Use of mesna prophylaxis in patients with cyclophosphamide-treated ANCA-associated vasculitis: cross-sectional survey of practitioners.

BACKGROUND: There may be some diversity in the practice of co-prescribing 2-mercaptoethane sodium sulfonate (mesna) with cyclophosphamide (CYC) for ANCA-associated vasculitis (AAV). OBJECTIVES: To assess the practice of prescribing mesna prophylaxis for CYC-treated patients with AAV. METHODS: We invited authors of publications on AAV referenced in MEDLINE over the previous 10 years to participate in an anonymous online survey. Respondents were eligible if they were involved in CYC treatments for AAV. The survey asked about the characteristics of the respondents and their practice in using CYC and mesna to treat AAV and the underlying rationale. We compared 18 variables between mesna prescribers and their counterparts to identify factors associated with mesna use. RESULTS: In total, 139 eligible individuals completed the survey. The participants were from 34 countries and were essentially physicians (98%). Overall, 68%, 19% and 13% of respondents prescribed mesna systematically, never, or on a selective basis. As compared with never/selective-prescribers, systematic-prescribers were more often ≤ 39 years old (P = 0.008), more often used intermittent pulse therapy as the exclusive/predominant CYC administration scheme (P < 0.001), were more frequently based in France/Germany/Italy than in England/United States (P < 0.001), and more often indicated adherence to local standards (P = 0.003) or (inter)national guidelines for AAV (P < 0.001) as a rationale for their mesna practice. Never/selective-prescribers more commonly reported that their mesna prescription pattern had changed as compared with their former practice (P < 0.001). CONCLUSIONS: Systematic co-prescription of mesna is the prevailing practice for CYC treatments for AAV. The practice seems to involve practicability considerations and differs between generations.

Incidence of giant cell arteritis in six districts of Paris, France (2015-2017).

This prospective population-based study estimated the incidence of giant cell arteritis (GCA) in northeastern Paris. GCA cases diagnosed between 2015 and 2017 were obtained from local hospital and community-based physicians and the national health insurance system database. Criteria for inclusion were living in the study area at that time and fulfilling the 1990 American College of Rheumatology classification criteria and/or its expanded version. Cranial and large-vessel GCA cases were defined by the presence or absence of cranial signs and/or symptoms, respectively. Annual incidence was calculated by dividing the number of incident cases by the size of the study population ≥ 50 years old. Completeness of case ascertainment was assessed by a three-source capture-recapture analysis. Among the 62 included cases, 42 (68%) were women, mean (± SD) age 77.3 ± 9.1 years. The annual incidence of GCA in northeastern Paris and completeness of case ascertainment were estimated at 7.6 (95% CI 5.9-9.8) per 100,000 inhabitants ≥ 50 years old and 66% (95% CI 52-92%), respectively. Incidence increased with age, peaked at age 80-89 years, and was almost twice as high in women versus men. Large-vessel GCA cases, mean (± SD) age 68.6 ± 11.5 years, accounted for 8% of all GCA cases. In this study, GCA epidemiology was mainly driven by cases with cranial GCA signs or symptoms and incidence results were consistent with recent European and past French studies.

Sensitivity of temporal artery biopsy in the diagnosis of giant cell arteritis: a systematic literature review and meta-analysis.

OBJECTIVES: Temporal artery biopsy (TAB) is a reference test for the diagnosis of GCA but reveals inflammatory changes only in a subset of patients. The lack of knowledge of TAB sensitivity hampers comparisons with non-invasive techniques such as temporal artery ultrasonography. We performed a systematic literature review and meta-analysis to estimate the sensitivity of TAB in GCA and to identify factors that may influence the estimate. METHODS: A systematic literature review involved searching electronic databases and cross-references. Eligibility criteria included publications reporting at least 30 GCA cases fulfilling the original or modified 1990 ACR classification criteria. The pooled proportion of TAB-positive GCA cases was calculated by using aggregated-data meta-analysis with a random-effects model and assessment of heterogeneity with the I2 statistic. Subgroup analyses and meta-regression were used to examine the effect of patient and study characteristics on TAB positivity. RESULTS: Among 3820 publications screened, 32 studies (3092 patients) published during 1993-2017 were analysed. The pooled proportion of TAB-positive GCA cases was 77.3% (95% CI: 71.8, 81.9%), with high between-study heterogeneity (I2 = 90%). The proportion of TAB-positive cases was slightly higher in publications before than in 2012 and after (P = 0.001). CONCLUSION: The estimated sensitivity of 77% provides indirect evidence that TAB is not less sensitive than temporal artery imaging. The unexplained high between-study heterogeneity could result from differences in TAB sampling, processing or interpretation. The decrease in TAB-positive GCA cases over time could reflect an increasing propensity for clinicians to accept a GCA diagnosis without proof by TAB.

