Idiopathic inflammatory myopathies (IIMs) are heterogeneous immune-mediated muscle disorders with variable extramuscular manifestations and outcomes. Myositis-specific auto-antibodies (MSAs) have transformed the IIM field by enabling a serology-informed taxonomy based on distinct clinical phenotypes, prognostic trajectories, and therapeutic responses. Anti-Jo-1 and other antisynthetase antibodies characterize interstitial lung disease (ILD)-prone forms of disease; anti-MDA5 identifies patients at risk of rapidly progressive ILD and early mortality; anti-TIF1-γ and anti-NXP2 are indicative of cancer-associated myositis; anti-SRP and anti-HMGCR delineate necrotizing myopathies; and anti-cN1A supports the diagnosis of inclusion-body myositis. Beyond diagnosis, MSA profiles provide prognostic information, including about survival, relapse risk, and organ-specific complications, and they are increasingly being used to guide treatment decisions and predict biologic responsiveness. This review summarizes the diagnostic, prognostic, and therapeutic implications of MSAs across the IIM spectrum and highlight their growing importance in clinical practice for risk stratification and patient management.

Background/Aims: Patients with postural tachycardia syndrome (POTS) commonly experience gastrointestinal (GI) symptoms. We aim to assess the feasibility and preliminary efficacy data for DA-9701, a prokinetic agent targeting 5-hy-droxytryptamine 1A, 5-hydroxytryptamine 4, and dopamine D 2 receptors, in patients with POTS. Methods: In a randomized, double-blind, placebo-controlled, single-center crossover trial, patients with POTS were given either 30 mg of DA-9701 or a placebo 3 times daily for eight weeks in a 1:1 ratio. After a 4-week washout, patients received the alternate treatment for another 8 weeks. The primary endpoint focused on assessing the change in GI symptoms (total Nepean Dyspepsia Index-Korean version [NDI-K] symptom score) from baseline over the 8 week-treatment period. Endpoints were assessed in all enrolled and randomized patients (intention-to-treat), and in those who completed the trial (per-protocol analysis). Results: Between January 2022 and August 2023, 24 patients were randomized (n = 12 per group), with 3 discontinuing after randomization. DA-9701 did not significantly improve primary endpoints for total NDI-K symptom scores in either the intention-to-treat (least-squares means, –13.9 vs. –9.5, P = 0.326) or per-protocol analyses (–17.2 vs –12.0, P = 0.242).Notably, a trend toward improvement in specific GI symptoms, such as upper abdominal pain, was observed in both intention-to-treat (–0.6 vs 0.7; P = 0.066) and per-protocol analyses (–0.9 vs 0.6; P = 0.045). No serious adverse events were observed. Conclusion DA-9701 did not improve GI symptoms in this crossover trial; however, its potential effect on specific GI symptoms merits further investigation.

Background: Recombinant human follicle-stimulating hormone (rhFSH) is commonly used to treat female infertility, but its short half-life necessitates multiple doses. Even corifollitropin alfa, with an extended half-life, requires supplementary injections of rhFSH after 7 days. This study aimed to develop and evaluate a long-acting follicle-stimulating hormone (FSH) formulation using anti-serum albumin Fab-associated (SAFA) technology to avoid additional injections and enhance ovarian function. Methods: SAFA-FSH was synthesized using a Chinese hamster ovary expression system. Its biological efficacy was confirmed through assays measuring its ability to stimulate cyclic adenosine monophosphate (cAMP) production, estradiol synthesis, and the expression of human cytochrome P450 family 19 subfamily A member 1 (hCYP19α1) and human steroidogenic acute regulatory protein (hSTAR) in human ovarian granulosa (KGN) cells. To evaluate the effects of SAFA-FSH, we compared its impact on serum estradiol levels and ovarian weight increase with that of rhFSH in Sprague-Dawley (SD) rats using the modified Steelman-Pohley test. Results: The results indicated that SAFA-FSH induces cAMP synthesis in KGN cells and upregulates the expression of hCYP19α1 and hSTAR in a dose-dependent manner. Female SD rats, aged 21 days, receiving daily subcutaneous human chorionic gonadotropin injections for 5 days exhibited a significant increase in serum estradiol levels and ovarian weight when administered SAFA-FSH on the first day or when given nine injections of rhFSH over 5 days. Notably, the group receiving SAFA-FSH on the first and third days demonstrated an even greater rise in serum estradiol levels and ovarian weight. Conclusion These findings suggest that SAFA-FSH presents a promising alternative to current rhFSH treatments for female infertility. However, further research is essential to thoroughly assess its safety and efficacy in clinical contexts.

