Clinical and Prognostic Factors in Patients with IgG4-Related Kidney Disease.

BACKGROUND: IgG4-related kidney disease is a major manifestation of IgG4-related disease, a systemic fibroinflammatory disorder. However, the clinical and prognostic kidney-related factors in patients with IgG4-related kidney disease are insufficiently defined. METHODS: We conducted an observational cohort study using data from 35 sites in two European countries. Clinical, biologic, imaging, and histopathologic data; treatment modalities; and outcomes were collected from medical records. Logistic regression was performed to identify the possible factors related to an eGFR ≤30 ml/min per 1.73 m 2 at the last follow-up. Cox proportional hazards model was performed to assess the factors associated with the risk of relapse. RESULTS: We studied 101 adult patients with IgG4-related disease with a median follow-up of 24 (11-58) months. Of these, 87 (86%) patients were male, and the median age was 68 (57-76) years. Eighty-three (82%) patients had IgG4-related kidney disease confirmed by kidney biopsy, with all biopsies showing tubulointerstitial involvement and 16 showing glomerular lesions. Ninety (89%) patients were treated with corticosteroids, and 18 (18%) patients received rituximab as first-line therapy. At the last follow-up, the eGFR was below 30 ml/min per 1.73 m 2 in 32% of patients; 34 (34%) patients experienced a relapse, while 12 (13%) patients had died. By Cox survival analysis, the number of organs involved (hazard ratio [HR], 1.26; 95% confidence interval [CI], 1.01 to 1.55) and low C3 and C4 concentrations (HR, 2.31; 95% CI, 1.10 to 4.85) were independently associated with a higher risk of relapse, whereas first-line therapy with rituximab was protective (HR, 0.22; 95% CI, 0.06 to 0.78). At their last follow-up, 19 (19%) patients had an eGFR ≤30 ml/min per 1.73 m 2 . Age (odd ratio [OR], 1.11; 95% CI, 1.03 to 1.20), peak serum creatinine (OR, 2.74; 95% CI, 1.71 to 5.47), and serum IgG4 level ≥5 g/L (OR, 4.46; 95% CI, 1.23 to 19.40) were independently predictive for severe CKD. CONCLUSIONS: IgG4-related kidney disease predominantly affected middle-aged men and manifested as tubulointerstitial nephritis with potential glomerular involvement. Complement consumption and the number of organs involved were associated with a higher relapse rate, whereas first-line therapy with rituximab was associated with lower relapse rate. Patients with high serum IgG4 concentrations (≥5 g/L) had more severe kidney disease.

The mediating effect of fatigue in impaired quality of life in systemic lupus erythematosus: mediation analysis of the French EQUAL cohort.

OBJECTIVES: Mediation analyses were conducted to measure the extent to which musculoskeletal (MSK) flares and depression affected physical health through excessive fatigue. METHODS: Mediation analyses were performed in a large multicentre cohort of SLE patients. Domains of the LupusQoL and SLEQOL questionnaires were selected as outcomes, MSK flares according to the SELENA-SLEDAI flare index (SFI-R) score and depression defined by Center for Epidemiologic Studies-Depression scale (CES-D) scale as exposures and different fatigue domains from MFI-20 and LupusQoL questionnaires as mediators. For each model, total, direct, indirect effects and proportion of effect mediated by fatigue (i.e. proportion of change in health-related quality of life) were determined. RESULTS: Of the 336 patients, 94 (28%) had MSK flares at inclusion and 99 (29.5%) were considered with depression. The proportion of the total effect of MSK flares on physical health impairment explained by fatigue ranged from 59.6% to 78% using the LupusQOL 'Physical health' domain and from 51.1% to 73.7% using the SLEQOL 'Physical functioning' domain, depending on the fatigue domain selected. The proportion of the total effect of depression on physical health impairment explained by fatigue ranged from 68.8% to 87.6% using the LupusQOL 'Physical health' domain and from 79.3% to 103.2% using the SLEQOL 'Physical functioning' domain, depending on the fatigue domain selected. CONCLUSIONS: The effect of MSK flares and depression on physical health impairment is largely mediated by fatigue. Thus, the patient's perception of disease activity as measured by physical health is largely influenced by fatigue. In addition, fatigue has a significant negative impact on quality of lifeof SLE patients with depression. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov, NCT01904812.

