{"title":"Osteopoikilosis in a young ankylosing spondylitis patient.","authors":"Vitaly Omelchenko, Elena Letyagina, Maxim Korolev","doi":"10.4078/jrd.2024.0040","DOIUrl":"10.4078/jrd.2024.0040","url":null,"abstract":"<p><p>Osteopoikilosis (OPK) is a rare benign congenital genetic-mediated sclerosing skeletal disease, characterized by the formation of osteosclerosis foci. OPK is usually clinically asymptomatic, but some patients (15%~20%) may have arthralgia and synovitis. OPK may be associated with rheumatic diseases and might lead to unreasonable over-examination in real clinical practice. Single cases of the OPK together with ankylosing spondylitis (AS) have been described. Here we present a 33-year-old patient diagnosed with AS coexisting with OPK. In the case considered, the combination of AS and OPK accompanied with a high activity of inflammation, peripheral arthritis, a rapid rate of structural progression in axial skeleton, inefficiency of disease-modifying antirheumatic drugs and nonsteroidal anti-inflammatory drugs, a lack of response to anti interleukin-17 and a good response to a tumor necrosis factor inhibitor golimumab. We describe the important points of differential diagnosis associated with the identification of focal changes in bone tissue, especially neoplastic lesion. Foci revealed had typical localization, so, acquaintance of practicing doctors with such rare cases would minimize unnecessary examinations.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 4","pages":"253-256"},"PeriodicalIF":2.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11439639/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142360701","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}
Min Jung Kim, Jun Won Park, Sun-Kyung Lee, Yumi Jang, Soyoung Kim, Matthias Stoelzel, Jonathan Lumen Chua, Kichul Shin
{"title":"Corrigendum: Treatment sequence after initiating biologic therapy for patients with rheumatoid arthritis in Korea: a nationwide retrospective cohort study.","authors":"Min Jung Kim, Jun Won Park, Sun-Kyung Lee, Yumi Jang, Soyoung Kim, Matthias Stoelzel, Jonathan Lumen Chua, Kichul Shin","doi":"10.4078/jrd.22.0024C","DOIUrl":"https://doi.org/10.4078/jrd.22.0024C","url":null,"abstract":"<p><p>[This corrects the article on p. 26 in vol. 30, PMID: 37476522.].</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 4","pages":"263"},"PeriodicalIF":2.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11439631/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142360699","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}
{"title":"Application of disease activity index in rheumatoid arthritis management in Korea.","authors":"Se Rim Choi, Soo-Kyung Cho, Yoon-Kyoung Sung","doi":"10.4078/jrd.2024.0077","DOIUrl":"10.4078/jrd.2024.0077","url":null,"abstract":"<p><p>Effective management of rheumatoid arthritis (RA) necessitates the accurate measurement of disease activity using a treat-to-target strategy established as a cornerstone approach. Disease activity assessment tools such as the Disease Activity Score in 28 joints (DAS28), Simplified Disease Activity Index, Clinical Disease Activity Index, and Routine Assessment of Patient Index Data 3 have been internationally validated and recognised. In Korea, the government initiated a quality assessment program mandating routine measurement of DAS28 to ensure high-quality RA management. However, whether the DAS28 is the most suitable disease activity measurement tool in the Korean clinical environment is a topic worth considering. In this review, we comprehensively examined disease activity measurement tools and their performance in the Korean context. We also propose a new strategy for measuring RA disease activity, tailored to the different situations encountered by physicians in routine clinical practice. This review may contribute to the improvement of the quality of care for patients with RA in Korea.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 4","pages":"193-199"},"PeriodicalIF":2.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11439630/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142360698","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}
Ah-Ra Choi, Ki-Jeong Park, Ji-Hyoun Kang, Yu Jeong Lee, Hyun Hee Jang, Moon-Ju Kim, Tae-Jong Kim
{"title":"The effectiveness of tumor necrosis factor-α blocker therapy in patients with axial spondyloarthritis who failed conventional treatment: a comparative study focused on improvement in ASAS Health Index.","authors":"Ah-Ra Choi, Ki-Jeong Park, Ji-Hyoun Kang, Yu Jeong Lee, Hyun Hee Jang, Moon-Ju Kim, Tae-Jong Kim","doi":"10.4078/jrd.2024.0029","DOIUrl":"10.4078/jrd.2024.0029","url":null,"abstract":"<p><strong>Objective: </strong>The purpose of this study is to evaluate the impact of tumor necrosis factor (TNF)-α blocker therapy on the Assessment of SpondyloArthritis international Society Health Index (ASAS-HI) among patients who have failed conventional nonsteroidal anti-inflammatory drugs.