OBJECTIVES: Low handgrip strength (HGS) in children and adolescents might be associated with the risk of metabolic syndrome (MetS) and insulin resistance. This study prospectively evaluated the association between HGS in childhood and MetS in adolescence. METHODS: Based on data from the Ewha Birth and Growth Study, this study analyzed HGS at ages 7 to 9 and metabolic indices at ages 13 to 15. In total, 219 participants were analyzed. The risk of MetS was evaluated using the continuous metabolic syndrome score (cMetS), and insulin resistance was assessed using fasting blood insulin and homeostasis model assessment of insulin resistance (HOMA-IR). Relative HGS in childhood was determined by dividing HGS by body weight and categorized as sex-specific quartiles. RESULTS: This study found an inverse association between relative HGS levels in childhood and MetS and insulin resistance in adolescence. For each 1-group increase in relative HGS quartiles, cMetS (standarard [Std] β=-0.64, p<0.01), HOMA-IR (Std β=-0.21, p<0.01), and fasting blood insulin (Std β=-0.21, p<0.01) all decreased on average. These associations remained significant even after adjusting for confounding factors. CONCLUSIONS Our study showed a prospective association between HGS in childhood and the risk of MetS and insulin resistance in adolescence. It provides significant epidemiological evidence, emphasizing the importance of efforts to increase muscle strength from a young age to mitigate the risk of MetS and insulin resistance in adolescence.

Purpose: To compare the clinical outcomes of XEN45 gel stent implantation surgery (ab externo, open conjunctiva) with those of trabeculectomy. Methods: We retrospectively reviewed electronic medical record of 57 patients (62 eyes) treated with XEN implantation surgery (ab externo, open conjunctival approach) between April 1, 2021, and July 31, 2023, by the same surgeon. Preoperative clinical data including intraocular pressure (IOP), the number of glaucoma medications, and visual acuity were collected from 1 day to 12 months postoperatively. These data were compared and analyzed with those of 67 patients (78 eyes) received trabeculectomy between February 1, 2017, and April 30, 2022, by the same surgeon. Statistical analyses were performed with p < 0.05 as significant. Results: Complete surgical success rate was 33.9% and 57.7% of the XEN and trabeculectomy groups, respectively (p = 0.005). The qualified success rate was 79.0% and 93.6%, respectively (p = 0.011). Postoperatively, the XEN group used more glaucoma medications than the trabeculectomy group (1.21 ± 1.05 vs. 0.69 ± 0.90, p = 0.003 at postoperative month 12). After postoperative month 1, the XEN group had a higher IOP (15.77 ± 5.07 mmHg vs. 13.17 ± 3.81 mmHg; at postoperative month 12, p = 0.001) and lower corneal astigmatism than the trabeculectomy group (1.32 ± 0.79 diopters vs. 1.88 ± 1.45 diopters, p = 0.020 at postoperative month 6). There was no significant difference in preoperative and postoperative best-corrected visual acuity (logMAR) between the groups at any of the follow-up period (favorable visual acuity subgroup; logMAR < 0.7). Postoperative complications were 0 cases of XEN group and 13 cases of trabeculectomy group (0% vs. 16.7%, p = 0.001). Also, XEN surgery (24 minutes 40 seconds ± 6 minutes 26 seconds) had a shorter operation time than the trabeculectomy (40 minutes 18 seconds ± 8 minutes 27 seconds, p < 0.001). Conclusions Compared to trabeculectomy, XEN surgery (ab externo, open conjunctiva) showed relatively lower effectiveness (surgical success rate, IOP reduction). However, it demonstrated advantages as a minimally invasive glaucoma surgery, including a surgical success rate approaching about 80%, stability in inducing corneal astigmatism, fewer postoperative complications, and shorter operation times.

Background: Recombinant human follicle-stimulating hormone (rhFSH) is commonly used to treat female infertility, but its short half-life necessitates multiple doses. Even corifollitropin alfa, with an extended half-life, requires supplementary injections of rhFSH after 7 days. This study aimed to develop and evaluate a long-acting follicle-stimulating hormone (FSH) formulation using anti-serum albumin Fab-associated (SAFA) technology to avoid additional injections and enhance ovarian function. Methods: SAFA-FSH was synthesized using a Chinese hamster ovary expression system. Its biological efficacy was confirmed through assays measuring its ability to stimulate cyclic adenosine monophosphate (cAMP) production, estradiol synthesis, and the expression of human cytochrome P450 family 19 subfamily A member 1 (hCYP19α1) and human steroidogenic acute regulatory protein (hSTAR) in human ovarian granulosa (KGN) cells. To evaluate the effects of SAFA-FSH, we compared its impact on serum estradiol levels and ovarian weight increase with that of rhFSH in Sprague-Dawley (SD) rats using the modified Steelman-Pohley test. Results: The results indicated that SAFA-FSH induces cAMP synthesis in KGN cells and upregulates the expression of hCYP19α1 and hSTAR in a dose-dependent manner. Female SD rats, aged 21 days, receiving daily subcutaneous human chorionic gonadotropin injections for 5 days exhibited a significant increase in serum estradiol levels and ovarian weight when administered SAFA-FSH on the first day or when given nine injections of rhFSH over 5 days. Notably, the group receiving SAFA-FSH on the first and third days demonstrated an even greater rise in serum estradiol levels and ovarian weight. Conclusion These findings suggest that SAFA-FSH presents a promising alternative to current rhFSH treatments for female infertility. However, further research is essential to thoroughly assess its safety and efficacy in clinical contexts.