Severe Libman-Sacks endocarditis complicating antiphospholipid syndrome: a retrospective analysis of 23 operated cases.

OBJECTIVE: Data on severe heart valve disease (HVD), including Libman-Sacks endocarditis, associated with SLE and/or APS requiring valvular surgery are scarce. We thus conducted a retrospective study, aimed at describing and clarifying clinical, laboratory, echocardiographic, histopathological and evolutional features of SLE and/or APS patients with severe associated-HVD. METHODS: An observational retrospective multicentric analysis of 23 adults with SLE and/or APS and HVD between 1996 and 2019 and available histopathological report evaluating long-term follow-up. RESULTS: Twenty-three individuals (20 females, median age 37 [range 17-76] years) were included. All had APS (thrombotic in 22, with an arterial phenotype in 15 and with catastrophic APS [CAPS] in six), and 11 (47%) had SLE. Systemic underlying disease had been diagnosed prior to HVD in 12 (52%). In 10 patients (43%), HVD was complicated by cerebral stroke prior to surgery. Twenty patients (87%) had only one pathological valve, the mitral valve in 18 patients (78%). Valvular thickening (n = 19) and valvular regurgitation (n = 19) were the most frequently reported lesions. Fifteen (62%) patients underwent mechanical valve replacement, six (26%) conservative valve repair (five were later re-operated after a median time of 1 [0-4] year), and two (9%) underwent biological valve replacement. Nine patients (39%) presented early-onset post-operative complications, including three CAPS immediately after surgery and one death. After surgery, 18 patients (78%) had normal postoperative valvular function, but almost half of the patients (43%) had post-operative neurological sequelae (median follow-up of 6 [2-20] years). CONCLUSION: Severe HVD leading to surgery was strongly associated with thrombotic APS, especially arterial phenotypes. Half of the reported patients presented cerebral stroke complicating the HVD. Valvular surgery carried a significant risk of CAPS.

A tiny baby intubation team improves endotracheal intubation success rate but decreases residents' training opportunities.

OBJECTIVE: To assess the educational and clinical impact of a tiny baby intubation team (TBIT). STUDY DESIGN: Retrospective study comparing endotracheal intubation (ETI) performed: pre-implementation of a TBIT (T1), 6 months post-implementation (T2), and 4 years post-implementation (T3). RESULTS: Post-implementation (T2), first-attempt success rate in tiny babies increased (44% T1; 59% T2, p = 0.04; 56% T3, p = NS) and the proportion of ETIs performed by residents decreased (53% T1; 37% T2, p = 0.001; 45% T3, p = NS). After an educational quality improvement intervention (prioritizing non-tiny baby ETIs to residents, systematic simulation training and ETI using videolaryngoscopy), in T3 residents' overall (67% T1; 60% T2, p = NS; 79% T3, p = 0.02) and non-tiny baby ETI success rate improved (72% T1; 60% T2, p = NS; 82% T3, p = 0.02). CONCLUSION: A TBIT improves success rate of ETIs in ELBW infants but decreases educational exposure of residents. Educational strategies may help maintain resident procedural competency without impacting on quality of care.

Giant cell arteritis in the elderly / Maladie de Horton du sujet âgé

Introduction: Giant cell arteritis (GCA) or Horton's disease is a segmental and focal inflammation of large and medium-sized arteries mostly seen in patients of 50 years and older. There is also a peak frequency in individuals between the ages of 70 and 80. However, clinical data is scarce in this age group and especially in patients over 80. Methods: A retrospective study comprised of patients diagnosed with Horton's arteritis between 2012 and 2017, according to the American Society of Rheumatology, was conducted at Reims University Hospital. Patients were assigned to two groups according to age (≤ 75 and < 75) in order to evaluate and compare the impact of age on diagnosis, treatment and prognosis. Results: A total of 67 patients were studied. The mean age upon diagnosis was 75,85 ±8.5 years; 36 patients (53.7%) 75 years or younger and 31 patients older than 75. There was a female predominance (43 patients), 22 patients aged 75 years or younger and 21 older than 75. The mean follow up duration was 43.02 months in patients aged 75 years or younger and 30.99 in patients older than 75. This represents a difference of more than one year in terms of follow up, but is not statistically significant (p = 0.620). Eleven patients (16.4%) died during follow up: 5 patients (13.9%) aged 75 years or younger and 6 patients (19.4%) older than 75 (p = 0.547). Aortitis was significantly less seen in patients older than 75 (p = 0.0410). Conclusion: Our study showed no significant difference in either age group. However, aortitis was less seen in patients older than 75 years. Patients aged 75 or younger seemed more prone to relapses, but their follow up periods were shorter.