</p><p><strong>Methods: </strong>A comparative study was conducted involving axial spondyloarthritis (axSpA) patients treated with either TNF-α blocker or conventional therapy. Patient data, including demographics, disease characteristics, and ASAS-HI scores, were collected before and after treatment. Statistical analysis was performed to compare changes in ASAS-HI scores between the TNF-α blocker and the conventional therapy group.</p><p><strong>Results: </strong>The study population consisted of patients with axSpA, with a mean age of 38.3 years in conventional treatment group and 29.3 years in TNF-α blocker group. Most variables, including C-reactive protein levels, other comorbidities, and disease assessment scores showed no significant difference between groups. Longitudinal analysis within each treatment group from Week 0 to 12 showed no significant change in the conventional treatment group, whereas the TNF-α blocker group experienced a significant reduction in ASAS-HI scores, demonstrating the effectiveness of the treatment. The TNF-α blocker group exhibited a significantly greater improvement in ASAS-HI scores compared to the conventional therapy group. The Bath Ankylosing Spondylitis Functional Index and the Bath Ankylosing Spondylitis Disease Activity Index demonstrated strong positive correlations with ASAS-HI scores, indicating higher disease activity and functional limitation are associated with worse health outcomes in patients.</p><p><strong>Conclusion: </strong>The research demonstrates that ASAS-HI scores significantly improve with TNF-α blocker therapy in axSpA patients, underscoring ASAS-HI's effectiveness as a tool for evaluating drug responses.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 3","pages":"171-177"},"PeriodicalIF":2.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11215249/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494426","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}
{"title":"Clinical characteristics and prognostic value of autoantibody profile in children with monogenic lupus.","authors":"Sulaiman M Al-Mayouf, Alaa Hamad, Wassima Kaidali, Raghad Alhuthil, Alhanouf Alsaleem","doi":"10.4078/jrd.2023.0065","DOIUrl":"10.4078/jrd.2023.0065","url":null,"abstract":"<p><strong>Objective: </strong>To report the frequency of selected autoantibodies and their associations with clinical features in Arab children with monogenic lupus.</p><p><strong>Methods: </strong>This study was retrospective single-center study of genetically confirmed monogenic lupus cases at childhood lupus clinic at King Faisal Specialist Hospital and Research Center, from June 1997 to July 2022. We excluded familial lupus without genetic testing and patients with insufficient data. Collected data comprised clinical and laboratory findings, including the autoantibody profile, which included the anti-double-stranded DNA (anti-dsDNA), anti-Smith, anti-Sjögren's-syndrome-related antigen A (anti-SSA), anti-Sjögren's-syndrome-related antigen B (anti-SSB), and antiphospholipid (APL) antibodies. Also, disease activity and accrual disease damage were collected at the last follow-up visit.</p><p><strong>Results: </strong>This study enrolled 27 Arab patients (14 males) with a median age of 11 years (interquartile range 8.0~16 years), with 63% having early-onset disease. The consanguinity rate and family history of lupus were high (74.1% and 55.6%, respectively). The most frequent clinical features were hematological (96.3%), fever (81.5%), mucocutaneous lesions (85.2%), and renal (66.7%). The frequency of the APL antibodies was 59.3%, anti-dsDNA was 55.6%, and anti-Smith and anti-SSA were 48.2% and 44.4%, respectively. Moreover, dsDNA antibodies were significantly associated with musculoskeletal complaints (p<0.05). Likewise, both anti-Smith and anti-SSA antibodies were linked to failure to thrive and recurrent infections in the univariate analysis (p<0.05).</p><p><strong>Conclusion: </strong>Our study reveals autoantibody frequencies and their association with clinical and prognostic in a substantial monogenic lupus cohort. Distinct clinical manifestations and prognosis association with certain autoantibodies support the idea that monogenic lupus is a distinctive form of lupus. Larger studies needed to validate these findings.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 3","pages":"143-150"},"PeriodicalIF":2.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11215254/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494422","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}
Frederico Rajão Martins, Margarida Lucas Rocha, Ana Teodósio Chícharo, Vítor Silvestre-Teixeira
{"title":"Jaccoud's arthropathy in osteogenesis imperfecta.","authors":"Frederico Rajão Martins, Margarida Lucas Rocha, Ana Teodósio Chícharo, Vítor Silvestre-Teixeira","doi":"10.4078/jrd.2023.0090","DOIUrl":"10.4078/jrd.2023.0090","url":null,"abstract":"","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 3","pages":"188-189"},"PeriodicalIF":2.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11215248/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494425","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}
