Somanshi Sehgal, Zoha Zahid Fazal, Latika Gupta, Thomas Sheeran
{"title":"Progressive dermopathy akin to pseudoxanthoma elasticum after D-penicillamine use in a patient with cystinuria.","authors":"Somanshi Sehgal, Zoha Zahid Fazal, Latika Gupta, Thomas Sheeran","doi":"10.1093/rheumatology/keac117","DOIUrl":"https://doi.org/10.1093/rheumatology/keac117","url":null,"abstract":"","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"e324"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39960867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Prevalence of moderate to severe lower urinary tract symptoms in systemic sclerosis.","authors":"Ukrit Rompsaithong, Wichien Sirithanaphol, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Chingching Foocharoen","doi":"10.1093/rheumatology/keac052","DOIUrl":"https://doi.org/10.1093/rheumatology/keac052","url":null,"abstract":"<p><strong>Objective: </strong>Lower urinary tract symptoms (LUTS) are common in SSc. The severity of symptoms can affect the quality of life (QOL); however, LUTS is often neglected during routine assessments. We determined the prevalence of moderate to severe LUTS in SSc and its associated factors.</p><p><strong>Methods: </strong>A cross-sectional study was conducted between March 2020 and June 2020. Adult SSc patients were enrolled from the Scleroderma Clinic, Khon Kaen University, Thailand. All completed a self-administered questionnaire on LUTS using the International Prostate Symptom Score (IPSS), categorized into absent, mild, moderate or severe LUTS. In addition, we investigated the factors associated with moderate to severe LUTS, and the correlation between IPSS-QOL score and IPSS severity.</p><p><strong>Results: </strong>A total of 135 patients were enrolled. Most cases were female (87 cases; 64.4%) and had dcSSc (88 cases; 65.2%). Twenty-six were defined as having moderate to severe LUTS, for a prevalence of 19.3% (95% CI 13.0, 26.9%). In addition, most had storage symptoms (63.0%), followed by voiding symptoms (19.3%) and post-voiding symptoms (12.6%). The factors associated with moderate to severe LUTS per the multivariable logistic regression included a modified Rodnan skin score ≥20 points and gastrointestinal symptoms with adjusted odds ratios 7.64 and 5.78, respectively. In addition, the IPSS-QOL score had a moderate positive correlation with IPSS severity (rho = 0.560, P < 0.001).</p><p><strong>Conclusion: </strong>Moderate to severe LUTS occurred in about one-fifth of SSc patients, particularly those with extensive skin tightness and gastrointestinal involvement. The more severe the LUTS, the poorer the QOL.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"4016-4023"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39742987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Easy-BILAG: as easy as ABC?","authors":"Kathryn Connelly, Eric F Morand","doi":"10.1093/rheumatology/keac062","DOIUrl":"https://doi.org/10.1093/rheumatology/keac062","url":null,"abstract":"The BILAG-2004 is regarded as the most comprehensive disease activity index currently used in SLE. However, its complexity can limit the efficiency and accuracy of scoring, and this has both posed a barrier to its broader adoption and potentially impacted on its utility as part of the standard clinical trial endpoints. In this issue of Rheumatology, Carter and colleagues describe development and initial accuracy and usability testing of a more streamlined version of the BILAG-2004 [1]. Dubbed the ‘Easy-BILAG’, this new version is proposed as the recommended format for scoring the BILAG-2004 index in routine clinical care. The BILAG-2004 is an organ-based scale with gradations of severity based on physician intention-to-treat. It covers nine organ systems and comprises 97 items defined in an extensive glossary. Each item is scored as new/recurrence, worse, same, improving or not present during the most recent month, relative to the month prior. These scores are integrated in an algorithm resulting in each organ being assigned a grade from A to E, where A, B and C represent major, intermediate and mild disease activity, respectively; D represents previous involvement that is currently inactive; and E represent no previous activity [2]. The proposed ‘Easy-BILAG’ maintains full fidelity to the BILAG-2004, which has been well-studied in regard to reliability and construct validity [3], but with changes in scoring format intended to enhance accuracy and feasibility. These changes, prototyped based on feedback from expert clinicians, are several, and include (i) grouping of items based on their frequency to allow better visibility and prioritization of common manifestations; (ii) the ability to derive A–E grading at the time of assessment, which eliminates the opacity of the previous computer-based algorithm; and (iii) an