Introduction: L'artérite à cellules géantes (ACG) ou maladie de Horton est une artérite inflammatoire segmentaire et focale des artères de gros et moyen calibre du sujet de plus de 50 ans, avec un pic de fréquence chez le sujet très âgé entre 70 et 80 ans. Dans cette classe d'âge et au-delà de 80 ans, les données cliniques concernant l'AGC sont peu nombreuses. Notre objectif est de documenter ces dernières à travers une étude monocentrique menée sur une population avec une AGC avérée. Patients et méthode: Nous avons mené une étude rétrospective, monocentrique sur les dossiers médicaux de patients diagnostiqués artérite de Horton selon les critères de l'ASR entre 2012 et 2017 au CHU de Reims. Pour évaluer l'influence de l'âge sur le plan diagnostic, thérapeutique, du suivi et du pronostic, nous avons comparé des patients de 75 ans et moins (≤ 75 ans) à ceux de plus de 75 ans (> 75 ans) sur ces différents points. Résultats: Soixante-sept patients ont été inclus. L'âge moyen au diagnostic de ces patients était de 75,85 ± 8,5 ans ; 36 patients (53,7 %) étaient âgés de 75 ans ou moins (dont 22 femmes) et 31 patients (46,3 %) étaient âgés de plus de 75 ans (dont 21 femmes). La médiane de suivi était de 43,02 mois chez les patients ≤ 75 ans et de 30,99 mois chez les > 75 ans, soit près d'un an de différence, mais non significative (p = 0,620). Onze patients (16,4 %) étaient décédés au cours du suivi, 5 (13,9 %) chez les patients ≤ 75 ans et 6 (19,4 %) chez les patients de > 75 ans (p = 0,547). Les patients > 75 ans avaient significativement moins d'aortite (p = 0,0410). Il y avait une tendance à moins de rechute chez les patients de > 75 ans (p = 0,067). Pour les autres symptômes ou anomalies biologiques, les résultats de la biopsie d'artère temporale, la prise en charge thérapeutique, les complications iatrogènes et les décès, aucune différence significative n'était mise en évidence entre les deux groupes (p = ns). Conclusion: Notre étude montre peu de différence en ce qui concerne l'AGC entre les patients > 75 ans et ceux ≤ 75 ans. Toutefois, les patients > 75 ans ont moins d'aortite que les sujets plus jeunes. Il semble également y avoir une tendance à davantage de rechute chez les sujets les plus jeunes, sous réserve d'une durée de suivi plus courte d'un an, cliniquement pertinente, chez les sujets les plus âgés.

Absence of Anti-Glomerular Basement Membrane Antibodies in 200 Patients With Systemic Lupus Erythematosus With or Without Lupus Nephritis: Results of the GOODLUPUS Study.

