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  • Editorial 2024-07-01

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    Effect of recombinant human bone morphogenetic protein-2 and osteoprotegerin-Fc in MC3T3-E1 cells: beyond challenges to success

    Chang Hoon Lee , M.D., Ph.D.

    J Rheum Dis 2024; 31(3): 133-134
  • Review Article 2024-07-01

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    Overview of childhood vasculitis

    Jong Gyun Ahn , M.D., Ph.D.

    J Rheum Dis 2024; 31(3): 135-142
    Abstract

    Abstract : Pediatric vasculitis and adult vasculitis differ in several aspects. While both involve inflammation of blood vessels, pediatric vasculitis tends to present with distinct clinical features and may involve different types of blood vessels compared to adult vasculitis. Despite its relatively rare occurrence compared to adult vasculitis, pediatric vasculitis warrants careful attention due to its potential for profound and diverse clinical manifestations, ranging from mild cutaneous symptoms to life-threatening systemic complications. Childhood vasculitis should be suspected in children who present symptoms attributable to systemic inflammation and complications arising from multi-organ dysfunction. However, achieving a diagnosis necessitates thorough exclusion of alternative conditions manifesting similar symptoms and findings. Hence, children suspected of vasculitis should undergo meticulous history-taking, comprehensive physical examination, and requisite laboratory investigations, imaging studies, and sometimes tissue biopsies to elucidate the diagnosis. Early detection and treatment of childhood vasculitis are crucial, as the condition can affect various organs and potentially lead to life-threatening complications or long-term sequelae in adulthood if left untreated. This review aimed to provide an exhaustive overview of childhood vasculitis, outlining its epidemiology, classification, clinical presentation, diagnostic modalities, therapeutic strategies and outcome.

  • Original Article 2024-07-01

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    Clinical characteristics and prognostic value of autoantibody profile in children with monogenic lupus

    Sulaiman M. Al-Mayouf , M.D., Alaa Hamad , M.D., Wassima Kaidali , M.D., Raghad Alhuthil , M.S., Alhanouf Alsaleem , M.D.

    J Rheum Dis 2024; 31(3): 143-150
    Abstract

    Abstract : Objective: To report the frequency of selected autoantibodies and their associations with clinical features in Arab children with monogenic lupus.
    Methods: 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.
    Results: 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).
    Conclusion: 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.

  • Original Article 2024-07-01

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    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

    Jang Woo Ha , M.D., Ph.D., Sung Soo Ahn , M.D., Ph.D., Jason Jungsik Song , M.D., Ph.D., Yong-Beom Park , M.D., Ph.D., Sang-Won Lee , M.D., Ph.D.

    J Rheum Dis 2024; 31(3): 151-159
    Abstract

    Abstract : Objective: 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.
    Methods: 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.
    Results: 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.
    Conclusion: 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.

  • Original Article 2024-07-01

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    Clinical characteristics and courses of Korean patients with giant cell arteritis: a multi-center retrospective study

    Jee-In Lee , M.D., Jun Won Park , M.D., Youjin Jung , M.D., Kichul Shin , M.D., Ph.D., Se Rim Choi , M.D., Eun Ha Kang , M.D., Ph.D., M.P.H., Yun Jong Lee , M.D., Ph.D., Jong Jin Yoo , M.D., Ph.D., You-Jung Ha , M.D., Ph.D.

