Simran Heera, Matthew Sholdice, Julie Herrington, Tania Cellucci, Stephanie Garner, Liane Heale, Mark Matsos, Karen Beattie, Michelle Batthish
{"title":"Lost in Transition: Examining Loss to Follow-up in JIA and cSLE.","authors":"Simran Heera, Matthew Sholdice, Julie Herrington, Tania Cellucci, Stephanie Garner, Liane Heale, Mark Matsos, Karen Beattie, Michelle Batthish","doi":"10.1097/RHU.0000000000002276","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002276","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145126918","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}
Rajaie Namas, Sarah Al Qassimi, Mohamed Elarabi, Saniya Khan, Ahlam Almarzooqi, Asia Mubashir, Waleed Hafiz, Jeffery Chapman
{"title":"Real-world Outcomes and Tolerability of Nintedanib in Patients With Systemic Autoimmune Rheumatic Disease-associated Interstitial Lung Disease: A Retrospective Study From a Single Center in a Middle Eastern Population.","authors":"Rajaie Namas, Sarah Al Qassimi, Mohamed Elarabi, Saniya Khan, Ahlam Almarzooqi, Asia Mubashir, Waleed Hafiz, Jeffery Chapman","doi":"10.1097/RHU.0000000000002281","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002281","url":null,"abstract":"<p><strong>Background: </strong>Nintedanib, a tyrosine kinase inhibitor with antifibrotic properties, has been shown to significantly slow the progression of pulmonary fibrosis. The aim of this study is to assess the clinical characteristics, longitudinal pulmonary function tests, serial high-resolution computed tomography (HRCT) findings, and the efficacy, tolerability, and outcomes of nintedanib treatment in patients with systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD) within a Middle Eastern population.</p><p><strong>Patients and methods: </strong>This is a cohort study conducted between May 2015 and March 2024, all patients with SARD-ILD treated with nintedanib were analyzed. Demographic and clinical data were collected before the initiation of nintedanib. Serial changes in percentage predicted forced vital capacity (ppFVC) and percentage predicted diffusing capacity for carbon monoxide (ppDLCO), as well as HRCT findings, were recorded at 6, 12, and 24 months posttreatment initiation. Adverse events of nintedanib were documented. Descriptive statistics were employed for data analysis.</p><p><strong>Results: </strong>A total of 47 patients with SARD-ILD received nintedanib. The most common diagnoses were systemic sclerosis (36.2%) and rheumatoid arthritis (17%). Over 24 months, patients showed stable or slightly improved ppFVC and ppDLCO values, and 89% had stable ILD on HRCT. Gastrointestinal adverse events were reported in 27.7% of patients. Five patients (10.6%) underwent lung transplantation, with an average time of 3.2 ± 2.2 years from treatment initiation to transplantation.</p><p><strong>Conclusion: </strong>Our findings indicate that nintedanib is well-tolerated, with no new safety concerns identified. In addition, we observed clinically meaningful improvements in pulmonary function tests by 6 months, which were sustained through 24 months.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145081791","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}
Marlon J Sandino-Bermúdez, Ana Sarahí Mulia-Soto, Juan M Mejía-Vilet, Eduardo Martín-Nares, Agustín Hernández-López, Andrea Hinojosa-Azaola
{"title":"Factors Associated With Relapses and Performance of the French Vasculitis Study Group Relapse Score in a Cohort of Mexican Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.","authors":"Marlon J Sandino-Bermúdez, Ana Sarahí Mulia-Soto, Juan M Mejía-Vilet, Eduardo Martín-Nares, Agustín Hernández-López, Andrea Hinojosa-Azaola","doi":"10.1097/RHU.0000000000002282","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002282","url":null,"abstract":"<p><strong>Background/objective: </strong>Relapses occur in 14% to 44% of patients with antineutrophil cytoplasm antibody-associated vasculitis (AAV). The French Vasculitis Study Group Relapse Score (FRS) was recently proposed to predict relapse risk. This study aimed to identify relapse-associated factors and evaluate the FRS performance in a Mexican cohort.</p><p><strong>Patients and methods: </strong>We performed a medical records review study including patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) who were followed for ≥12 months at a Mexican tertiary care center. Demographic, clinical, laboratory, and treatment data were analyzed using descriptive statistics, survival analysis, and ROC curves.</p><p><strong>Results: </strong>Among 147 patients (110 GPA, 37 MPA), the median age at diagnosis was 49 years (IQR: 36 to 59). Over a median follow-up of 93 months (IQR: 48 to 152), 90 patients (61%) relapsed. Cumulative relapse rates at 12, 24, 36, 48, and 60 months were 13.6%, 32.3%, 40.3%, 47.5%, and 58.0%, respectively. FRS scores of 1, 2, and 3 corresponded to median relapse-free survivals of 85, 68, and 33 months, with 5-year relapse risks of 40.5%, 48.4%, and 68.3%, respectively. Discrimination was significant (log-rank p < 0.0004). The C-statistic for FRS alone was 0.648 (95% CI: 0.586-0.710); for model 1 (adding cluster 4), 0.666 (95% CI: 0.605-0.728); and for model 2 (adding cluster 4 and rituximab as maintenance), 0.700 (95% CI: 0.643-0.757). An age cutoff of ≤50 years showed better accuracy (AUC: 0.67, p = 0.0006) for relapse prediction.</p><p><strong>Conclusions: </strong>In this cohort, relapses were frequent. Incorporating clinical clusters and rituximab therapy to the FRS may enhance its predictive performance.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145071591","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}