optional self-adjudication checklist that draws attention to common scoring errors. On face value, the changes made are logical and lead to an enhanced user experience compared with the BILAG-2004. This is reflected in operational improvements, whereby the paper-case-based validation exercises described in the study found higher accuracy of the Easy-BILAG across organ domains compared with the traditional BILAG-2004 (median accuracy 96.7% vs 87.8%), achievable in shorter completion times (average 59.5 vs 80.0 min for ten cases), as well as higher levels of inter-rater agreement and better usability ratings among clinicians. Importantly, the validation exercises were deployed using various participants, including lupus specialists and general rheumatologists, rheumatology trainees and lupus nurse specialists across both tertiary and general settings, with Easy-BILAG maintaining its better performance characteristics across this range of potential real-world users. As acknowledged by the authors, however, the Easy-BILAG is yet to be formally tested in a patient-facing context, or among clinicians for whom English is not a first language;","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"3879-3880"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39927769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Autoantibody profiles associated with morbidity and mortality in scleroderma renal crisis.","authors":"Hideaki Tsuji, Nobuo Kuramoto, Tsuneo Sasai, Mirei Shirakashi, Hideo Onizawa, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Ryu Watanabe, Akira Onishi, Kosaku Murakami, Hajime Yoshifuji, Masao Tanaka, Motomu Hashimoto, Koichiro Ohmura, Akio Morinobu","doi":"10.1093/rheumatology/keac047","DOIUrl":"https://doi.org/10.1093/rheumatology/keac047","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study was to investigate association of autoantibodies with scleroderma renal crisis (SRC) among Japanese patients.</p><p><strong>Methods: </strong>The clinical characteristics and mortality of 330 patients with sytemic screlosis (SSc) at Kyoto University Hospital were retrospectively analysed, focusing on possible association with anti-topoisomerase I (anti-topo I), anti-centromere (ACA), anti-RNA polymerase III (RNAPIII) and/or anti-U1-RNP. Logistic regression analyses were performed to reveal any association of these autoantibodies with the development and mortality of SRC.</p><p><strong>Results: </strong>SRC was observed in 24 out of 330 SSc patients, including patients with anti-topo I (n = 12/24, 50%), anti-RNAPIII (n = 7/24, 29%), anti-U1-RNP (n = 5/24, 21%) and ACA (n = 3/24, 13%). Anti-U1-RNP [odds ratio (95% CI), 3.63 (1.11, 10.2)], anti-topo I [3.22 (1.37, 7.57)] and anti-RNAPIII (3.29 [1.16, 8.70]) were associated with the development of SRC. Furthermore, anti-topo I [6.00 (1.11, 41.1)] was associated with 1-year mortality of SRC. The 1-year survival rate after the onset of SRC among all patients and among those positive for anti-topo I was 54% and 33%, respectively. In contrast, the survival rate in patients negative for anti-topo I was 75%, of which the survival rate of patients positive for anti-RNAPIII and ACA was 83% and 100%, respectively.</p><p><strong>Conclusion: </strong>Specific SSc-related autoantibodies were associated with the morbidity and mortality of SRC.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"4130-4135"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39859926","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ahmad A Sherbini, James M Gwinnutt, Kimme L Hyrich, Suzanne M M Verstappen
{"title":"Rates and predictors of methotrexate-related adverse events in patients with early rheumatoid arthritis: results from a nationwide UK study.","authors":"Ahmad A Sherbini, James M Gwinnutt, Kimme L Hyrich, Suzanne M M Verstappen","doi":"10.1093/rheumatology/keab917","DOIUrl":"https://doi.org/10.1093/rheumatology/keab917","url":null,"abstract":"Abstract Objectives To estimate prevalence rates and identify baseline predictors of adverse events (AEs) over the first year of treatment in patients with RA starting MTX. Methods Data came from the UK Rheumatoid Arthritis Medication Study (RAMS), a prospective cohort of patients with RA starting MTX. This analysis included patients aged ≥18 years with physician diagnosed RA and symptom duration ≤2 years, who were commencing MTX for the first time. AEs were recorded by interviewing patients at 6- and 12-month follow-up visits. The period prevalence rates of AEs are reported for 0–6 months, 6–12 months and 0–12 months of follow-up. The associations between baseline characteristics and AEs were assessed using multivariable logistic regression. Results A total of 1069 patients were included in the analysis. Overall, 77.5% experienced at least one AE. The most commonly reported