Introduction: Anti-glomerular basement membrane (GBM) antibodies are pathogenic antibodies first detected in renal-limited anti-GBM disease and in Goodpasture disease, the latter characterized by rapidly progressive crescentic glomerulonephritis combined with intra-alveolar hemorrhage. Studies have suggested that anti-GBM antibody positivity may be of interest in lupus nephritis (LN). Moreover, severe anti-GBM vasculitis cases in patients with systemic lupus erythematosus (SLE) have been described in the literature, but few studies have assessed the incidence of anti-GBM antibodies in SLE patients. Objective: The main study objective was to determine if positive anti-GBM antibodies were present in the serum of SLE patients with or without proliferative renal damage and compared to a healthy control group. Methodology: This retrospective study was performed on SLE patients' sera from a Franco-German European biobank, developed between 2011 and 2014, from 17 hospital centers in the Haut-Rhin region. Patients were selected according to their renal involvement, and matched by age and gender. The serum from healthy voluntary blood donors was also tested. Anti-GBM were screened by fluorescence enzyme immunoassay (FEIA), and then by indirect immunofluorescence (IIF) in case of low reactivity detection (titer >6 U/ml). Results: The cohort was composed of 100 SLE patients with proliferative LN (27% with class III, 67% with class IV, and 6% with class V), compared to 100 SLE patients without LN and 100 controls. Patients were mostly Caucasian and met the ACR 1997 criteria and/or the SLICC 2012 criteria. Among the 300 tested sera, no significant levels of anti-GBM antibodies were detected (>10 U/ml) by the automated technique, three sera were found "ambivalent" (>7 U/ml): one in the SLE with LN group and two in the SLE without LN group. Subsequent IIF assays did not detect anti-GBM antibodies. Conclusion: Anti-GBM antibodies were not detected in the serum of Caucasian patients with SLE, even in case of renal involvement, a situation favoring the antigenic exposure of glomerular basement membranes. Our results reaffirm the central role of anti-GBM antibodies as a specific diagnostic biomarker for Goodpasture vasculitis and therefore confirm that anti-GBM antibody must not be carried out in patients with SLE (with or without LN) in the absence of disease-suggestive symptoms.

Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations.

OBJECTIVE: Systemic lupus is a chronic autoimmune disease characterised by its phenotypic heterogeneity. Neutropaenia is a frequent event in SLE occurring in 20%-40% of patients depending on the threshold value of neutrophil count. On a daily basis, the management of neutropaenia in SLE is difficult with several possible causes. Moreover, the infectious consequences of neutropaenia in SLE remain not well defined. METHODS: 998 patients from the Lupus BioBank of the upper Rhein (LBBR), a large German and French cohort of patients with SLE, mostly of Caucasian origin (83%), were included in this study. Neutropaenia was considered when neutrophil count was below 1800×106/L. An additional analysis of detailed medical records was done for 65 LBBR patients with neutropaenia. RESULTS: 208 patients with neutropaenia (21%) were compared with 779 SLE patients without neutropaenia. Neutropaenia in SLE was significantly associated with thrombocytopaenia (OR 4.11 (2.57-10.3)), lymphopaenia (OR 4.41 (2.51-11.5)) and low C3 (OR 1.91 (1.03-4.37)) in multivariate analysis. 65 representative patients with neutropaenia were analysed. Neutropaenia was moderate to severe in 38%, chronic in 31%, and both severe and chronic in 23% of cases. Moderate to severe and chronic neutropaenia were both associated with lymphopaenia and thrombopaenia. Chronic neutropaenia was also associated anti-Ro/SSA antibodies and moderate to severe neutropaenia with oral ulcers. CONCLUSION: This study is to date the largest cohort to describe neutropaenia in SLE. Neutropaenia displays a strong association with other cytopaenias, suggesting a common mechanism. Chronic neutropaenia is associated with anti-Ro/SSA antibodies with or without identified Sjögren's disease.

Techniques to Communicate Better With Parents During End-of-Life Scenarios in Neonatology.

BACKGROUND AND OBJECTIVES: Clinicians are urged to optimize communication with families, generally without empirical practical recommendations. The objective of this study was to identify core behaviors associated with good communication during and after an unsuccessful resuscitation, including parental perspectives. METHODS: Clinicians from different backgrounds participated in a standardized, videotaped, simulated neonatal resuscitation in the presence of parent actors. The infant remained pulseless; participants communicated with the parent actors before, during, and after discontinuing resuscitation. Twenty-one evaluators with varying expertise (including 6 bereaved parents) viewed the videos. They were asked to score clinician-parent communication and identify the top communicators. In open-ended questions, they were asked to describe 3 aspects that were well done and 3 that were not. Answers to open-ended questions were coded for easily reproducible behaviors. All the videos were then independently reviewed to evaluate whether these behaviors were present. RESULTS: Thirty-one participants' videos were examined by 21 evaluators (651 evaluations). Parents and actors agreed with clinicians 81% of the time about what constituted optimal communication. Good communicators were more likely to introduce themselves, use the infant's name, acknowledge parental presence, prepare the parents (for the resuscitation, then death), stop resuscitation without asking parents, clearly mention death, provide or enable proximity (clinician-parent, infant-parent, clinician-infant, mother-father), sit down, decrease guilt, permit silence, and have knowledge about procedures after death. Consistently, clinicians who displayed such behaviors had evaluations >9 out of 10 and were all ranked top 10 communicators. CONCLUSIONS: During a neonatal end-of-life scenario, many simple behaviors, identified by parents and providers, can optimize clinician-parent communication.