Gregory J Challener, Mohamad El Labban, Amjad N Kanj, Gabriel E Ortiz Jaimes, Sarah B Leung, Jay H Ryu, Misbah Baqir
{"title":"Pneumocystis jirovecii Pneumonia in Patients Without HIV, Transplant, or Cancer: Missed Opportunities for Prevention.","authors":"Gregory J Challener, Mohamad El Labban, Amjad N Kanj, Gabriel E Ortiz Jaimes, Sarah B Leung, Jay H Ryu, Misbah Baqir","doi":"10.1097/RHU.0000000000002279","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002279","url":null,"abstract":"<p><strong>Background: </strong>Pneumocystis jirovecii pneumonia (PJP) is life-threatening for immunocompromised patients. No consensus exists on PJP prophylaxis for immunosuppressed patients without HIV, transplant, or cancer.</p><p><strong>Methods: </strong>We retrospectively reviewed the electronic health records of adult immunosuppressed patients with PJP diagnosed between 1990 and 2020 at Mayo Clinic. Patients with HIV, solid organ transplants, or cancer were excluded. Demographic data, treatments, and outcomes were manually abstracted.</p><p><strong>Results: </strong>The most common indications for immunosuppression were rheumatoid arthritis (19.7%), vasculitis (18.1%), and interstitial lung disease (ILD) not related to connective tissue disease (17.6%). Despite having high risk of PJP, 86.0% of patients did not receive PJP prophylaxis. Corticosteroids were the most common immunosuppressive agent used (84.5%), with 64.4% of patients receiving high-dose treatment. Nonbiologic disease-modifying antirheumatic drugs were used for 49.7%, including methotrexate (51.0%), azathioprine (22.9%), and hydroxychloroquine (11.5%). Biologics were prescribed for 25.4%, primarily rituximab (59.2%) and infliximab (22.4%). Hospitalization occurred for 76.7% of patients; 70.3% required intensive care unit (ICU) admission, and 46.6% received mechanical ventilation. The in-hospital mortality rate was 30.4% overall and 53.6% for patients on ventilation. Predictors of death included ILD [odds ratio (OR), 4.61; 95% CI, 1.75-13.00], ICU admission (OR, 3.60; 95% CI, 1.19-11.08), and ventilator use (OR, 3.46; 95% CI, 1.30-9.79). Biologic use was associated with lower odds of death (OR, 0.34; 95% CI, 0.11-0.89).</p><p><strong>Conclusions: </strong>Most patients in our cohort did not receive PJP prophylaxis, and outcomes were poor with high mortality rates. Standardized risk stratification and prophylaxis protocols are needed to improve outcomes.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145071594","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}
Li Shanshan, Wang Xiaoxing, Zhang Ling, Duan Jianghui, Liu Xia, Zhang Lu
{"title":"Prophylaxis With Valganciclovir in Anti-Melanoma Differentiation Associated 5 Gene Antibody Dermatomyositis: A Cohort Study in China.","authors":"Li Shanshan, Wang Xiaoxing, Zhang Ling, Duan Jianghui, Liu Xia, Zhang Lu","doi":"10.1097/RHU.0000000000002274","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002274","url":null,"abstract":"<p><strong>Background: </strong>Cytomegalovirus (CMV) infection is prevalent in patients with anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis (MDA5+DM) and affects the prognosis. This study aimed to evaluate the efficacy of valganciclovir prophylaxis during MDA5+DM treatment.</p><p><strong>Methods: </strong>Adult patients with active MDA5+DM were consecutively recruited from May 1, 2023 to December 31, 2023, and followed up for 6 months. Participants were categorized into 2 groups based on whether they received valganciclovir or not. The incidence of CMV infection and changes in disease activity were analyzed.</p><p><strong>Results: </strong>This study included 49 patients with active MDA5+DM. Prophylaxis with valganciclovir was administered to 18 patients, including 9 patients with rapidly progressive interstitial lung disease (RP-ILD). Of the remaining 31 patients, 15 had RP-ILD. During the follow-up, no CMV infection, discomfort, or abnormal laboratory findings associated with valganciclovir were observed during the prophylaxis period. Nine patients in the control group were infected with CMV (p = 0.032). In the survival analysis, 2 and 9 patients with RP-ILD died in the valganciclovir and control groups, respectively (p = 0.084). No significant difference in disease activity and glucocorticoid dosage was observed between two groups at 3 and 6 months. Furthermore, the use of biological and targeted synthetic disease-modifying antirheumatic drugs at the initiation of treatment was identified as a risk factor for CMV infection in active MDA5+DM patients in the control group (p = 0.007).</p><p><strong>Conclusion: </strong>Prophylaxis with valganciclovir can reduce the incidence of CMV infection during MDA5+DM treatment. It exerts a potential auxiliary effect on MDA5+DM treatment.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145071605","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":"Psoriatic Arthritis Sine Psoriasis With Severe Deformity: Don't Miss Nail Involvement!","authors":"Yoshinori Taniguchi, Hirotaka Yamamoto","doi":"10.1097/RHU.0000000000002278","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002278","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145042908","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":"Letter to the Editor: Childhood Familial Mediterranean Fever in the USA: Spectrum of Clinical Phenotypes and MEFV Genotypes in a Cohort From Southeast Michigan.","authors":"Ibrahim Nagmeldin Hassan","doi":"10.1097/RHU.0000000000002259","DOIUrl":"10.1097/RHU.0000000000002259","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e132"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144556518","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}