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OBJECTIVE Advanced chronic kidney disease (CKD) carries an increased risk for progression to end-stage renal disease (ESRD). We aimed to determine the rate of progression and the factors that drive the decline of renal function in lupus nephritis (LN). METHODS Patients with advanced LN-related CKD were identified from our longterm longitudinal cohort. Advanced CKD was defined as stage 3b (eGFR=30-44ml/min/1.73m2) and stage 4 (eGFR=15-29ml/min/1.73m2). All individuals were followed until progression to ESRD or the last visit and were divided into "progressors" and "non-progressors". Demographic, clinical, immunological, and therapeutic variables were compared at baseline. Multivariable Cox regression analysis (both time-dependent and independent) was performed to identify predictors for progression. [https://www.selleckchem.com/products/bi-3231.html check details] RESULTS One hundred eighteen patients (74 CKD 3b and 44 CKD 4) were included. Forty-five patients progressed (29 to ESRD and 16 from CKD 3b to CKD 4) after six years on average. No significant decline in the renal function was observed in 73 patients ("non-progressors") after 10 years on average. Active serology (high anti-dsDNA titers and low complements C3/C4) at the time of CKD diagnosis and any increase of the daily prednisone dose after baseline were strongly associated with progression. Treatment with renin angiotensin system (RAS) blockers was associated with less risk for progression. CONCLUSION Dialysis is not inevitable in LN-related advanced CKD since 62% of our patients did not progress over 10 years of follow-up on average. Certain predictors were identified to affect progression to ESRD.OBJECTIVE Acne fulminans (AF) is a rare, explosive systemic form of acne. Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is a primarily pediatric autoinflammatory disorder characterized by sterile osteolytic bone lesions. Concomitant occurrence of CNO/CRMO and AF is very rare and little is known about the epidemiological and clinical particularities of this association. The aim of this retrospective observational study was to describe the characteristics of pediatric patients with CNO/CRMO associated to AF. METHODS Electronic mailing lists of French medical societies were used to call for patients with CNO/CRMO and AF. A search for published patients with CNO/CRMO and AF was performed by screening PubMed. RESULTS We identified 5 original patients and 10 patients from the literature. All patients were adolescent boys. Mean age at disease onset was 14.9 years. 9/15 patients had received isotretinoin before the sudden onset of AF. Osteoarticular symptoms appeared within less than 1-3 months after the onset of AF. The mean numbers of clinical and radiological bone lesions were 3.6 and 5.6. The percentages of patients with involvement of vertebrae, pelvis, chest and cranial were 40%, 40%, 30%, 6.6%. Arthritis was observed in 69.2% of patients and sacroiliac arthritis in 46.2%. CONCLUSION CNO/CRMO associated to AF occurs predominantly in male adolescents and is characterized by frequent involvement of the axial skeleton and arthritis. Epidemiological and clinical features of these patients differ from general CNO/CRMO cohorts. Clinical management requires careful handling of isotretinoin doses.OBJECTIVE Well-designed randomized controlled trials (RCTs) mitigate bias and confounding, but prior evaluations of rheumatology trials found high rates of methodological flaws. Outside of rheumatoid arthritis, no studies in the modern era have assessed the quality of rheumatology RCTs over time or with respect to industry funding. METHODS We identified all RCTs published in three high impact rheumatology journals from the years 1998, 2008, and 2018. Quality metrics derived from a modified Jadad scale were analyzed by the year of publication and by funding source. RESULTS Ninety-six publications met inclusion criteria; 82 of these described the primary analysis of an RCT. Over time (1998 - 2008 - 2018), trials were less likely to adequately report dropouts and withdrawals (100% vs. 82% vs. 60%, p less then 0.01) or include an active comparator (44% vs. 12% vs. 13%, p = 0.01). Later trials were more likely to evaluate biologic therapy (11% vs. 38% vs. 83%, p less then 0.01) and report adequate randomization procedures (39% vs. 29% vs. 60%, p = 0.04). Seventy nine percent of trials received industry funding. Industry funded trials were more likely to report double blinding (86% vs. 53%, p less then 0.01), patient reported outcome measures (77% vs. 41%, p less then 0.01), and intention to treat analyses (86% vs. 65%, p = 0.04). CONCLUSION Industry funded trials comprise the majority of RCTs published in high impact rheumatology journals and more frequently report metrics associated with RCT quality. RCTs assessing active comparators and non-biologic therapies have become less common in high impact rheumatology journals.OBJECTIVE To assess the validity and clinical utility of the Brief Illness Perception Questionnaire (BIPQ) to measure illness perceptions in multiple forms of vasculitis. METHODS Patients with giant cell arteritis (GCA), Takayasu's arteritis (TAK), ANCA-associated vasculitis (AAV) and relapsing polychondritis (RP) were recruited into a prospective, observational cohort. Patients independently completed the BIPQ, Multidimensional Fatigue Inventory (MFI), SF-36 Health Survey (SF-36) and a Patient Global Assessment (PtGA) at successive study visits. Physicians concurrently completed a Physician Global Assessment (PhGA) form. Illness perceptions, as assessed by the BIPQ, were compared to responses from the full-length Revised Illness Perception Questionnaire (IPQ-R) and to other clinical outcome measures. RESULTS 196 patients (GCA=47, TAK=47, RP=56, AAV=46) were evaluated over 454 visits. Illness perception scores in each domain were comparable between the BIPQ and IPQ-R (3.28 vs 3.47, p=0.22). Illness perceptions differed by type of vasculitis, with the highest perceived psychological burden of disease in RP. The BIPQ was significantly associated with all other patient-reported outcome measures (ρ=0.50-0.70, p less then 0.0001) but did not correlate with PhGA (ρ=0.13, p=0.13). A change in the BIPQ composite score of ≥7 over successive visits was associated with concomitant change in the PtGA. Change in the MFI and BIPQ scores significantly correlated over time (ρ= 0.38, p=0.0008). CONCLUSION The BIPQ is an accurate and valid assessment tool to measure and monitor illness perceptions in patients with vasculitis. Use of the BIPQ as an outcome measure in clinical trials may provide complimentary information to physician-based assessments.