Vaccination and Risk of Childhood IgA Vasculitis.

BACKGROUND AND OBJECTIVES: Immunoglobulin A vasculitis (IgAV) might develop after vaccination. However, this potential relationship is essentially based on case reports, and robust pharmaco-epidemiologic data are scarce. We aimed to investigate the effect of vaccination on short-term risk of IgAV in children. METHODS: We enrolled children <18 years old with IgAV seen in 5 pediatric departments from 2011 to 2016. Data on vaccinations administered during the year preceding IgAV onset were collected from immunization records. With a case-crossover method and by using conditional logistic-regression analyses, odds ratios (ORs) and 95% confidence intervals (CIs) were calculated by comparing vaccine exposure during the 3-month "index period" immediately preceding IgAV onset to that during 3 consecutive 3-month "control" periods immediately before the index period. Stratifications by season, year of onset, infection history, age, sex, type, or number of vaccines were performed. Sensitivity analyses used 1-, 1.5-, or 2-month index and control periods. RESULTS: Among 167 children (mean age: 6.7 years) enrolled, 42 (25%) received ≥1 vaccine during the year before IgAV onset. Fifteen (9%) children were vaccinated during the 3-month index period as compared with 4% to 7% during the 3 control periods. The OR for IgAV occurring within the 3 months after vaccination was 1.6 (95% CI: 0.8-3.0). Analyses of IgAV risk within 1, 1.5, or 2 months of vaccination yielded ORs of 1.4 (95% CI: 0.5-3.5), 1.4 (95% CI: 0.6-3.2), and 1.3 (95% CI: 0.6-2.6), respectively. Stratifications revealed no significant association. CONCLUSIONS: Vaccination may not be a major etiological factor of childhood IgAV.

Mortality causes and trends associated with giant cell arteritis: analysis of the French national death certificate database (1980-2011).

OBJECTIVES: Comprehensive analyses of cause-specific death patterns in GCA are sparse. We studied the patterns and time trends in GCA-related mortality using a large death certificate database. METHODS: We obtained multiple-cause-of-death data from the French national death certificate database for 1980-2011. GCA-associated deaths were defined as decedents ⩾55 years old with GCA listed as an underlying or non-underlying cause of death. Time trends of death rates were analysed and the mean age at death with GCA and in the general population ⩾55 years old were calculated. Standardized mortality odds ratios (SMORs) were calculated for 17 selected causes of death (based on 2000-11 data). RESULTS: The analyses pertained to approximately 15 000 death certificates listing GCA (including approximately 6300 for 2000-11). Annual standardized death rates for GCA increased to a peak in 1997 and then decreased (Spearman's correlation test, both P < 0.0001). Mean age at death was higher for GCA than for general population decedents (Student's t-test, P < 0.0001). GCA deaths were frequently or strongly associated with aortic aneurysm and dissection (1.85% of death certificates, SMOR: 3.09, 95% CI: 2.48, 3.82), hypertensive disease (20.78%, SMOR: 2.22, 95% CI: 1.97, 2.50), diabetes mellitus (11.27%, SMOR: 1.96, 95% CI: 1.72, 2.23), certain infectious and parasitic diseases (12.12%, SMOR: 1.76, 95% CI: 1.55, 2.00) and ischaemic heart disease (16.54%, SMOR: 1.45, 95% CI: 1.35, 1.64). CONCLUSION: GCA is associated with increased risk of dying from large-vessel disease, other cardiovascular diseases and potentially treatment-related co-morbidities. These findings help provide better insights into the outcomes of GCA.

Poor Predictive Value of Isolated Adventitial and Periadventitial Infiltrates in Temporal Artery Biopsies for Diagnosis of Giant Cell Arteritis.