AEs were: gastrointestinal (42.0%), neurological (28.6%), mucocutaneous (26.0%), pulmonary (20.9%), elevated alanine transaminase (18.0%) and haematological AEs (5.6%). Factors associated with increased odds of AEs were: women vs men (gastrointestinal, mucocutaneous, neurological) and alcohol consumption (nausea, alopecia, mucocutaneous). Older age, higher estimated glomerular filtration rate and alcohol consumption were associated with less reporting of haematological AEs. Conclusions AEs were common among patients over the first year of MTX, although most were not serious. Knowledge of the rates and factors associated with AE occurrence are valuable when communicating risks prior to commencing MTX. This can help patients make informed decisions whether to start MTX, potentially increasing adherence to treatment.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"3930-3938"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/40/77/keab917.PMC9536779.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39720920","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Falk Schumacher, Nikola Mrdenovic, Dennis Scheicht, Jörn Pons-Kühnemann, Christine Scheibelhut, Johannes Strunk
{"title":"Humoral immunogenicity of COVID-19 vaccines in patients with inflammatory rheumatic diseases under treatment with Rituximab: a case-control study (COVID-19VacRTX).","authors":"Falk Schumacher, Nikola Mrdenovic, Dennis Scheicht, Jörn Pons-Kühnemann, Christine Scheibelhut, Johannes Strunk","doi":"10.1093/rheumatology/keac036","DOIUrl":"https://doi.org/10.1093/rheumatology/keac036","url":null,"abstract":"<p><strong>Objectives: </strong>Patients with inflammatory rheumatic diseases (IRDs) treated with the anti-CD20 mAb rituximab (RTX) have been identified as high-risk for severe COVID-19 outcomes. Additionally, there is increased risk due to reduced humoral immune response, induced by therapeutic B cell depletion. This study sought to quantify humoral response after vaccination against SARS-CoV-2 in patients with IRD treated with RTX. It also sought to elucidate the influence of the time frame between the last RTX dose and the first vaccination, or the status of B cell depletion on antibody titre.</p><p><strong>Methods: </strong>In this case-control study, patients with IRDs previously treated with RTX were examined for humoral immune response after completing the first series of vaccinations with approved vaccines [BNT162b2 (Biontech/Pfizer), RNA-1273 (Moderna), AZD1222 (AstraZeneca/Oxford), Ad26.COV2.S (Janssen/Johnson & Johnson)]. Antibody levels were quantified using the Euroimmun Anti-SARS-CoV-2 QuantiVac ELISA (EI-S1-IgG-quant). Blood samples were taken just before the next infusion with RTX after the vaccination. The interval between the last RTX infusion and the first vaccination against SARS-CoV-2 and other possible factors influencing the antibody levels were evaluated.</p><p><strong>Results: </strong>A total of 102 patients were included. Of these, 65 (64%) showed a negative antibody level (<24 IU (international unit)/ml) after the vaccination. The comparative univariate analysis of the antibody levels achieved a significant result (P = 0.0008) for the time between the last RTX infusion and first vaccination against SARS-CoV-2. No CD19+ peripheral B-cells could be detected in 73 of the patients (72%).</p><p><strong>Conclusion: </strong>The study confirms the negative impact of RTX on antibody level after vaccination against SARS-CoV-2. A clear relationship exists between the antibody titre and the interval between the last RTX infusion and the first vaccination, the number of peripheral B-cells, and immunoglobulin quantity. Improved understanding of the effect of these parameters can help guide synchronization of vaccination in relation to the RTX therapy regimen.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"3912-3918"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8807328/pdf/keac036.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39747861","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hanna Lythgoe, Eve M D Smith, Orla G Killeen, Ruth Murphy, Clarissa Pilkington, Clare E Pain, Michael W Beresford