    J Rheum Dis 2024; 31(3): 160-170
    Abstract

    Abstract : Objective: Giant cell arteritis (GCA) is a large-vessel vasculitis that primarily affects elderly individuals. However, data regarding Korean patients with GCA are scarce owing to its extremely low prevalence in East Asia. This study aimed to investigate the clinical characteristics of Korean patients with GCA and their outcomes, focusing on relapse.
    Methods: The medical records of 27 patients with GCA treated at three tertiary hospitals between 2007 and 2022 were retrospectively reviewed.
    Results: Seventeen (63.0%) patients were females, and the median age at diagnosis was 75 years. Large vessel involvement (LVI) was detected in 12 (44.4%) patients, and polymyalgia rheumatica (PMR) was present in 14 (51.9%) patients. Twelve (44.4%) patients had fever at onset. The presence of LVI or concurrent PMR at diagnosis was associated with a longer time to normalization of the C-reactive protein level (p=0.039) or erythrocyte sedimentation rate (p=0.034). During follow-up (median: 33.8 months), four (14.8%) patients experienced relapse. Kaplan-Meier analyses showed that relapse was associated with visual loss (p=0.008) and the absence of fever (p=0.004) at onset, but not with LVI or concurrent PMR.
    Conclusion: Concurrent PMR and LVI were observed in approximately half of Korean patients with GCA, and the elapsed time to normalization of inflammatory markers in these patients was longer. The relapse rate in Korean GCA is lower than that in Western countries, and afebrile patients or patients with vision loss at onset have a higher risk of relapse, suggesting that physicians should carefully monitor patients with these characteristics.

  • Original Article 2024-07-01

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    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

    Ah-Ra Choi , M.S., Ki-Jeong Park , M.D., Ji-Hyoun Kang , M.D., Ph.D., Yu Jeong Lee , M.S., Hyun Hee Jang , M.S., Moon-Ju Kim , Ph.D., Tae-Jong Kim , M.D., Ph.D.

    J Rheum Dis 2024; 31(3): 171-177
    Abstract

    Abstract : Objective: 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.
    Methods: 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.
    Results: 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.
    Conclusion: 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.

  • Case Report 2024-07-01

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    Corticosteroid-free adalimumab-cyclophosphamide combination therapy for acute phase neuro-Behçet’s disease: a case report

    Ji Hyoun Kim , M.D., Sang Wan Chung , M.D., Ph.D., Yun Jong Lee , M.D., Ph.D.

    J Rheum Dis 2024; 31(3): 178-181
    Abstract

    Abstract : 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.

  • Case Report 2024-07-01

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    Unraveling the diagnostic odyssey: stimulator of interferon gene-associated vasculopathy with onset in infancy in a 30-year-old female

    Hae Ryung Kim , M.D., Seon Hee Lim , M.D., Ji Soo Park , M.D., Dong In Suh , M.D., Ph.D., Seungbok Lee , M.D., Soo Yeon Kim , M.D., Jong Hee Chae , M.D., Ph.D., Seong Heon Kim , M.D.

    J Rheum Dis 2024; 31(3): 182-187
    Abstract

    Abstract : Stimulator of interferon gene (STING)-associated vasculopathy with onset in infancy (SAVI) is an extremely rare autoinflammatory disease. We present the case of a female Korean patient with early-onset interstitial lung disease who was initially suspected to have systemic lupus erythematosus (SLE) but was ultimately diagnosed with SAVI. The patient exhibited signs of interstitial lung disease and cutaneous manifestations before the age of 1 year and continued to have recurrent fever accompanied by pulmonary infiltrates. Based on positive findings for antibodies associated with SLE, such as antinuclear antibodies and anti-double-stranded DNA, the pulmonary involvement was considered a manifestation of SLE. Another significant symptom was recurrent skin ulceration, which led to partial spontaneous amputation of most of the toes due to inflammation. Given the early onset of interstitial lung disease, severe skin ulcers, and symptoms resembling SLE, autoinflammatory syndrome, especially SAVI was suspected. Following confirmation by genetic testing at age 29 years, the patient was started on tofacitinib, a Janus kinase inhibitor. Despite the prolonged use of multiple immunosuppressive therapies, the patient’s lung condition continued to worsen, ultimately requiring lung transplantation. This observational report highlights the importance of considering SAVI as a potential diagnosis when manifestations of interstitial lung disease are observed during infancy. Early proactive treatment is crucial for lung involvement, as this can have long-term effects on patient’s prognosis.

  • Clinical Image 2024-07-01

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    Jaccoud’s arthropathy in osteogenesis imperfecta

    Frederico Rajão Martins , M.D., Margarida Lucas Rocha , M.D., Ana Teodósio Chícharo , M.D., Vítor Silvestre-Teixeira , M.D.

    J Rheum Dis 2024; 31(3): 188-189
JRD
Jul 01, 2024 Vol.31 No.3, pp. 133~189
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pISSN 2093-940X
eISSN 2233-4718
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