Disease-specific quality of life following a flare in systemic lupus erythematosus: an item response theory analysis of the French EQUAL cohort.

OBJECTIVE: To explore, at an item-level, the effect of disease activity (DA) on specific health-related quality of life (HRQoL) in SLE patients using an item response theory longitudinal model. METHODS: This prospective longitudinal multicentre French cohort EQUAL followed SLE patients over 2 years. Specific HRQoL according to LupusQoL and SLEQOL was collected every 3 months. DA according to SELENA-SLEDAI flare index (SFI) and revised SELENA-SLEDAI flare index (SFI-R) was evaluated every 6 months. Regarding DA according to SFI and each SFI-R type of flare, specific HRQoL of remitting patients was compared with non-flaring patients fitting a linear logistic model with relaxed assumptions for each domain of the questionnaires. RESULTS: Between December 2011 and July 2015, 336 patients were included (89.9% female). LupusQoL and SLEQOL items related to physical HRQoL (physical health, physical functioning, pain) were most affected by musculoskeletal and cutaneous flares. Cutaneous flares had significant influence on self-image. Neurological or psychiatric flares had a more severe impact on specific HRQoL. Patient HRQoL was impacted up to 18 months after a flare. CONCLUSION: Item response theory analysis is able to pinpoint items that are influenced by a given patient group in terms of a latent trait change. Item-level analysis provides a new way of interpreting HRQoL variation in SLE patients, permitting a better understanding of DA impact on HRQoL. This kind of analysis could be easily implemented for the comparison of groups in a clinical trial. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov, NCT01904812.

[Late-onset rheumatoid arthritis: clinical, biological, and therapeutic features About a retrospective study]. / Polyarthrite rhumatoïde à révélation tardive : caractéristiques cliniques, biologiques et thérapeutiques À propos d'une étude rétrospective.

Rheumatoid arthritis (RA) is the most common chronic inflammatory rheumatism in adults. The objective of our study was to analyze the clinical, biological and therapeutic characteristics in subjects over 60 years old. PATIENTS AND METHODS: We performed a retrospective, monocentric, descriptive study on medical records consultations. The data collection concerned subjects over 60 years of age who had been diagnosed with "rheumatoid arthritis" in the rheumatology and internal medicine departments of CHU Reims over a period stretching from 2010 to 2015. RESULTS: Thirty-two patients were included in our study for this period. The mean age of diagnosis was 66.6 years, for a median age of 67.5 years (min: 60 years, max: 88 years). There were 22 female (69%) and 10 male (31%) patients, with a sex ratio H/F of 2.2. The mean duration of symptom progression before diagnosis was 33.2 months. What dominates our series is the inaugural involvement of the interphalangeal proximal, wrists, shoulders and metacarpophalangeal for the vast majority of cases. Oral corticosteroids were used in 27 patients and were the only treatment in 3 patients. Methotrexate (MTX) was introduced in 27 patients. Nine patients received biotherapy: it was tocilizumab (Roactemra®) for 5 patients, adalimumab (Humira®) for 2 patients, abatacept (Orencia®) for 2 patients, etanercept (Enbrel®) for 2 patients, golimumab (Simponi®) for 1 patient and infliximab (Remicade®) for one patient. In our series, 7 patients are over 75 years old at the time of diagnosis of RA. CONCLUSION: The rheumatoid arthritis of the elderly remains a common condition and constitutes a diagnostic and therapeutic challenge. Because of the co-morbidities, the clinician's perception of the patient's overall condition and the inaccuracies in the use of certain molecules in these patients, under-treatment may, on the contrary, weaken a patient whose remission will be postponed. This was not the case in our series, with a methodical use of methotrexate as well as effective dose biotherapies.