OBJECTIVE: We investigated the diagnostic value of inflammation limited to the adventitia (ILA), and isolated vasa vasorum or small-vessel vasculitis (VVV, SVV) in temporal artery biopsies (TAB) for giant cell arteritis (GCA). METHODS: Two pathologists reviewed consecutive first TAB. Using the clinical diagnoses as the gold standard, positive predictive values (PPV) were calculated. RESULTS: Among the 75 patients without classic TAB features of GCA, 8 had GCA diagnoses. The PPV of ILA, VVV, and SVV seen by either or both pathologists were 17%, 0%, and 7%, and 17%, 0%, and 10%, respectively. CONCLUSION: (Peri)adventitial infiltrates in TAB poorly predict GCA.

Dietary and Nondietary Triggers of Oral Ulcer Recurrences in Behçet's Disease.

OBJECTIVE: The nature and impact of food and other external triggers in recurrences of Behçet's disease (BD)-related oral ulcers (OUs) remain unknown. This survey investigated dietary and nondietary triggers of BD-related OU recurrences. METHODS: Patients with BD who were followed in 7 French hospital departments completed a self-administered patient questionnaire. General and specific dietary triggering factors were sought in open questions. The questionnaire also included closed questions, notably to evaluate the effect of 6 general triggering situations and 24 selected foods. The results were expressed as number (percentage) of positive responses. RESULTS: Among the 101 questionnaires distributed, 81 were usable. Among the 81 patients, 96% fulfilled the International Criteria for Behçet's Disease classification criteria, and 53% qualified their OU recurrences during the previous 12 months as very discomforting or discomforting. For the 6 general situations suggested, 50 patients (62%) declared ≥1 as a "sure" trigger of OU recurrences. In both open and closed questions, the most frequent triggers were fatigue/stress (37-47% of patients) and food (32-35%). Among the 24 suggested foods, nuts (48%), pineapple (42%), peanuts (32%), Emmental cheese (30%), almonds (23%), lemons (22%), and other cheeses (21%) were the most frequently reported. The corresponding open question gave consistent findings but with lower frequencies. CONCLUSION: Most patients can identify triggers of recurring BD-related OUs, with fatigue/stress and food representing the most frequent triggers. The management of OU must consider such external factors. The histamine-rich or -liberating properties of the commonly cited OU-triggering foods suggest a hyperreactivity mechanism.

Use of clean intermittent self-catheterization in France: A survey of patient and GP perspectives.

AIMS: To estimate the prevalence of GP (general practitioner) patients performing ISC (intermittent self-catheterization), to describe GP and patient knowledge about ISC and to assess the patients' quality of life. METHODS: A cross-sectional study was conducted in metropolitan France between November 2012 and September 2013 with French Sentinelles GPs and their patients (≥18 years) performing ISC. Linear regression models assessed independent associations between quality of life scores (Qualiveen and SF-12 scores) and patients' characteristics. RESULTS: The prevalence of ISC performers was estimated to be 61.7 (95% confidence interval [CI] = 61.0-62.4) of 100,000 inhabitants. Patients were males in 58.8% of cases and 53.8 years of age on average. They performed 4.84 catheterizations per day for 10.5 years. Over 1 year, there were 118 (55.1%) cases of patient-reported urinary incontinence (UI) and 158 (63.3%) cases of patient-reported urinary tract infection (UTI). Symptomatic UTIs were treated with antibiotics in 79.7% of cases. The average Qualiveen score was 1.38 (95%CI: 1.23-1.53). The average SF-12 Physical Component Score (PCS) and Mental Component Score (MCS) were 38.6 (95%CI: 36.8-40.4) and 46.4 (95%CI: 44.3-48.5), respectively. According to multivariate analysis, a poorer Qualiveen score and constipation reduced the PCS and a urinary medication prescription reduced the MCS. A poorer Qualiveen score was also associated with UI and UTI on bivariate analysis. CONCLUSIONS: ISC seems to be significant in French general practice whereas practical skills remain heterogeneous. UI, UTI, and constipation are recurrent issues among ISC performers that significantly decrease their quality of life. Education and adapted guidelines for GP guidance could improve these patients' conditions. Neurourol. Urodynam. 35:528-534, 2016. © 2015 Wiley Periodicals, Inc.