{"title":"Prospective epidemiological study of juvenile-onset systemic lupus erythematosus in the UK and Republic of Ireland.","authors":"Hanna Lythgoe, Eve M D Smith, Orla G Killeen, Ruth Murphy, Clarissa Pilkington, Clare E Pain, Michael W Beresford","doi":"10.1093/rheumatology/keac064","DOIUrl":"https://doi.org/10.1093/rheumatology/keac064","url":null,"abstract":"<p><strong>Objectives: </strong>The primary objective was to define the incidence of JSLE in children <16 years of age in the UK and Republic of Ireland (ROI). The secondary objective was to describe presenting features, classification criteria, initial management and disease damage in newly presenting JSLE patients.</p><p><strong>Methods: </strong>A prospective JSLE epidemiological study was undertaken between September 2017 and September 2019 with support of the British Paediatric Surveillance Unit and other professional groups involved in diagnosis and management of JSLE patients. Treating consultants reported all cases of JSLE seen. A follow-up study at 1 year examined management and progression of disease and treatment.</p><p><strong>Results: </strong>There were 124 incident cases included in the final analysis. Incidence was estimated using ACR-1997 classification criteria (0.36/100 000), SLICC-2012 classification criteria (0.41/100 000) and clinician expert opinion (0.46/100 000). A high disease burden was seen, with 71.0% of patients requiring ongoing systemic CS treatment at 1 year; 98.2% receiving immunomodulatory treatment; and 20.4% accruing damage in the year following diagnosis (predominantly neuropsychiatric-related), with substantial involvement from multiple speciality teams.</p><p><strong>Conclusions: </strong>The minimum UK and ROI incidence of JSLE is between 0.36 and 0.46/100 000, depending on the case definition used. Challenges in classification of patients with JSLE are highlighted, but overall this study supports the use of SLICC-2012 classification criteria. The high levels of disease damage and ongoing CS use 1 year after diagnosis is concerning, highlighting the need for further interventions to improve outcomes in JSLE.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"4097-4106"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39775976","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Michelle L M Mulder, Anouk M Bertram, Mark H Wenink, Johanna E Vriezekolk
{"title":"Defining the minimal important change (MIC) and meaningful change value (MCV) for the Psoriatic Arthritis Disease Activity Score (PASDAS) in a routine practice cohort of patients with psoriatic arthritis.","authors":"Michelle L M Mulder, Anouk M Bertram, Mark H Wenink, Johanna E Vriezekolk","doi":"10.1093/rheumatology/keac025","DOIUrl":"https://doi.org/10.1093/rheumatology/keac025","url":null,"abstract":"<p><strong>Objective: </strong>The aim of the study was to determine the Minimal Important Change (MIC) values and Meaningful Change (MCV) values for the Psoriatic Arthritis Disease Activity Score (PASDAS) and the Standard Error of Measurement (s.e.m.) of the PASDAS.</p><p><strong>Methods: </strong>The routine practice data for 544 patients with PsA was available for analysis. An anchor-based method using linear regression analyses was used to determine the MICs and MCVs for the PASDAS. With this anchor-based method, we compared changes in the PASDAS score with an external reference (anchor). The anchor question inquired whether the patient's well-being had changed since their previous visit. It consisted of a 7-point Likert scale (range: very much improved to very much deteriorated). Interperiod correlation matrix analysis was performed to determine the s.e.m.</p><p><strong>Results: </strong>The overall MIC and MCV for the PASDAS were 0.67 (95% CI: 0.55, 0.79) and 1.34 (95% CI: 1.21, 1.46), respectively. Results for improvement and deterioration were 0.65 (95% CI: 0.46, 0.83) and 0.71 (95% CI: 0.49, 0.93) for the MIC, respectively, and 1.29 (95% CI: 1.11, 1.48) and 1.42 (95% CI: 1.19, 1.64) for the MCV, respectively. The s.e.m. was determined at 0.81.</p><p><strong>Conclusion: </strong>The MIC for the PASDAS is a tool for physicians treating patients with PsA enabling them to give context to the patient's perspective of disease activity, while the MCV might aid the use of the PASDAS in PsA clinical trials.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"4119-4123"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39818354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Angeles S Galindo-Feria, Begum Horuluoglu, Jessica Day, Catia Fernandes-Cerqueira, Edvard Wigren, Susanne Gräslund, Susanna Proudman, Ingrid E Lundberg, Vidya Limaye
{"title":"Autoantibodies against four-and-a-half-LIM domain 1 (FHL1) in inflammatory myopathies: results from an Australian single-centre cohort.","authors":"Angeles S Galindo-Feria, Begum Horuluoglu, Jessica Day, Catia Fernandes-Cerqueira, Edvard Wigren, Susanne Gräslund, Susanna Proudman, Ingrid E Lundberg, Vidya Limaye","doi":"10.1093/rheumatology/keac003","DOIUrl":"https://doi.org/10.1093/rheumatology/keac003","url":null,"abstract":"<p><strong>Objectives: </strong>To determine the prevalence and associations of autoantibodies targeting a muscle-specific autoantigen, four-and-a-half-LIM-domain 1 (FHL1), in South Australian patients with histologically-confirmed idiopathic inflammatory myopathies (IIM) and in patients with SSc.