Predictors of fatigue and severe fatigue in a large international cohort of patients with systemic lupus erythematosus and a systematic review of the literature.

OBJECTIVE: Fatigue is reported in up to 90% of patients with SLE. This study was conducted to identify the determinants associated with fatigue in a large cohort of patients with SLE, as well as to provide a systematic review of the literature. METHODS: Patients from the Lupus BioBank of the upper Rhein, a large German-French cohort of SLE patients, were included in the FATILUP study if they fulfilled the 1997 ACR criteria for SLE and had Fatigue Scale for Motor and Cognitive Functions scores collected. Multivariate logistic regression analyses were performed to assess the determinants of fatigue and severe fatigue. RESULTS: A total of 570 patients were included (89.1% female). The median age was 42 years (interquartile range 25-75: 34-52). The median value of the SAfety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI was 2 (0-4). Fatigue was reported by 386 patients (67.7%) and severe fatigue by 209 (36.7%). In multivariate analyses, fatigue was associated with depression [odds ratio (OR): 4.72 (95% CI: 1.39-16.05), P = 0.01], anxiety [OR: 4.49 (95% CI: 2.60-7.77), P < 0.0001], glucocorticoid treatment [OR: 1.59 (95% CI 1.05-2.41), P = 0.04], SELENA-SLEDAI scores [OR: 1.05 (95% CI: 1.00-1.12) per 1 point increase, P = 0.043] and age at sampling [OR: 1.01 (95% CI: 1.00-1.03) per 1 year increase, P = 0.03]. Severe fatigue was independently associated with anxiety (P < 0.0001), depression (P < 0.0001), glucocorticoid treatment (P = 0.047) and age at sampling (P = 0.03). CONCLUSION: Both fatigue and severe fatigue are common symptoms in SLE, and are strongly associated with depression and anxiety. Disease activity and the use of glucocorticoids were also independently associated with fatigue, although more weakly.

Characteristics and clinical outcomes after treatment of a national cohort of PCR-positive Lyme arthritis.

OBJECTIVES: To describe the clinical and microbiological characteristics and outcomes after antibiotic treatment of a national cohort of patients with Lyme arthritis confirmed by PCR testing on synovial fluid and by serology, when available. METHODS: Using the French National Reference Center for Borrelia database, patients with a positive PCR on synovial fluid for Borrelia were identified. Patient clinical and biological characteristics were reviewed from patient records. Long-term outcomes after treatment were studied through a questionnaire and with follow-up data. RESULTS: Among 357 synovial fluid testing by PCR between 2010 and 2016, 37 (10.4%) were positive for Borrelia. Patients' median age was 36 years (range 6-78) with 61% of men and 28% patients under 18. The presentation was monoarticular in 92% and the knee was involved in 97%. Contrary to the Borrelia species repartition in European ticks, B. burgdorferi sensu stricto was the most prevalent species found in synovial fluid (54%) followed by B. azfelii (29%) and B. garinii (17%). Antibiotic treatments were mainly composed of doxycycline (n = 24), ceftriaxone (n = 10) and amoxicillin (n = 6), for a median duration of 4 weeks (range 3-12). Despite a properly conducted treatment, 34% of patients (n = 12) developed persistent synovitis for at least 2 months (median duration 3 months, range 2-16). Among those, 3 developed systemic inflammatory oligo- or polyarthritis in previously unaffected joints with no signs of persistent infection (repeated PCR testing negative), which mandated Disease-Modifying Antirheumatic Drugs (DMARD) introduction, leading to remission. CONCLUSION: In France and contrary to ticks ecology, Lyme arthritis is mainly caused by B. burgdorferi sensu stricto. Despite proper antibiotic therapy, roughly one third of patients may present persistent inflammatory synovitis and a small proportion may develop systemic arthritis. In such cases, complete remission can be reached using DMARD.

