activity (p=0.002). Our results have confirmed that RTX is efficacious in the treatment of refractory IIM. Ad hoc controlled trials are needed to better clarify the specific subset of patients who may better respond to the treatment and the optimal therapeutic schedule.

EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome.

OBJECTIVES: Develop recommendations for women's health issues and family planning in systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). METHODS: Systematic review of evidence followed by modified Delphi method to compile questions, elicit expert opinions and reach consensus. RESULTS: Family planning should be discussed as early as possible after diagnosis. Most women can have successful pregnancies and measures can be taken to reduce the risks of adverse maternal or fetal outcomes. Risk stratification includes disease activity, autoantibody profile, previous vascular and pregnancy morbidity, hypertension and the use of drugs (emphasis on benefits from hydroxychloroquine and antiplatelets/anticoagulants). Hormonal contraception and menopause replacement therapy can be used in patients with stable/inactive disease and low risk of thrombosis. Fertility preservation with gonadotropin-releasing hormone analogues should be considered prior to the use of alkylating agents. Assisted reproduction techniques can be safely used in patients with stable/inactive disease; patients with positive antiphospholipid antibodies/APS should receive anticoagulation and/or low-dose aspirin. Assessment of disease activity, renal function and serological markers is important for diagnosing disease flares and monitoring for obstetrical adverse outcomes. Fetal monitoring includes Doppler ultrasonography and fetal biometry, particularly in the third trimester, to screen for placental insufficiency and small for gestational age fetuses. Screening for gynaecological malignancies is similar to the general population, with increased vigilance for cervical premalignant lesions if exposed to immunosuppressive drugs. Human papillomavirus immunisation can be used in women with stable/inactive disease. CONCLUSIONS: Recommendations for women's health issues in SLE and/or APS were developed using an evidence-based approach followed by expert consensus.

Kinematics of ACL and anterolateral ligament. Part II: anterolateral and anterior cruciate ligament reconstruction.

PURPOSE: To quantify the influence of Single-Bundle with Lateral Plasty and Double-Bundle reconstruction on static and dynamic laxity in combined ACL- and ALL-deficient knees. METHODS: The study included 10 fresh-frozen human knees. The joints were analyzed in the following conditions: ACL + ALL resection, Single-Bundle with Lateral Plasty (SBLP) reconstruction, Double-Bundle (DB) reconstruction. Testing parameters were: anterior displacement at 30° and 90° of flexion (AP30, AP90) applying a manual maximum load; internal rotation at 30° and 90° of flexion (INT30, INT90) applying a 5 Nm torque and acceleration and internal rotation Pivot-Shift (PS) test. Kinematics was acquired by a navigation system. Paired Student's t test was conducted to assess statistical difference (P < 0.05). RESULTS: At both 30° and 90° of knee flexion, both SBLP and DB surgical techniques showed a significant reduction (P < 0.01) of anterior-posterior tibial displacement compared to the resection of ACL + ALL. At 30° on knee flexion it is the SBLP that allows the greatest reduction of internal rotational laxity when compared to DB reconstruction. Concerning the PS test, only SBPL procedure had a significant laxity decrease considering the acceleration reached by the joint when compared with the ACL + ALL state (P < 0.01). CONCLUSION: Clinical relevance of this study is that the internal rotation and PS test were more efficiently controlled by the SBLP technique than by the DB one at both 30° and 90° of flexion in case of ACL + ALL lesions.

Bioelectrical impedance vector analysis in patients with irritable bowel syndrome on a low FODMAP diet: a pilot study.

BACKGROUND: The aim of this study was to determine the effects of a low fermentable oligo-, di- and monosaccharides and polyols (FODMAP) diet on the nutritional status and body composition, abdominal symptoms, quality of life, anxiety/depression and sleep quality of patients with irritable bowel syndrome (IBS). METHODS: Consecutive patients were given a low FODMAP diet for 8 weeks. At baseline and after 8 weeks, blood tests were taken to evaluate nutritional status and a bioelectrical impedance analysis was performed to assess body composition. Anthropometric data, IBS Symptom Severity Score, results of a bowel habits questionnaire, Bristol Stool Chart classification, SF36, Hamilton Depression Anxiety Scale outcome and Pittsburgh Sleep Quality Index were also recorded. During the 8-week diet period, the patients were phoned periodically by the nutritionist to verify their compliance. RESULTS: Twenty-six IBS patients with a mean age of 46.2 ± 13.8 years were studied. After 8 weeks, there were no abnormalities in anthropometric data, bioelectrical impedance parameters and blood tests. The patients' IBS Symptom Severity Score improved (305.2 ± 84.1 vs 156.3 ± 106.4; p < 0.0001), as did bowel habits, Bristol Stool Chart classification, quality of life and HADS anxiety score, whereas sleeping quality and depression were unchanged. The degree of relief from symptoms and satisfaction with the diet was high. CONCLUSIONS: A low FODMAP diet improved IBS symptoms without effects on nutritional status and body composition.

Insomnia symptoms, perceived stress and coping strategies in patients with systemic lupus erythematosus.