{"title":"Corticosteroid-free adalimumab-cyclophosphamide combination therapy for acute phase neuro-Behçet's disease: a case report.","authors":"Ji Hyoun Kim, Sang Wan Chung, Yun Jong Lee","doi":"10.4078/jrd.2023.0069","DOIUrl":"10.4078/jrd.2023.0069","url":null,"abstract":"<p><p>Neuro-Behçet's disease (NBD) represents a significant complication of Behçet's syndrome, potentially leading to elevated mortality and disability rates. The standard treatment for parenchymal NBD typically entails administering high-dose corticosteroids to prompt rapid-onset effects, coupled with immunosuppressants to prevent subsequent relapses. A 48-year-old male with NBD presented with progressively worsening dysarthria over 9 months. This patient experienced increased intraocular pressure while using glucocorticoids, which worsened his pre-existing glaucoma. The patient had a prior diagnosis of NBD and presented with progressive dysarthria over a period of nine months, leading to a brain magnetic resonance imaging (MRI) scan. The brain MRI revealed multifocal punctate high signal intensities in the left frontoparietal area, insula, and basal ganglia. Instead of the standard steroid pulse therapy, the patient received adalimumab-cyclophosphamide combination as an alternative induction therapy. Subsequent serial brain MRI scans exhibited no emergence of new lesions, and the patient remained devoid of clinical relapses even after 17 months from the commencement of induction treatment. Adalimumab-cyclophosphamide combination could be used as a corticosteroid-free induction strategy for NBD. Further investigations are warranted to establish the most suitable combination regimen.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 3","pages":"178-181"},"PeriodicalIF":2.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11215246/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494423","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}
{"title":"Effect of recombinant human bone morphogenetic protein-2 and osteoprotegerin-Fc in MC3T3-E1 cells: beyond challenges to success.","authors":"Chang Hoon Lee","doi":"10.4078/jrd.2024.0079","DOIUrl":"10.4078/jrd.2024.0079","url":null,"abstract":"","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 3","pages":"133-134"},"PeriodicalIF":2.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11215253/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494424","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}
Jang Woo Ha, Sung Soo Ahn, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
{"title":"The monocyte-to-high-density lipoprotein-cholesterol ratio at diagnosis is associated with cerebrovascular accident during follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis.","authors":"Jang Woo Ha, Sung Soo Ahn, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee","doi":"10.4078/jrd.2024.0001","DOIUrl":"10.4078/jrd.2024.0001","url":null,"abstract":"<p><strong>Objective: </strong>In this study, the association between the monocyte-to-high-density lipoprotein cholesterol ratio (MHR) at diagnosis and poor outcomes of atherosclerosis-related antineutrophil cytoplasmic antibody-associated vasculitis (AAV) during follow-up in patients with AAV was investigated.</p><p><strong>Methods: </strong>This retrospective study included 138 patients diagnosed with AAV. Their comprehensive medical records were meticulously reviewed. All-cause mortality, cerebrovascular accident (CVA), and acute coronary syndrome (ACS) were evaluated as atherosclerosis-related poor outcomes of AAV. MHR was obtained by dividing monocyte counts (/mm3) by high-density lipoprotein cholesterol (mg/dL) levels.</p><p><strong>Results: </strong>The median age of the 138 patients was 58.3 years with 44 being male (31.9%). Among the 138 patients, 11 (8.0%) died, and 11 (8.0%) and 9 (6.5%) had CVA, and ACS, respectively. MHR at diagnosis was significantly correlated with the Birmingham vasculitis activity score, erythrocyte sedimentation rate, and C-reactive protein at diagnosis. Among the three poor outcomes of AAV, only CVA during follow-up was significantly associated with MHR at diagnosis, and thus, only CVA was considered an atherosclerosis-related poor outcome of AAV. In the multivariable Cox hazards model analysis, MHR (hazard ratio [HR] 1.195) and serum albumin (HR 0.203) at diagnosis were independently associated with CVA during follow-up. Additionally, patients with MHR at diagnosis ≥3.0 exhibited a significantly higher risk for CVA and lower cumulative CVA-free survival rate than those with MHR at diagnosis <3.0.</p><p><strong>Conclusion: </strong>This study is the first to demonstrate clinical implications of MHR suggesting that MHR at diagnosis is significantly and independently associated with CVA during follow-up in patients with AAV.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"31 3","pages":"151-159"},"PeriodicalIF":2.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11215250/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141494427","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}