</p><p><strong>Material and methods: </strong>Sera from patients with IIM (n = 267) from the South Australian Myositis Database (SAMD), SSc (n = 174) from the Australian Scleroderma Cohort Study (ASCS) and healthy controls (HC, n = 100) were analysed for anti-FHL1 autoantibodies by Enzyme-Linked ImmunoSorbent Assay (ELISA).</p><p><strong>Results: </strong>Autoantibodies to FHL1 were more frequent in patients with IIM (37/267, 13.8%) compared with SSc (12/174, 7%) (P < 0.02) and HC (2/100, 2%) (P < 0.001). The most common IIM subtypes among FHL1+ IIM patients were (32%) and IBM (2/37, 32%). No statistically significant differences in muscular or extra-muscular manifestations of IIM were found when comparing patients who were anti-FHL1+ with their anti-FHL1- counterparts. In 29/37 (78%) anti-FHL1+ patients, no myositis-specific autoantibodies (MSA) were present. In FHL1+ muscle biopsies, there was less frequent infiltration by CD45+ cells (P = 0.04). There was a trend for HLA alleles DRB1*07 and DRB1*15 to be more frequent in anti-FHL1+ compared with anti-FHL1- patients (9/25 vs 19/113, P = 0.09 and 8/25 vs 15/114, P = 0.09, respectively).</p><p><strong>Conclusions: </strong>We report a substantial prevalence (13.8%) of anti-FHL1 autoantibodies in a large cohort of patients with histologically confirmed IIM; 75% of these cases did not have a detectable myositis-specific autoantibody. Anti-FHL1 autoantibodies were also detected in a subgroup of patients with SSc (7%), indicating that anti-FHL1 autoantibodies may not be myositis-specific. The trend towards an HLA-DR association might indicate a specific immune response to the FHL1 protein.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"4145-4154"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/5e/2e/keac003.PMC9536793.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39817282","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pauline Morel, Alexandre Karras, Raphaël Porcher, Xavier Belenfant, Vincent Audard, Cédric Rafat, Guillaume Hanouna, Séverine Beaudreuil, Cédric Vilain, Aurélie Hummel, Benjamin Terrier, Evangeline Pillebout, Matthieu Groh, Romain Jouenne, Robin Dhote, Olivier Fain, Matthieu Ponsoye, Nicolas Noel, Nicolas Limal, Xavier Puéchal, Claire Le Jeunne, Loïc Guillevin, Luc Mouthon, Alexis Régent
{"title":"Management of severe renal disease in anti-neutrophil-cytoplasmic-antibody-associated vasculitis: the place of rituximab and plasma exchange?","authors":"Pauline Morel, Alexandre Karras, Raphaël Porcher, Xavier Belenfant, Vincent Audard, Cédric Rafat, Guillaume Hanouna, Séverine Beaudreuil, Cédric Vilain, Aurélie Hummel, Benjamin Terrier, Evangeline Pillebout, Matthieu Groh, Romain Jouenne, Robin Dhote, Olivier Fain, Matthieu Ponsoye, Nicolas Noel, Nicolas Limal, Xavier Puéchal, Claire Le Jeunne, Loïc Guillevin, Luc Mouthon, Alexis Régent","doi":"10.1093/rheumatology/keac046","DOIUrl":"https://doi.org/10.1093/rheumatology/keac046","url":null,"abstract":"<p><strong>Objective: </strong>The optimal induction therapy for severe glomerulonephritis of ANCA-associated vasculitis (AAV) is debated. We compared the efficacy of glucocorticoid and rituximab (RTX) or CYC induction therapy for severe AAV-related glomerulonephritis and evaluated the potential benefit of plasma exchange (PE) as adjunct therapy to CYC.</p><p><strong>Methods: </strong>This retrospective, multicentre study included AAV patients with severe renal active disease (serum creatinine level ≥350 µmol/l and/or estimated glomerular filtration ratio ≤15 ml/min/1.73 m2). Propensity-score analysis was used to adjust for potential confounders.</p><p><strong>Results: </strong>Between 2005 and 2017, 153 patients with AAV-related glomerulonephritis were studied (96 [60%] men; mean [s.d.] age 63 [13.1] years): 19 (12%) were treated with RTX and 134 (88%) with CYC. Remission rates did not differ between RTX- and CYC-treated groups. Although more patients with RTX than CYC were dialysis-free at month (M) 12 (79% vs 68%), the difference was not significant after adjustment. Among 134 patients with CYC-treated glomerulonephritis, 76 (57%) also had PE. M3 and M6 remission rates were comparable for weighted CYC groups with or without PE. For weighted groups, the dialysis-free survival rate with CYC was higher with than without PE at M6 (72% vs 64%; odds ratio 2.58) and M12 (74% vs 60%; odds ratio 2.78) reaching statistical significance at M12.</p><p><strong>Conclusion: </strong>We could not find any difference between RTX and CYC as induction therapy for patients with severe AAV-related glomerulonephritis. In patients receiving CYC induction regimen, the addition of PE conferred short-term benefits with higher dialysis-free rate at M12.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"4056-4064"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39757913","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}