[Rheumatoid arthritis and cardiovascular risk factor : literature review]. / Polyarthrite rhumatoïde et facteur de risque cardiovasculaire. Revue de littérature.

The rheumatoid arthritis remains a common condition and constitutes a diagnostic and therapeutic challenge, especially in the elderly. Rheumatoid arthritis is known to be associated with increased mortality, including coronary and cerebrovascular atherosclerosis. A literature review is conducted on the role of rheumatoid arthritis as a cardiovascular risk factor.

La polyarthrite rhumatoïde demeure une affection courante et constitue un défi diagnostique et thérapeutique, notamment chez le sujet âgé. Il est établi que la polyarthrite rhumatoïde est associée à une augmentation de la mortalité, notamment par athérosclérose coronaire et cérébrovasculaire. Une revue de littérature est réalisée sur le rôle de la polyarthrite rhumatoïde comme facteur de risque cardiovasculaire.

[Lupus erythematosus treatment with belimumab in daily practice: Retrospective study of 15 patients]. / Traitement du lupus érythémateux par le bélimumab en pratique courante : étude rétrospective de 15 malades.

BACKGROUND: Belimumab (an anti-BLyS monoclonal antibody) was recently approved for the treatment of systemic lupus erythematosus (SLE). The aim of the study was to describe efficacy and safety of the drug as well as its impact on serologic parameters and the role of long-term systemic sparing of treatment in clinical practice in LE. PATIENTS AND METHODS: We conducted a retrospective study at Reims University Hospital between 2012 and 2016 including consecutive patients with LE treated with belimumab. Efficacy was evaluated in terms of clinical progression, and normalisation of laboratory factors (anti-DNA antibody and C3 serum levels) and sparing of associated long-term systemic therapies for LE. RESULTS: Among the 15 patients included, a therapeutic response was obtained in 9 patients (60%), with partial remission in 8 of 9 cases. The median titre of anti-DNA antibody was 50IU/mL (range: 4-50) and the median C3 level was 0.82g/L (range: 0.36-1.23) before initiation of belimumab, vs. 25.5IU/mL (range: 2-50) and 0.89g/L (range: 0.34-1.22) at the last evaluation, respectively, without significant modification (P=0.12 and P=0.45). The median dose of prednisone at the time of the first belimumab infusion was reduced from 9.5mg/day (range: 0-18) to 6mg/day (range: 0-20) at the last clinical evaluation. Eight patients (53%) experienced adverse events, and these were very slight or moderate in all cases. CONCLUSION: Belimumab appears to be an effective and well-tolerated treatment for moderately severe systemic LE, allowing sparing of maintenance corticosteroid therapy in order to decrease its frequent adverse events.

Long-term efficacy and safety of rituximab in IgG4-related disease: Data from a French nationwide study of thirty-three patients.

OBJECTIVES: To assess efficacy and safety of rituximab (RTX) as induction therapy, maintenance of remission and treatment of relapses in a cohort of IgG4-related disease (IgG4-RD) patients. METHODS: Nationwide retrospective multicenter study of IgG4-RD patients treated with at least one course of RTX. Clinical, biological and radiological response, relapse rate and drug tolerance were analyzed. Kaplan-Meier curves were plotted and risk factors for relapse studied with a Cox regression model. RESULTS: Among 156 IgG4-RD patients included in the French database, 33 received rituximab. Clinical response was noted in 29/31 (93.5%) symptomatic patients. Glucocorticoids withdrawal was achieved in 17 (51.5%) patients. During a mean follow-up of 24.8 ±21 months, 13/31 (41.9%) responder patients relapsed after a mean delay of 19 ±11 months after RTX. Active disease, as defined by an IgG4-RD Responder Index >9 before RTX, was significantly associated with relapse (HR = 3.68, 95% CI: 1.1, 12.6) (P = 0.04), whereas maintenance therapy with systematic (i.e. before occurrence of a relapse) RTX retreatment was associated with longer relapse-free survival (41 versus 21 months; P = 0.02). Eight severe infections occurred in 4 patients during follow-up (severe infections rate of 12.1/100 patient-years) and hypogammaglobulinemia ≤5 g/l in 3 patients. CONCLUSION: RTX is effective for both induction therapy and treatment of relapses in IgG4-RD, but relapses are frequent after B-cell reconstitution. Maintenance therapy with systematic RTX infusions is associated with longer relapse-free survival and might represent a novel treatment strategy. Yet, the high rate of infections and the temporary effect of RTX might be hindrances to such strategy.