OBJECTIVE: The aim of this study is to evaluate perceived stress and coping strategies in individuals with systemic lupus erythematosus (SLE) according to the presence of insomnia symptoms, using a set of variables that include anxiety and depressive symptoms evaluation. METHODS: Ninety SLE women were evaluated in a cross-sectional study using the Perceived Stress Scale (PSS), Brief COPE, Pittsburgh Sleep Quality Index (PSQI), Insomnia Severity Index (ISI), Beck Depression Inventory (BDI) and Self-rating Anxiety Scale (SAS). RESULTS: Individuals with insomnia symptoms (n = 57, 66%) presented higher PSS (p < 0.001), PSQI (p < 0.0001), BDI, (p < 0.0001) scores and showed less-effective coping strategies such as the use of behavioral disengagement (p = 0.04), self-blame (p = 0.02) and emotional-focused coping (p = 0.001). In a multi-regression model ISI was the independent determinant of high PSS and of behavioral disengagement; PSQI was the only determinant of self-blame (p = 0.02) and emotional-focused coping. CONCLUSIONS: SLE individuals with insomnia symptoms show high levels of perceived stress and more frequent use of disengaging and emotional-focused coping strategies. This body of evidence suggests that individuals with SLE and comorbid insomnia symptoms may therefore require additional interventions for insomnia.

Understanding remission in real-world lupus patients across five European countries.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease associated with increased mortality and significant personal, psychological and socioeconomic consequences. An agreed definition of remission is needed and lacking. We sought to visualize 'remission in SLE' in European patients considered by their physicians to be 'in remission' by comparing the reported symptom burden as reported by treating physicians for patients considered to be 'in remission' and those not considered to be 'in remission'. Data for 1227 patients drawn from a multinational, real-world survey of patients with SLE consulting practising rheumatologists and nephrologists in France, Germany, Italy, Spain, and the UK show that physicians classed their patients as 'in remission' despite a considerable ongoing symptom burden and intensive immunosuppressive medication. Patients considered to be 'in remission' still had a mean of 2.68 current symptoms vs 5.48 for those considered to be not 'in remission' (p < 0.0001). The most common symptoms among those seen to be 'in remission' were joint symptoms, fatigue, pain, mucocutaneous involvement, haematological manifestations and kidney abnormalities. The current analysis highlights important ongoing disease activity, symptom burden and immunosuppressive medication in European patients with SLE considered by their treating physician to be 'in remission'. For a further improvement of outcome, there is an urgent need for an international consensus on the definitions for remission among patients with SLE.

Post-occlusive reactive hyperaemia (POHR) in systemic sclerosis: very early disease (VEDOSS) represents a separate entity compared to established disease.

OBJECTIVES: Vascular involvement is a key feature of systemic sclerosis (SSc). Vascular changes are central to the pathogenesis of the disease and the assessment of vascular involvement has a prognostic value. This assessment therefore has a pivotal role in the management of SSc patients. The aim of our study was to evaluate post-occlusive reactive hyperaemia (PORH) in consecutive SSc patients and to test whether a PORH test might be a useful tool for the early diagnosis of SSc. METHOD: Between April 2011 and April 2015, 60 consecutive SSc patients (mean age 56 ± 15 years, females:males = 18:1) were enrolled in the study. The patients were divided into those with full-blown SSc (n = 50) and those with very early diagnosis of SSc (VEDOSS) (n = 10) according to the literature. Laser speckle contrast analysis (LASCA) was used to assess PORH. RESULTS: A statistically significant difference was detected in the post-ischaemic hyperaemic peak flow between VEDOSS and established SSc (424% vs. 137%, p = 0.0011). PORH peak flow decreased according to the capillaroscopic pattern (early = 419%, active = 163%, late = 145%, p = 0.0027). Moreover, a correlation between capillary density and peak flow was revealed (rho = 0.33, p < 0.01). CONCLUSIONS: These data show a different pattern of vascular involvement in VEDOSS compared to established disease that mirrors capillaroscopic changes. Functional features of very early and established disease seem to be the physiological counterpart of abnormalities detected by capillaroscopy. The POHR test might be a useful aid for further characterization of vascular involvement in SSc. In particular, blunted POHR might prove a tool to separate pre-clinical from full-blown SSc.

Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients.

OBJECTIVES: To assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later. METHODS: In 1999, we started an observational study of 1000 APS patients from 13 European countries. All had medical histories documented when entered into the study and were followed prospectively during the ensuing 10 years. RESULTS: 53.1% of the patients had primary APS, 36.2% had APS associated with systemic lupus erythematosus and 10.7% APS associated with other diseases. Thrombotic events appeared in 166 (16.6%) patients during the first 5-year period and in 115 (14.4%) during the second 5-year period. The most common events were strokes, transient ischaemic attacks, deep vein thromboses and pulmonary embolism. 127 (15.5%) women became pregnant (188 pregnancies) and 72.9% of pregnancies succeeded in having one or more live births. The most common obstetric complication was early pregnancy loss (16.5% of the pregnancies). Intrauterine growth restriction (26.3% of the total live births) and prematurity (48.2%) were the most frequent fetal morbidities. 93 (9.3%) patients died and the most frequent causes of death were severe thrombosis (36.5%) and infections (26.9%). Nine (0.9%) cases of catastrophic APS occurred and 5 (55.6%) of them died. The survival probability at 10 years was 90.7%. CONCLUSIONS: Patients with APS still develop significant morbidity and mortality despite current treatment. It is imperative to increase the efforts in determining optimal prognostic markers and therapeutic measures to prevent these complications.