OBJECTIVES: Low handgrip strength (HGS) in children and adolescents might be associated with the risk of metabolic syndrome (MetS) and insulin resistance. This study prospectively evaluated the association between HGS in childhood and MetS in adolescence. METHODS: Based on data from the Ewha Birth and Growth Study, this study analyzed HGS at ages 7 to 9 and metabolic indices at ages 13 to 15. In total, 219 participants were analyzed. The risk of MetS was evaluated using the continuous metabolic syndrome score (cMetS), and insulin resistance was assessed using fasting blood insulin and homeostasis model assessment of insulin resistance (HOMA-IR). Relative HGS in childhood was determined by dividing HGS by body weight and categorized as sex-specific quartiles. RESULTS: This study found an inverse association between relative HGS levels in childhood and MetS and insulin resistance in adolescence. For each 1-group increase in relative HGS quartiles, cMetS (standarard [Std] β=-0.64, p<0.01), HOMA-IR (Std β=-0.21, p<0.01), and fasting blood insulin (Std β=-0.21, p<0.01) all decreased on average. These associations remained significant even after adjusting for confounding factors. CONCLUSIONS Our study showed a prospective association between HGS in childhood and the risk of MetS and insulin resistance in adolescence. It provides significant epidemiological evidence, emphasizing the importance of efforts to increase muscle strength from a young age to mitigate the risk of MetS and insulin resistance in adolescence.

Background: and Purpose This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM). Methods: We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with antiSRP54 antibodies were determined. Results: The ELISA for anti-SRP54 antibodies had a sensitivity and specificity of 88% and 99%, respectively, along with a test–retest reliability of 0.92 (p<0.001). The 32 patients diagnosed with anti-SRP IMNM using a line-blot immunoassay included 28 (88%) who tested positive for anti-SRP54 antibodies using the ELISA, comprising 12 (43%) males and 16 (57%) females whose median ages at symptom onset and diagnosis were 43.0 years and 43.5 years, respectively. Symptoms included proximal muscle weakness in all 28 (100%) patients, neck weakness in 9 (32%), myalgia in 15 (54%), dysphagia in 5 (18%), dyspnea in 4 (14%), dysarthria in 2 (7%), interstitial lung disease in 2 (7%), and myocarditis in 2 (7%). The median serum creatine kinase (CK) level was 7,261 U/L (interquartile range: 5,086–10,007 U/L), and the median anti-SRP54 antibody level was 2.0 U/mL (interquartile range: 1.0–5.6 U/mL). The serum CK level was significantly higher in patients with coexisting anti-Ro-52 antibodies. Conclusions This study has confirmed the reliability of the ELISA for anti-SRP54 antibodies and provided insights into the clinical, serological, and pathological characteristics of South Korean patients with anti-SRP IMNM.

Purpose: This study aimed to explore the relationship between grit and work engagement among millennial and Generation Z (MZ generation) nurses, as well as the moderating effect of work friendships on this relationship. Methods: The study included 191 MZ generation nurses with over six months of experience, surveyed via a self-administered questionnaire at four general hospitals in Daejeon and Seoul from December 2020 to February 2021. Descriptive statistics, t-tests, one-way analysis of variance, Pearson’s correlation analysis, and hierarchical multiple regression were used to analyze the collected data. The PROCESS macro program with bootstrapping was employed to evaluate the significance of mediating effects. Results: Work friendships partially mediated the association between grit and work engagement, with an explanatory power of 29.9%. The significance of the indirect mediating effect using bootstrapping analysis indicated an effect size of 0.33, a lower limit confidence interval of 0.17, and an upper limit confidence interval of 0.52, which was significant as it did not include Zero. Conclusion Developing a program to improve grit is necessary to increase the work engagement of MZ generation nurses, and establishing and implementing a program to improve work friendships, along with education for individual nurses, is crucial.

Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a neurodegenerative disorder caused by a biallelic expansion of pentanucleotide repeats in the RFC1 gene. Previous studies have reported up to 22% of patients with late-onset ataxia harbor this pathogenic repeat expansion. Despite its relatively high prevalence, CANVAS is often underdiagnosed because the disease is not well recognized and genetic testing is not performed in clinical practice. Here, we present a patient with characteristic clinical features, confirmed by genetic testing.