Fluid restriction for treatment of preterm infants with chronic lung disease.

BACKGROUND: Fluid restriction is often recommended as part of the management of infants with early or established bronchopulmonary dysplasia (BPD). OBJECTIVES: To determine whether fluid restriction as part of the therapeutic intervention for early or established BPD improves clinical outcomes. SEARCH METHODS: We used the standard search strategy of Cochrane Neonatal to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 1) in the Cochrane Library (searched 16 February 2016), MEDLINE via PubMed (1966 to 16 February 2016), Embase (1980 to 16 February 2016), and CINAHL (1982 to 16 February 2016). We also searched clinical trials' databases, conference proceedings, and the reference lists of retrieved articles for randomised controlled trials and quasi-randomised trials. SELECTION CRITERIA: Prospective randomised clinical trials comparing two distinct fluid administration volumes in preterm infants with early or established BPD. DATA COLLECTION AND ANALYSIS: We used the standard methods of Cochrane Neonatal. For the included trial, we extracted data and assessed the risk of bias, and used GRADE methods to assess the quality of the evidence. The outcomes considered in this review are effects on mortality or requirement for oxygen at 36 weeks' postmenstrual age (primary outcome measure), the duration of supplemental oxygen therapy, proportion of infants discharged from hospital on oxygen, duration of assisted ventilation, duration of hospitalisation, weight gain, feeding tolerance, apnoea, necrotizing enterocolitis, renal dysfunction or nephrocalcinosis, lung mechanics, and use of diuretic therapy (secondary outcome measures). MAIN RESULTS: One trial was found, including 60 preterm infants at 28 days of age with persistent oxygen requirements. Infants were randomised to either 180 mL/kg/day of standard formula or 145 mL/kg/day of concentrated formula. This single study did not provide data regarding our primary outcome. No effects of the intervention were found on any of our secondary outcomes. The quality of the evidence from this study was graded low. AUTHORS' CONCLUSIONS: There is no evidence to support the practice of fluid restriction in infants with early or established BPD.

In Vitro Fertilization in 37 Women with Systemic Lupus Erythematosus or Antiphospholipid Syndrome: A Series of 97 Procedures.

OBJECTIVE: To compile and assess data about complication and success rates for in vitro fertilization (IVF) of women with systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). To date, such data are sparse. METHODS: This retrospective study described women with SLE and/or APS who have had at least 1 IVF cycle. RESULTS: Thirty-seven women with SLE (n = 23, including 8 with antiphospholipid antibodies), SLE with APS (n = 4), or primary APS (n = 10) underwent 97 IVF procedures. For 43% of cases, the infertility was female in origin, for 19% male, 14% mixed, and 24% unexplained. No women had premature ovarian insufficiency because of cyclophosphamide. Median age at IVF was 34 years (range 26-46). The median number of IVF cycles was 2.6 (1-8). Patients were treated with hydroxychloroquine (72%), steroids (70%), azathioprine (3%), aspirin (92%), and/or low molecular weight heparin (62%). There were 27 (28%) pregnancies, 23 live births among 26 neonates (3 twin pregnancies), 2 miscarriages, and 2 terminations for trisomy 13 and 21. Six spontaneous pregnancies occurred during the followup. Finally, 26 women (70%) delivered at least 1 healthy child. Complications occurred in or after 8 IVF cycles (8%): SLE flares in 4 (polyarthritis in 3 and lupus enteritis in 1) and thromboembolic events in 4 others. One SLE flare was the first sign of previously undiagnosed SLE. Poor treatment adherence was obvious in 2 other flares and 2 thromboses. No ovarian hyperstimulation syndrome was reported. CONCLUSION: These preliminary results confirm that IVF can be safely and successfully performed in women with SLE and/or APS.